Journal of Clinical Rheumatology and Immunology

Nonsteroidal anti-inflammatory drugs (NSAIDs) can induce hypersensitivity reactions with various clinical manifestations including acute/delayed reactions and three common phenotypes, NSAID/aspirin-exacerbated respiratory disease (NERD or AERD) with/without chronic rhinosinusitis (CRS) and nasal polyps, NSAID-exacerbated cutaneous disease (NECD). NSAID-induced urticaria/angioedema (NIUA). NIAU is commonly combined with anaphylaxis and named as NIUAA. The major pathogenic mechanism is the inhibition of cyclooxygenase-1 with a reduction in prostaglandin E2 levels, leading to the overproduction of cysteinyl leukotrienes and activation of inflammatory cells, including eosinophils and mast cells.

To confirm the diagnosis, provocation testing via the oral route or inhalation remains the gold standard; in vitro diagnostic methods are still not available. Essential managements are: (1) avoidance of cross-reacting NSAIDs along with the use of alternative analgesics; (2) pharmacologic treatment should follow standard guidelines in patients with underlying asthma/rhinitis (NERD, CRS), and urticaria (NECD). Aspirin desensitization and biologic treatment can be done when indicated.

Delayed reactions, including fixed drug eruptions, maculopapular eruptions, and severe cutaneous adverse reactions, are rare and mediated by T-cell responses. Symptomatic treatment with avoidance is essential. NSAID is a cofactor of food-dependent exercise-induced anaphylaxis. In this talk, we will update various clinical phenotypes and discuss practical issues faced in real clinical practice.

The 2022 European League Against Rheumatism (EULAR) guidelines have introduced a significant advancement in the diagnostic approach to Giant Cell Arteritis (GCA). According to these updated guidelines, Ultrasound (US) examinations of the cranial and axillary arteries should be considered the primary imaging method for evaluating patients who are suspected to have GCA, provided that the examinations are performed by adequately trained specialists using high-quality equipment. This recommendation marks a notable shift in the traditional management practices for GCA, as it acknowledges the effectiveness of ultrasound as a non-invasive, efficient, and reliable point-of-care test for individuals with suspected GCA. As a result, this development has paved the way for the establishment of fast-track clinics dedicated to GCA diagnosis and treatment across various parts of the globe.

However, it is important to note that the use of ultrasound for diagnosing GCA comes with certain challenges. The highly technical nature of the ultrasound procedure, combined with its dependence on the expertise of the operator, often leads to criticism regarding its reliability as a clinical tool. This can create confusion among clinicians when they attempt to interpret formal ultrasound reports, especially if they are not trained in sonography themselves.

In this presentation, we will thoroughly examine the evidence supporting the use of ultrasonography in the diagnosis of Giant Cell Arteritis. The discussion will encompass several key concepts, including:

1. The validity of ultrasonography as a diagnostic tool for GCA.

2. The diagnostic performance of ultrasound in identifying the condition.

3. The practical utility of ultrasound in an Australian Fast Track Clinic setting.

4. The assessment of treatment response and sensitivity to change, as measured by ultrasound findings.

5. The various scoring tools utilized in ultrasound evaluations, such as the Halo Count, the Halo Score, and the OMERACT GCA Ultrasound Score.

6. A review of the EULAR guidelines and how they have evolved over time.

7. Critical information that clinicians who are not trained in sonography need to understand regarding the use of ultrasound in GCA.

Through this comprehensive review, we aim to clarify the role of ultrasonography in the diagnosis and management of Giant Cell Arteritis, empowering clinicians with the knowledge they need to effectively utilize this diagnostic tool in their practice.

Psoriatic arthritis (PsA) is a complex inflammatory condition characterized by both catabolic and anabolic changes that significantly impact patient outcomes.

Catabolic processes in PsA primarily manifest as bone erosions and joint destruction. These changes result from the inflammation triggered by cytokines, which promotes osteoclast activation and increased bone resorption.

On the other hand, anabolic changes are observed through the formation of enthesiophytes, bony projections that develop at the sites where tendons and ligaments attach to bone. This process is often seen as compensatory response to chronic inflammation and mechanical stress.

Clinicians should adopt a holistic approach that not only focuses on alleviating symptoms but also considers the long-term implications of these bone changes.

Understanding the dynamics of catabolic and anabolic changes in PsA is essential for developing effective treatment strategies and improving patient outcomes. By recognizing the significance of both bone erosions and enthesiophyte formation, healthcare providers can better tailor their interventions, ensuring a comprehensive management plan that addresses both inflammation and structural integrity.

Managing spondyloarthritis (SpA) presents several complex challenges that impact patient outcomes. One of the foremost issues is the delay in diagnosis, often due to overlapping symptoms with other conditions and insufficient awareness among clinicians. This delay can lead to significant joint damage and increased disability.

Differentiating between axial psoriatic arthritis (axial PsA) and ankylosing spondylitis (AS) is crucial for effective management. While both conditions share inflammatory features, axial PsA and AS may exhibit distinct clinical manifestations and imaging findings. An emphasis on comprehensive clinical assessment, including patient history and physical examination, can aid in accurate differentiation.

Non-radiographic axial spondyloarthritis (nr-axSpA) poses additional challenges, as conventional imaging may not reveal significant changes. Current strategies focus on early intervention and utilizing MRI for detecting inflammatory changes, thereby facilitating timely treatment initiation.

Treating early PsA before irreversible bone damage occurs is essential. Evidence suggests that aggressive management with disease-modifying antirheumatic drugs (DMARDs) and biologics may prevent long-term joint impairment.

In summary, addressing these multifaceted challenges, requires a comprehensive approach. By focusing on these areas, healthcare professionals can significantly improve the quality of care and long-term outcomes for patients with spondyloarthritis.

Psoriatic arthritis (PsA) is a chronic, immune-mediated, inflammatory arthropathy that presents with inflammation of the joints and entheses, including those of the axial skeleton, and is associated with increased mortality from cardiovascular diseases. Diagnosis is primarily based on clinical phenotype because of the diversity of the associated features, which can include skin and nail disease, dactylitis, uveitis, and arthritis. Early detection and timely management are crucial for improving clinical outcomes and patients’ quality of life. This lecture will focus on the management of the multiple domains in psoriatic arthritis and clinical insights on incorporating insights from clinical trials into daily clinical practice by streamlining clinical assessment in a short session.

The lecture will begin by reviewing the epidemiology and pathophysiology of PsA, emphasizing the inflammatory nature of PsA. The clinical domains of PsA including skin psoriasis, arthritis, dactylitis, enthesitis and axial involvement will be discussed, highlighting the importance of targeting multiple PsA domains to improve PsA patient’s physical functions and quality of life.

The lecture will then delve into the clinical management of PsA. We will discuss the current recommendations for PsA assessment, including measurement of different clinical domains of PsA. We will explore the PsA assessment in clinical trials and the streamlining strategies of assessment in real-life setting.

The management strategies for PsA will then be focused, emphasizing the importance of targeting improvement in multiple clinical disease domains of PsA. We will explore the current evidence-based guidelines for pharmacological treatments, including the role of different biological disease-modifying antirheumatic drugs.

Finally, the lecture will end with a real-world case sharing session, highlighting the importance of targeting multiple PsA domain by individualized treatment approaches to improve patient’s health outcomes and quality of life.

This lecture aims to equip clinicians by overseas medical expertise on optimizing routine clinical management of multiple disease domains in PsA, ultimately facilitating individualized care to PsA patient to improve their overall clinical outcomes.

Baricitinib, an oral selective Janus kinase (JAK)1/JAK2 inhibitor, is approved as monotherapy or in combination with methotrexate for treating adults with moderate-to-severe active rheumatoid arthritis (RA) and provides improvements in clinical signs, symptoms and patient-reported outcomes. In several pivotal Phase II and III RA trials, up to seven years of baricitinib treatment was well tolerated and provided rapid and sustained efficacy. These findings have been substantiated in the context of “real-world” settings. Real-world evidence (RWE) helps health authorities and healthcare providers gain further insights regarding treatment effectiveness and safety in real-life settings as they focus on heterogenous patient populations (who may be older, more treatment refractory, and with more comorbid conditions than clinical trial populations). RWE permits the identification of associations between treatment and rare AEs and also helps the medical community to better understand treatment patterns, such as line of therapy utilisation, and to define risks associated with lower effectiveness or poorer drug survival. This talk will explore the clinical and real-world data investigating the benefit: risk profile of baricitinib, confirming that the improved disease activity and physical function of patients with RA treated with this JAK inhibitor observed in clinical trials is translated into effectiveness in real-world clinical practice, with a low rate of discontinuations. The post-marketing Phase IIIb/IV trial Oral Rheumatoid Arthritis Trial (ORAL) Surveillance identified venous thrombotic events (VTE), major adverse cardiovascular events (MACE), and malignancy as potential safety signals associated with tofacitinib, a JAK1,3 inhibitor, in comparison to biologic anti-TNFs. In the case of baricitinib, while some, but not all, RWE points to potential VTE risks, data regarding MACE have been generally reassuring. Several real world and registry studies indicate that drug survival is consistently higher with baricitinib than with biological anti-cytokine drugs. In the case of patients with inadequate response to a first anti-TNF, EULAR recommendations for treatment change include cycling within drug class or switching the mode of action of advanced therapy. However, clinical trials and RWE confirm that switching to an advanced therapy of a different mode of action after anti-TNF failure is more effective and associated with lower rates of withdrawal than cycling to a different anti-TNF. In the case of patients with inadequate response to a first JAK inhibitor, RWE indicates that patients who cycle to another JAK inhibitor are more likely to achieve a good clinical response and have higher drug survival than patients switched to bDMARDs.

Psoriatic arthritis (PsA) is a chronic inflammatory arthritis that is associated with psoriasis (PsO) and affects about 20% of patients with psoriasis. PsA is characterized by a wide range of symptoms that can vary over time, affecting both the musculoskeletal system as well as the skin and nails. The heterogeneity of PsA means that patients might experience fluctuating and diverse manifestations of the disease, requiring a nuanced approach to treatment.

In light of this, the Group for Research and Assessment of Psoriasis and Psoriatic Arthritis (GRAPPA) released their 2021 treatment recommendations, which emphasize the importance of individualized therapy based on six specific domains: peripheral arthritis, enthesitis, dactylitis, axial disease, psoriasis, and nail disease. This domain-based approach is designed to address the multifaceted nature of PsA, ensuring that treatment is tailored to the specific clinical manifestations present in each patient. Following this, the European Alliance of Associations for Rheumatology (EULAR) in their 2023 PsA treatment recommendations also adopted a similar approach. They provided strong endorsements for newer therapeutic classes, such as Interleukin-23 inhibitors (IL-23i), particularly in the treatment of peripheral arthritis, significant skin involvement, and enthesitis.

Risankizumab stands out as the most recently approved IL-23 inhibitor for the treatment of PsA. Its mechanism of action involves specifically binding to the p19 subunit of IL-23, thereby blocking downstream signaling pathways and suppressing inflammatory responses. Recent analyses have demonstrated Risankizumab’s consistent efficacy across various patient demographics and disease characteristics, making it a versatile and effective option for managing PsA.

In this context, Professor Östör will present key trial data and share case experiences with IL-23 inhibitors across the different manifestations of PsA. The aim of this lecture is to enhance the understanding of how to tailor treatment plans according to individual patient preferences and clinical profiles. By delving into these data and case studies, Professor can provide valuable insights into optimizing treatment strategies, ultimately leading to improved patient satisfaction and overall disease control.

Axial spondyloarthritis (axSpA) is a chronic inflammatory disease that primarily affects the axial skeleton, encompassing both radiographic and non-radiographic forms. The disease is characterized by chronic back pain and spinal stiffness, which are the most common symptoms. However, it often includes peripheral manifestations and extra-musculoskeletal symptoms as well, making the clinical presentation quite diverse.

JAK inhibitors work by blocking the intracellular signaling pathways of several proinflammatory cytokines, which play a crucial role in the pathogenesis of inflammatory diseases. Given their proven efficacy in managing rheumatoid arthritis and their ability to mediate the signaling of cytokines involved in the pathogenesis of axSpA, JAK inhibitors have been the subject of numerous clinical trials in this context.

The Assessment of SpondyloArthritis international Society and the European Alliance of Associations for Rheumatology (ASAS-EULAR) joint recommendations provide comprehensive guidance for the management of axSpA in 2022. The guideline introduced the use of Janus kinase inhibitors (JAKi), as a completely new drug class in the treatment landscape, with efficacy demonstrated in radiographic axSpA. JAKi was recommended, as a means to treatment intensification, in axSpA patients with persistently high disease activity despite conventional treatment. Since then, more trial data results have been released, showing efficacy in non-radiographic axSpA population as well.

In this lecture, Professor Östör will delve into the critical role of shared decision-making in the management of axial spondyloarthritis. He will emphasize the importance of tailoring treatment plans to individual patient preferences and clinical profiles, a practice that is increasingly recognized as vital in achieving optimal outcomes. By examining current international guidelines and the latest study data, Professor Östör will provide a comprehensive overview of the benefit-risk profile of Upadacitinib in the treatment of axSpA. Professor Östör will also share his clinical experience with Upadacitinib, discussing various case studies to illustrate its application in real-world settings. This will include an exploration of how patient preferences and clinical profiles can influence treatment decisions, ensuring a personalized approach to managing this complex disease.

Treat-to-target (T2T) is a therapeutic approach used in several rheumatologic disorders such as rheumatoid arthritis, gout, axial spondylarthritis, and other inflammatory diseases such as inflammatory bowel disease. Developing a T2T strategy for systemic lupus erythematosus (SLE) has been difficult due to the complexity of the disease. The 2019 EULAR recommendations for the management of SLE, reestablished in the 2023 EULAR guideline, specified that the primary treatment goal should be remission or the lowest possible disease activity. In SLE, remission is associated with reduced organ damage, fewer flares, reduced hospitalisation, reduced mortality, and improved health-related quality of life. Previously remission in SLE was seen as an unattainable goal; however, the treatment landscape for SLE has evolved, and recent advancements, particularly the use of biologics, have emerged as promising therapeutic options to improve patient outcomes.

A new post-hoc analysis of the TULIP Phase III programme provides evidence across four years that remission is an achievable goal with anifrolumab, a type I interferon (IFN) receptor antagonist for adult patients with moderate to severe (SLE) who are receiving standard therapy. At Week 208, 30.3% (n=58/194) of patients treated with anifrolumab were in remission compared with 18.3% (n=12/65) of those in the standard therapy alone group. This was an increase from 19.7% (n=49/251) of patients treated with anifrolumab who achieved remission at the first LTE visit (Week 64) compared with 9.9% (n=10/104) of those in the standard therapy alone group. Treatment with anifrolumab was also associated with more frequent, prolonged, and sustained DORIS remission attainment compared with placebo during the 4-year TULIP+LTE period. Real-world case studies have also illustrated the effectiveness of biologics in controlling disease activity, enhancing quality of life, and establishing long-term safety profiles.

In conclusion, biologic therapies have emerged as a valuable treatment option for achieving remission or lupus low disease activity state. Biologics has opened a new era revolutionizing the management of SLE, offering renewed hope for patients living with this challenging autoimmune disease.

The International Association for the Study of Pain (IASP) agreed in 2020 on a revised definition of pain: “An unpleasant sensory and emotional experience associated with, or resembling that associated with, actual or potential tissue damage”.

Patients with rheumatic diseases frequently report pain as a primary symptom. However, rheumatology healthcare providers often lack the training to adequately differentiate between different causes of pain, which are mandatory for optimal treatment.

IASP classifies pain into 3 descriptive categories: nociceptive, neuropathic, and nociplastic pain. A recent review (Murphy AE et al, Arthritis Care & Research, 2023) gives a nice overview of these different forms of pain. Nociceptive pain derives from tissue injury, with subsequent sensation of pain by nociceptors. It is usually well-localized and can be precisely described by patients (i.e. rheumatoid arthritis, primary osteoarthritis) and there are localized pathologies that explain the pain symptoms. Neuropathic pain occurs with injury or insult to a peripheral or central nerve. There may be pressure on the nerve as in carpal tunnel syndrome, or changes in nerves caused by diabetes or other causes of nerve pathologies like mononeuritis multiplex. The pain and paresthesia typically follow the distribution of peripheral nerves in a dermatomal distribution. Nociplastic pain describes pain characterized by altered nociceptive processing (e.g., hypersensitivity), suggestive of dysregulation of CNS pain processing pathways. Nociplastic pain likely encompasses many different CNS pathways that lead to amplified processing of pain signals, decreased inhibition of pain, or both. Prototypical nociplastic pain conditions include both widespread (e.g., fibromyalgia) and localized conditions (e.g., chronic temporomandibular pain disorders and irritable bowel syndrome).

Patients with inflammatory or degenerative joint diseases may have one, two or three of the presently described forms of pain, and an important objective for the rheumatologists is to understand which types of pain our patients are suffering from. About one-quarter of the patients with inflammatory rheumatic joint diseases have additional fibromyalgia causing increased scores of patient-reported outcomes like patient’s global VAS and number of tender joints, and this results in elevated composite scores independent on the degree of inflammation. Thus, rheumatologists must try to differentiate between nociceptive pain caused by inflammatory or degenerative pathologies which may be treated by different ant-inflammatory treatments, neuropathic pain which may need operative of medical treatment, or nociplastic pain which should be non-medically treated.

The lung is a frequent target of autoimmune-mediated injury in patients with rheumatic diseases. Not infrequently, respiratory involvement may be the presenting manifestation of connective tissue diseases (CTDs). The prevalence of interstitial lung disease (ILD) in rheumatoid arthritis is about 20 to 30% of patients, while ILD may occur in 20 to 50% of patients with idiopathic inflammatory myositis, 40-50% or more of patients with systemic sclerosis, 2 to 5% of patients with systemic lupus erythematosus, 20 to 60% of patients with mixed connective tissue disease, and up to 25% of patients with Sjögren’s syndrome.

Rheumatic disease-associated lung involvement is a major cause of morbidity and mortality in these patients. It shows a considerable heterogeneity in incidence and prevalence, clinical course, and the involved lung structure depending on the underlying rheumatic disease. Each rheumatic disease is associated with a characteristic pattern of their lung disease.

The standard therapy has traditionally been immunosuppressant. With the evolvement of treatment, anti-fibrotic is now indicated for chronic fibrosing ILDs with a progressive phenotype, including autoimmune disease-associated ILD. By targeting the underlying mechanism of pulmonary fibrosis, it is shown that the annual rate of FVC decline can be reduced by more than 50%.

In this lecture, Prof. Oliver Distler and Prof. Toby Maher will review the importance of timely treatment, the management practices and treatment guidelines for CTD-ILD.

While many agents now exist for osteoporosis treatment, their clinical use is attenuated my questions about comparative effectiveness and safety. Calcium and Vitamin D are essential for optimal bone health and may have other health benefits but excessive supplemental doses, in the absence of deficiencies, may be deleterious. Greatest interest has been directed to rare occurrences of osteonecrosis of the jaw and atypical femoral fractures associated with bisphosphonates and denosumab. These potential adverse effects may be offset by selective use of a bisphosphonate “drug holiday”. On the other hand, denosumab discontinuation leads to rapid bone loss and a heightened risk of fracture, necessitating an “exit strategy.” Teriparatide may have comparatively greater efficacy than other drugs in high risk patients. Romosozumab, while highly efficacious in comparator studies with bisphosphonates, has shown a controversial increase in cardiovascular adverse outcomes. Data is emerging on novel osteoanabolics and their regulatory approval will be aided by surrogate endpoints. Using incidental CT scans and other newer technologies will help detect more at risk patients sooner. Despite the potential for mostly rare adverse effects, for the vast majority of patients the benefits of osteoporosis therapy far outweigh their risks.

2023 ACR/EULAR APS classification criteria started a new era for doctors to comprehensively understand this disease. According to current research evidence and clinical practice experiences, we summarize 10 rules for management of APS as followed:

Rule 1: APS is a systemic autoimmune disease with multi-systemic involvement and heterogeneity of clinical phenotypes.

Rule 2: Each APS related thrombotic event requires a comprehensive assessment of “risk factors”.

Rule 3: APS arterial n venous events w/ different mechanisms require separate predict models of recurrency.

Rule 4: APS-related arterial thrombosis are not only “thromboembolic” but also “vasculopathic”.

Rule 5: Microangiopathy is an unawareness and underestimated but pivotal phenotype of APS.

Rule 6: Effective screening & timely diagnosis of APS-valvular heart diseases are crucial for prevention and prognosis improvement.

Rule 7: APS-related hematological involvement includes thrombocytopenia & autoimmune hemolytic anemia.

Rule 8: Significant heterogeneity of OAPS indicates different mechanisms and the necessity of stratification before Treatment.

Rule 9: APLs is one of major contributing factors related irreversible organ damage in SLE

Rule 10: Treatment of APS requires individualized precision stratification strategies including immunomodulators.

Dr. Jose Alfredo Gómez Puerta’s upcoming lecture at ICCR 2024 will delve into the critical issue of organ damage in patients with systemic lupus erythematosus (SLE), highlighting the limitations of current standard care practices. He will begin by discussing the risks associated with persistent disease activity and the traditional use of high-dose steroids, which often lead to significant organ damage over time. Dr. Gómez Puerta will then present a paradigm shift in SLE treatment as recommended by the European League Against Rheumatism (EULAR) 2023 guidelines and share the clinical impact brought by the new recommendations. The guidelines advocate for reducing chronic steroid dosage and emphasize early intervention with biologic therapies to minimize the risk of long-term organ damage.

The lecture will feature new evidence supporting the benefits of early biologic intervention in managing SLE. Dr. Gómez Puerta will present data from recent studies demonstrating improved patient outcomes, including reduced disease flares and lower drug-related toxicities. These findings underscore the importance of integrating biologic therapies earlier in the treatment regimen to achieve better disease control and enhance long-term patient health.

In addition to discussing empirical data, Dr. Gómez Puerta will share real-world cases illustrating the positive impacts of early biologic intervention. These case studies will provide practical insights into how this approach can be effectively implemented in clinical practice, showcasing its potential to alter the natural course of SLE by delaying organ damage and improving the quality of life for patients.

Finally, the lecture will emphasize the need to optimize SLE management beyond short-term symptom relief. Dr. Gómez Puerta will advocate for strategies focused on long-term disease management, aiming to reduce flares, mitigate drug toxicities, and ultimately prevent organ damage. This comprehensive approach is crucial for altering the disease’s trajectory and achieving disease modification for SLE patients.

Systemic lupus erythematosus (SLE) is a chronic autoimmune disease with a diverse clinical presentation, including pulmonary involvement. While pulmonary complications are common in SLE, a subset of patients develop pulmonary arterial hypertension (PAH), a life-threatening condition characterized by elevated pulmonary vascular resistance and right ventricular dysfunction. Early detection and timely management are crucial for improving outcomes in this vulnerable patient population. This lecture will focus on the importance of PAH screening in SLE, highlighting the clinical insights and management strategies for this challenging condition.

The lecture will begin by reviewing the epidemiology and pathophysiology of PAH in SLE, emphasizing the role of autoimmunity, inflammation, and microvascular dysfunction. The clinical manifestations of PAH in SLE, including dyspnea, fatigue, chest pain, and syncope will be discussed. These symptoms may be subtle and often overlap with other SLE manifestations, making early recognition challenging.

The lecture will then delve into the importance of PAH screening in SLE. We will discuss the current recommendations for screening, including the use of blood markers to evaluate the PAH risk factors, echocardiography to assess right ventricular function and pulmonary artery pressure. We will also explore the role of other diagnostic tools, such as NT-proBNP test, pulmonary function tests and right heart catheterization, in confirming the diagnosis of PAH.

The management strategies for PAH in SLE will then be focused, emphasizing the importance of multidisciplinary care involving rheumatologists, pulmonologists, cardiologists, and other specialists. We will explore the current evidence-based guidelines for pharmacotherapy, including the role of vasodilators, endothelin receptor antagonists, and phosphodiesterase type 5 inhibitors.

Finally, the lecture will end with real-world cases, highlighting the heterogeneity of the disease, the need of identifying the disease early, and the need for personalized medicine approaches to improve outcomes for this vulnerable patient population.

This lecture aims to equip clinicians with the knowledge and tools to effectively identify PAH in SLE, facilitating early diagnosis and timely initiation of appropriate management strategies, ultimately improving patient outcomes.

Dermatomyositis (DM) is a systemic autoimmune rheumatic disease which can affect multi-organs, such as skin, muscle and lung. Five myositis-specific autoantibodies, i.e., TIF1r, MDA5, NXP2, Mi2 and SAE, are sero-markers of diagnostic value. The treatments for DM included glucocorticoids, csDMARDs, IVIg and biologics, e.g., rituximab. Type I IFN pathway is considered to be crucial in the pathogenesis of DM and the evidence of JAK inhibitors as treatment option in DM is accumulating. Herein, four clinical scenarios of DM, i.e., MDA5+DM-ILD, TIF1r+ cancer-associated DM, NXP2+ adult DM with GI involvement, and MSA negative DM, are presented to reveal the trials and tribulations to tackle this very difficult disease.

Over the past several decades, the treatment of rheumatoid arthritis (RA) has advanced significantly, and clinical, structural, and functional remission are achievable therapeutic goals. However, a substantial number of patients show resistance to multiple drugs. In particular, patients whose disease activity cannot be controlled despite the use of two or more biological disease-modifying antirheumatic drugs (DMARDs) or targeted synthetic DMARDs with different mechanisms of action have recently been referred to as having difficult-to-treat RA (D2T RA). D2T RA is a heterogeneous and multifactorial disease state, and the major problems are uncontrolled disease activity and decreased quality of life, as well as the economic burden. Since the concept of D2T RA is relatively new and publication regarding D2T RA is limited, the mechanism underlying DMARD inefficacy and which factors form a “difficult-to-treat” state are not yet fully understood. It is also possible that factors contributing to D2T RA may differ by patient, sex, country, and race. The present seminar introduces the current concept and unsolved problems of D2T RA, including the definition, prevalence, and factors contributing to D2T RA. Then, the management and therapeutic strategies for D2T RA are discussed including our multicentre ANSWER cohort experience. Finally, a clinical approach to prevent patients from developing D2T RA is explored.

The gastrointestinal (GI) system frequently experiences complications in systemic sclerosis. Pathological changes in the GI system can be revealed from the early onset of the disease, such as esophageal hypomotility, to the late stage of the disease, where severe complications like intestinal pseudo-obstruction may occur. Systemic sclerosis can cause pathological changes throughout the entire gastrointestinal tract. Common symptoms include dysphagia, choking, heartburn, hoarseness, abdominal distension, diarrhea, constipation, and fecal incontinence.

Oropharyngeal involvement in systemic sclerosis presents as weakened pharyngeal muscles, leading to oropharyngeal dysphagia, choking, food leakage, and aspiration, attributed to the dysfunction between the pharyngeal muscles and the upper esophageal sphincter due to muscle weakness. However, weak pharyngeal muscles may also be a clinical feature of the systemic sclerosis-polymyositis overlap syndrome.

Esophageal involvement is frequently observed, with conditions such as esophageal hypomotility and lower esophageal sphincter incompetence, leading to early symptoms of dysphagia, gastroesophageal reflux, and potential complications such as esophagitis, esophageal stricture, Barrett’s esophagus, and aspiration pneumonia. Furthermore, gastric involvement includes gastric antral vascular ectasia, commonly known as “watermelon stomach,” which leads to gastrointestinal bleeding and chronic anemia.

Intestinal manifestations comprise malabsorption, intestinal pseudo-obstruction, and bacterial overgrowth. Additionally, wide-mouthed diverticulosis and fecal incontinence are frequently found.

Liver involvement includes primary biliary cirrhosis, most prevalent in limited cutaneous systemic sclerosis or certain overlap syndromes rather than diffuse cutaneous systemic sclerosis.

The severity of these changes varies among patients, ranging from asymptomatic to severe conditions, which may result in malnutrition. Severe and chronic GI involvement significantly impacts daily life, making normal eating difficult, diminishing the quality of life, and potentially causing depression. Managing patients with systemic sclerosis who have GI involvement requires a multidisciplinary approach, involving collaboration among rheumatologists, gastroenterologists, nutritionists, surgeons, and possibly psychiatrists. This teamwork is essential to achieve successful patient care outcomes.

Objective:

We aimed to provide an up-to-date synthesis of the effectiveness of patient reported outcome measures (PROMs) use to self-monitor symptoms compared to conventional follow-up care in patients with rheumatic diseases. We also compared the effect of providing feedback via PROMs to a control group in which PROMs feedback was not provided.

Methods:

This review was guided by the Preferred Reporting Items for Systematic review and Meta-Analysis (PRISMA) guidelines. Articles published before October 2023 were retrieved from PubMed$Ⓡ$, Cochrane Library$Ⓡ$, Embase$Ⓡ$, and PsycINFO$Ⓡ$(Ovid). Studies were included if (1) they compared PROMs use compared against not using PROMs, (2) used PROMs as an intervention with feedback provided to healthcare professionals/patients to compare against PROMs use but no feedback was provided. Methodological quality and level of evidence were evaluated according to the Risk of Bias tool and the Risk of Bias in Non-randomized Studies of Interventions tool.

Results:

A total of 15, 571 articles were screened, 5 articles met the inclusion criteria, and 3 additional articles were identified from handsearching. Most studies reported positive results on symptom control, health-related quality of life, patient perception, shared decision making, and a reduction in clinic visits. No studies reported on survival/mortality. In general, we found conflicting evidence regarding whether feedback from physicians improved patient satisfaction in clinical care.

Conclusion:

We identified 8 articles through a systematic review and synthesised evidence on their effectiveness. There is a lack of studies comparing PROMs use to conventional care in rheumatic diseases. Future studies should explore the implications of using PROMs in clinical care.

Introduction

Axial spondyloarthritis (axSpA) is a group of autoinflammatory disease that hypothetically caused by tumor necrosis factor (TNF) axis and interleukin (IL)-23/IL-17 axis. Based on algorithm of treatment from ASAS/EULAR 2022, biologic agent is indicated if patient did not response to NSAIDs, the first line treatment. TNF inhibitor and IL-17 inhibitor are two biologic agent that has been approved and gave significant result in improving disease activity.

Case Description

A 47 years old woman who has been diagnosed with axSpA since 2022. At first she was given medication with NSAIDs. She has also been given conventional DMARD, sulfasalazine an methotrexate, until maximum dose but still no response in controlling the disease activity.

After about one year of treatment, she finally tried using the biologic agent, Secukinumab. She was given 150 milligram weekly initial dose for a month, followed by monthly administration. After administering the eight dose, she achieved inactive disease activity. However, after the fourteenth dose, symptoms began to reappear and disease activity became very high like the first time of diagnosis.

Discussion

IL-17A inhibitor (IL-17Ai) has been widely used for treating axSpA since ASAS20/40 and partial response at 16 week was reached 61,36 and 14%. IL-17Ai seem more effective in ameliorating entheseal inflammation and new bone formation, in accordance with role of IL-23/IL-17 axis in stimulating osteoclast.

ASAS/EULAR recommend patients who fail therapy with one biological agent in 12 weeks to switch to another type of biological agent. However there’s still no publication data for efficacy to give TNF inhibitor after IL-17Ai or JAK inhibitor failure.

Conclusion

The effectiveness of biological agents in axSpA is quite similar. In cases where therapy fails, it is still an open question whether the optimal therapy choice is to change the type of drug or a combination of two types of biological agents.

Background:

Early diagnosis of rheumatoid arthritis (RA) is crucial for timely intervention and improved outcomes. Although research on the preclinical phase of RA is a prominent topic, there remains an unmet need to effectively stratify patients at risk of developing RA based on basic clinical assessment and laboratory investigations. This prospective longitudinal study aimed to identify risk factors for RA development in individuals experiencing arthralgia.

Method:

Two hundred consecutive adults with arthralgia were enrolled from new referrals to our rheumatology clinic. Patients with synovitis or a known arthritis diagnosis were excluded. Follow-up assessments were conducted every 6 months, or sooner if symptoms worsened, for a maximum of 2 years. The study endpoint was the development of RA, according to the 2010 ACR/EULAR classification criteria. Baseline demographic characteristics, clinical parameters, serology, and acute phase reactant levels were compared between patients who developed RA and those who did not. In addition, the classification score based on the 2010 ACR/EULAR classification criteria was utilised as a composite weighted score summarising the clinical presentation in the cohort, although the patients were deemed not fulfilling the mandatory criteria of having synovitis at baseline.

Results:

By May 2024, 104 patients had been followed up for at least one year, with a median duration of 78 weeks (IQR: 58-97). The baseline symptom duration was 51 weeks (IQR: 29 – 97). Among these patients, 23 (22.1%) developed RA after a median follow-up duration of 41 weeks (IQR: 25 – 52). Patients who developed RA had a significantly higher proportion of joint symptoms <1 year, difficulty making a fist, positive rheumatoid factor (RF), anti-CCP antibodies, and elevated ESR and CRP levels at baseline. Multivariate logistic regression identified difficulty making a fist (OR: 4.87, 95% CI: 1.40 – 17.04, p = 0.013) and positive anti-CCP antibodies (OR: 13.04, 95% CI: 3.74 – 45.44, p < 0.001) as independent predictors for RA development. Meanwhile, patients who developed RA had significantly higher baseline scores extrapolating from the 2010 ACR/EULAR classification criteria compared to the non-RA group.

Conclusion:

Difficulty making a fist and positive anti-CCP antibodies are independent predictors of RA development. Additionally, patients who developed RA exhibited significantly higher baseline scores on the 2010 ACR/EULAR classification criteria. Early recognition of these variables and taking reference from the score of classification criteria may aid in RA risk stratification. Further research is needed to validate these findings and explore additional predictive markers.

Background:

Lupus nephritis (LN) frequently manifests as a significant complication in systemic lupus erythematosus (SLE) patients, with emerging research indicating a plausible correlation between subclinical retinal involvement and renal manifestations. This study aims to investigate the relationship between the neutrophil-to-C3 ratio (NC3R) and both LN as well as retinal microvasculature damage among SLE patients.

Methods:

In this cross-sectional study, a cohort of 220 participants was recruited, consisting of 78 LN patients and 142 patients without LN.

We assessed clinical indicators and organ involvement and conducted correlation analyses between NC3R and markers of lupus activity. Additionally, we analyzed the diagnostic performance of NC3R in diagnosing LN and constructing ROC curves. Logistic regression analysis was performed to investigate the impact of NC3R on LN. We further examined the influence of NC3R on retinal vasculature density based on its cutoff value and conducted correlation analyses accordingly.

Results:

The LN group exhibited a significant increase in NC3R compared to the group without LN (5.9 vs 4.5, P=0.007). NC3R demonstrated positive correlations with 24-hour proteinuria (R=0.329, P<0.001) and systemic lupus erythematosus disease activity index (SLEDAI) score (R=0.268, P<0.001). Multiple regression analysis revealed NC3R as an independent risk factor for LN (OR: 2.01, P=0.035). NC3R was proven valuable in distinguishing LN patients (AUC: 0.613), with an optimal cutoff value of 6.40 (sensitivity: 48.1%, specificity: 72%). Our results indicated that the lower NC3R group (NC3R<6.40) exhibited reduced vascular density, particularly within the macular region. Furthermore, we observed a positive correlation between NC3R levels and vascular density.

Conclusions:

NC3R demonstrated promising potential as a reliable indicator for predicting both LN and retinal microvasculature involvement. Consequently, the pre-treatment evaluation of NC3R had the potential to assist clinicians in identifying potential organ involvement among SLE patients.

Background:

Unlike axial spondyloarthritis (axSpA), no classification criteria are currently available for axial psoriatic arthritis (axPsA). This study aimed to evaluate the performance of the proposed cut-offs from the Assessment of Spondyloarthritis International Society (ASAS) data-driven definitions for active and structural magnetic resonance imaging (MRI) lesions typical of axSpA in distinguishing axial involvement in patients with psoriatic arthritis (PsA).

Methods:

Seventy-two consecutive PsA patients (67% male, aged 45± 14 years) meeting the CASPAR classification criteria, regardless of the presence of back pain, were included. All patients underwent radiography of the pelvis and spine, as well as sacroiliac joint (SIJ) MRI, while 52 (72%) of 72 patients also underwent whole-spine MRI. The final diagnosis of axPsA was ascertained by two experienced rheumatologists. One rheumatologist with expertise in imaging and one trained reader evaluated the radiography and MRI images.

Results:

AxPsA was diagnosed in 27/72 (38%) patients. The proposed cut-offs for active sacroiliitis demonstrated high specificity (95.6%) but relatively low sensitivity (51.9%) in distinguishing patients with and without axPsA. When structural lesions of the SIJ were included in addition to active lesions, the sensitivity significantly improved (96.3% vs. 51.9%) with a modest decrease in specificity (86.7% vs. 95.6%). Incorporating MRI spine lesions (using the proposed cut-offs for positive spine MRI from the SPACE cohort) alongside SIJ lesions did not further change sensitivity or specificity compared with assessing SIJ alone.

Conclusion: The ASAS-proposed cut-offs for identifying active and structural lesions of SIJ demonstrated satisfactory performance in discriminating axial involvement in PsA patients.

Background

Despite the wide availability of biologic/targeted synthetic disease-modifying anti-rheumatic drugs (b/tsDMARDs) for treating psoriatic arthritis (PsA), there remain controversies regarding the positioning of b/tsDMARDs in the management algorithm for patients with active peripheral disease. While the European League Against Rheumatism (EULAR) recommends conventional synthetic DMARDs (csDMARDs) as the first-line treatment (1), the American College of Rheumatology (ACR) guideline takes a more aggressive approach, and suggests upfront use of b/tsDMARDs (2). The study aimed to evaluate whether earlier use of b/tsDMARDs might increase the likelihood of achieving minimal disease activity (MDA) in patients with PsA under the treat-to-target framework.

Method

Consecutive patients with PsA who fulfilled the CASPSA criteria with active peripheral disease were recruited in this prospective cohort study. The analysis included patients who were (1) given b/tsDMARDs during the study period and (2) completed 1-year follow-up. A standardized treat-to-target strategy was adopted. Patients were categorized into csDMARDs-naïve group, single-csDMARD-inadequate response (IR) group and multiple-csDMARDs-IR group. The rate of achieving MDA at the end of one year was compared.

Result

A total of 100 PsA patients were recruited and 27 patients (mean age: 51± 13, 15 [68%] male, disease duration: 5.6± 6.9 years) were included in the analysis. At baseline, all patients exhibited moderate to high disease activity. Prior to initiating b/tsDMARDs, 4 patients were csDMARDs-naïve, 10 patients had failed 1 csDMARD and 13 patients had failed 2 or more csDMARDs. There was no significant difference between clinical features and disease activity among the 3 groups at baseline (Table 1). Within the study period, 3 (11%) and 24 (89%) patients were given anti-TNF and anti-IL-17 agents respectively. After 1-year, there was substantial improvement in disease activity in the whole cohort (Disease Activity in Psoriatic Arthritis [DAPSA]: 22.7 at baseline vs 9.0 at 1-year, p=0.039), and 17(63%) patients achieved MDA. A differential MDA achievement rate was observed across different groups. All csDMARDs-naïve patients achieved MDA, compared to 70% in the single-csDMARD-IR and 46.2% in the multiple-csDMARDs-IR groups (p=0.046) (Figure 1). There was also a trend of better DAPSA response at one-year in the csDMARDs-naïve group (4.9± 1.6 vs 10.2± 11.8 [single-csDMARD-IR] and 9.4± 8.1 [multiple-csDMARDs-IR]).

Conclusion

A lower MDA achievement rate was observed in b/tsDMARDs users with prior csDMARD exposure. Earlier initiation of b/tsDMARDs might improve treatment outcome in PsA.

Background:

Psoriatic arthritis (PsA) is an inflammatory musculoskeletal disease associated with psoriasis (PsO) that appears approximately 7-8 years after psoriasis and remains undiagnosed in most of patients, which is disabled and difficult to treat. So early risk assessment is particularly important. However, at present, the risk assessment of the status quo is not optimistic. The aim of this study was to establish and validate a prediction model for assessing the risk of PsA in psoriasis patients.

Methods:

Ambispective cohort study was conducted. Demographic and clinical records were collected and followed up and blindly reviewed. Single and multiple factors analysis were used to analyze the factors influencing PsA, and Logistic regression equation models were developed to verify their predictive value.

Results:

Among 1141 Pso patients, 221 patients had PsA. Logistic regression equation model showed that Arthralgia (OR= 438.079; 95% CI: 175.154-1095.682), nail dystrophy (OR= 7.453; 95% CI: 4.116-13.498), scalp lesions (OR= 3.047; 95% CI: 1.297-7.159), Inverse (intertriginous) lesions (OR= 2.45; 95% CI: 1.382-4.342) and BMI (OR= 0.909; 95% CI: 0.846-0.977) were identified as potential predictors affecting the risk of transition from PsO to PsA. And the receiver operating characteristic (ROC) curve of PsA was drawn, the area under the curve (AUC) was 0.974 (95% CI 0.963 - 0.985), the prediction sensitivity was 96.40%, the specificity was 92.10%, the calibration curve showed that the predicted results was in good agreement with the observed results.

Conclusion:

The independent predictors of PsA involve Arthralgia, nail dystrophy, scalp lesions, Inverse (intertriginous) lesions, BMI. The Logistic prediction model based on these predictors has reliable predictive value and can help early clinical assessment and treatment.

Background:

Axial spondyloarthritis (axSpA) is associated with accelerated atherosclerosis driven by chronic inflammation. Achieving LDA may result in significant benefits in articular disease, little is known about its effect on extra-articular disease, including CVD risk in axSpA. To investigate the effect of achieving Ankylosing Spondylitis Disease Activity Score (ASDAS) low disease activity (LDA) using a protocolized treatment strategy on the progression of subclinical atherosclerosis and arterial stiffness in axial spondyloarthritis (axSpA) patients.

Methods:

One hundred consecutive AxSpA patients will participate in this 2-year prospective, hospital-based, cohort study. All participants will receive 2 years of tight-control treatment. Treatment will be adjusted according to a standardized protocol every 3-6 monthly aiming at ASDAS LDA (ASDAS <2.1). Carotid intima-media thickness (IMT) and total plaque area (TPA) will be measured using high-resolution ultrasound. Arterial stiffness is measured by pulse wave velocity (PWV) and augmentation index (AIx). The main outcome measure is the change in subclinical atherosclerosis over a period of 2 years comparing between patients who achieve sustained LDA at year 2 (sLDA group: achieved LDA at both year 1 and year 2) to those who cannot achieve LDA (non-sLDA group). Secondary outcomes include differences in the changes in AIx and PWV over 2 years between the 2 groups.

Results:

In this interim analysis, a total of 56 axSpA patients (age: 38± 10 years, 43 (76.8%) male) who completed 2 year of follow-up were included. A significant improvement in disease activity was observed after 2 years of treatment (ASDAS-CRP at baseline: 2.9± 0.6 vs 1.9± 0.7 at year 2, p<0.001). Forty-one patients achieved LDA at year 1 and 31 patients achieved sustained LDA (sLDA) at year 2. The baseline traditional CV risk factors were comparable between the sLDA and non-sLDA groups. The multivariate analysis demonstrated that the sLDA group exhibited a smaller increase in mean IMT ($\beta$: -0.04, 95%CI -0.07 to -0.01, P=0.025) and max IMT ($\beta$: -0.06, 95%CI -0.12 to -0.004, P=0.038) after adjustment for covariates compared to the non-sLDA group. However, there were no significant differences in the progression of carotid plaque and arterial stiffness markers between the two groups in the multivariate analysis.

Conclusion:

Patients with AxSpA who are able to achieve sLDA through a protocolized treatment algorithm experience reduced progression of subclinical atherosclerosis compared to patients who cannot achieve sLDA. This indicates that effective suppression of inflammation in AxSpA patients who reach sLDA may potentially lower CV risk.

Background:

Despite the widespread advocacy of the treat-to-target(T2T) strategy for managing psoriatic arthritis (PsA), a significant number of patients fail to achieve minimal disease activity (MDA) even with advanced therapies. While a universal definition of difficult-to-treat(D2T) PsA is absent, investigating the heterogeneity of D2T PsA within a real-life T2T-cohort can offer valuable insights into comprehending this concept.

Methods:

This analysis included the first 111 PsA patients enrolled in the APLAR SpA registry who underwent 1-year T2T management. They were recruited from 6 Asia-Pacific regions. D2T was defined as the failure to achieve MDA despite receiving $\ge$1 conventional synthetic disease-modifying anti-rheumatic drugs(csDMARDs) and $\ge$1 biologic/targeted synthetic DMARDs(b/tsDMARDs) over 6-months.

Results:

A total of 111 patients (mean age: 48± 13 years, 59 [53%] male, mean disease duration: 5.3± 7.3 years) were included. At baseline, the patients exhibited moderate disease activity, with only 35% achieving MDA. After 1-year, a significant improvement in Disease Activity in Psoriatic Arthritis (DAPSA) was observed (16.3± 14.0 at baseline vs 10.1± 9.7 at 1-year, p<0.001), with 56% of patients achieving MDA. At one-year, 17(15%) patients fulfilled the definition of D2T-PsA. Compared to the non-D2T group, patients in the D2T group had lower education level, longer disease duration, worse disease activity across domains, and higher functional disability at baseline (Table 1). During the 1-year treatment, the T2T adherence rate was significantly lower in the D2T group (71% vs 90%, p=0.028). Reasons for not escalating treatment in the D2T group included patients’ preference (60%), physician’s decision (20%), and no alternative treatment available (20%). Using multivariate logistic regression, a lower education level (OR:4.64, 95%CI:1.16-16.9, p=0.029) and higher Ankylosing Spondylitis Disease Activity Score (ASDAS) (OR: 1.81, 95%CI:1.07-3.04, p=0.026) were significantly associated with D2T after adjusting for baseline disease duration, number of dactylitic digits, MDA status and protocol adherence.

Conclusions:

Despite the implementation of the T2T-strategy and increased utilization of b/tsDMARDs, more than 40% of patients were unable to achieve MDA. Factors such as higher axial disease activity and lower education level were associated with D2T PsA. Further studies are required to determine whether a more aggressive treatment approach focusing on the axial domain should be implemented for these individuals.

Background:

Janus kinases inhibitor (JAKi) is a potent drug in treating patients with rheumatoid arthritis (RA). However, the effect of JAKi on finite structural changes remained uncertain. This study aims to investigate the effect of JAKi on erosion repair evaluated by high-resolution peripheral quantitative computer tomography (HR-pQCT) in patients with active RA.

Methods:

This was a prospective, non-randomized pilot study. We enrolled 20 adult patients with active RA with $\ge$1 bone erosion on HR-pQCT. They were given upadacitinib 15mg once daily for 24 weeks. HR-pQCT of the metacarpophalangeal joint was performed at baseline and 24-week. The serum bone biomarkers level was evaluated before and after treatment. Twenty age-and-sex matched RA patients from another study treated with conventional synthetic disease modifying anti-rheumatic drugs (csDMARDs) were included as active controls.

Results:

Nineteen patients in the upadacitinib group completed the study procedures. After 24 weeks, despite similar improvement in disease activity, a reversed trend in the mean erosion volume change on HR-pQCT was observed comparing the upadacitinib and active control group (upadacitinib group: -0.23± 3.26mm³vs control group: 1.32± 6.05mm³, p=0.131). A greater proportion of erosions in the upadacitinib group demonstrated regression (27% vs 12%, p=0.085). Using general estimating equation (GEE), the use of upadacitinib was significantly associated with erosion regression (OR: 3.61, 95% CI: 1.00-13.00, p=0.049) after adjusting for the difference in disease duration. The serum levels of bone resorption markers reduced after upadacitinib treatment. No new safety signal was noted.

Conclusion:

Despite a similar improvement in RA disease activity after upadacitinib compared to csDMARDs, a differential regression of erosion on HR-pQCT was observed in patients received upadacitinib. The potential role of JAK1 inhibition in erosion repair should be investigated.

Background:

Cardiovascular system involvement in Behçet’s disease is rare, especially requires surgical intervention. We report a case of a 47-year-old Chinese male with Behçet’s disease, treated with immunosuppressive agents, who developed an infection at the surgical site following the postoperative implantation of a pacemaker. Little is known about the approach to managing postoperative wounds in Behçet’s disease. This article aims to provide a reference for nursing care, improve perioperative management, and assist in achieving a good prognosis for Behçet’s disease with cardiovascular system involvement.

Method:

We used the following three-phase process:

1. Followed authoritative guidelines for the perioperative management of patients with cardiac implantable electronic devices (CIED), which included strict aseptic techniques, pressure bandaging, and the preventive use of antibiotics.

2. Conducted a literature search for reasons and solutions to pain and infection symptoms at the surgical site.

3. Cleaned the wound using different disinfectants and evaluated the wound’s condition; managed pain through a multimodal approach within an Acute Pain Service (APS) team management model. Determined whether to continue immunosuppressive therapy based on the Pathergy test after the patient had suspended immunosuppressive therapy for over a month.

Results:

Our case illustrates the wound healing process during the patient’s 50-day hospitalization, from a secondary wound with a 5x5 cm rupture and exudation to Grade A healing. The patient’s Numeric Rating Scale (NRS) pain score decreased from 6 points to 0 points upon discharge. Three months post-discharge, follow-up revealed slight scarring at the wound site, normal pacemaker function, and stable control of Behçet’s disease.

Conclusion:

Patients with Behçet’s disease under immunosuppressive treatment have a higher risk of infection at the surgical site following pacemaker implantation. Using hydrogen peroxide first, followed by iodine, and finally physiological saline during wound debridement is significantly effective in controlling infection. The APS team management model, composed of attending physicians and nurses, can effectively improve pain relief quality during wound recovery. The Pathergy test is an effective method to evaluate whether disease activity affects wound healing after CIED implantation and whether continued use of immunosuppressive therapies is necessary.

Background:

Rheumatoid arthritis (RA) is characterized as an autoimmune inflammatory disease associated with synovial joints. Naringin (NG) is a flavanone type of glycoside found in grapes and citrus fruits, and reported to be active against RA. However, poor bioavailability and low solubility restricted its clinical use. Therefore, considering the issue, the present study was focused on the development, characterization and exploration of the protective efficacy of naringin-mediated silver nanoparticles (AgNNPs) against RA by using in vitro and in vivo methods.

Methods:

AgNNPs were biosynthesized by co-precipitation approach and characterized by different techniques. Biofabricated AgNNPs were employed to analyze the anti-arthritic effects by using an in vivo mouse model, i.e., type II collagen-induced arthritis (CIA) model and various parameters including degree of paw swelling, polyarthritis index score as well as level of cytokines in serum were evaluated. The effect of AgNNPs on the proliferation level of fibroblast-like synoviocytes (FLS) induced by tumor necrosis factor-$\alpha$(TNF-$\alpha$) was also taken into account. Western blot and immunofluorescence staining were employed to detect the status of autophagy and underlying PI3K/AKT/mTOR pathway in the CIA model and FLSs.

Results:

Various characterization techniques confirmed the formation of spherical crystalline nanoparticles with a size range of 40-80 nm. In vivo study results revealed that AgNNPs significantly reduced (P < 0.01) the RA-associated damaging effects such as level of paw edema, and pro-inflammatory cytokines along with inhibition of FLSs proliferation associated with TNF-$\alpha$. Level of autophagosome and protein expression of LC3B in TNF-$\alpha$induced FLSs, LC3-II and Beclin-1 were enhanced whereas mTOR and AKT phosphorylation were reduced by AgNNPs treatment.

Conclusion:

The study exhibited an ameliorative effect of biosynthesized AgNNPs against RA by modulating autophagy through PI3K/AKT/mTOR signaling pathway.

Introduction:

Nailfold Capillaroscopy (NFC) is a microscopic examination equivalent of an in vivo assessment of microcirculation. It is an invaluable examination in patients presenting with Raynaud’s phenomenon (RP) (triphasic color changes of fingers commonly on cold exposure) which might underlie a spectrum of rheumatic diseases e.g. scleroderma and mixed connective tissue diseases (CTD). Early diagnosis of these disease can allow investigation of other internal organ involvement and starting appropriate drug therapy e.g. immunosuppressants for immune-related part, and vasodilatory therapy as a symptomatic treatment.

Methodology

NFC was performed by Optilia$Ⓡ$Digital Video microscopy with 200x lens with LED illumination. Rheumatologists had performed NFC from 6/2020-11/2022 for existing scleroderma and mixed CTD patients. Rheumatology nurses started to perform NFC on patients with RP since 11/2022 (study period till 4/2024) after undergoing certified workshop training and having direct supervision from in-house rheumatologists. 3 rheumatology nurses had been involved in acquiring video images from NFC and 1 of them, an APN, drafted the reports which would be finally authorized by rheumatologists. Clinical data, revised diagnosis and drug changed of performed NFC patients were analyzed.

Result:

56 female and 15 male with average 51 years old were performed NFC. All had abnormal NFC findings. Crucially there were accompanying diagnosis revision and subsequent major medication changes (rheumatologists’ initiation or stepping-up of immunosuppressants and vasodilatory agents):

Rheumatologists verified all images and agreed on the final conclusions, except 1 (1.5%) reported as non-specifics findings by nurse and edited as late-stage scleroderma by rheumatologist.

Conclusion:

Rheumatology nurses are competent and reliable in performing NFC and had expanded patients’ group from known scleroderma and CTD to patients with RP in a wider scope. Waiting time was reasonably around 1 month. The significant clinical impact is apparent with the findings helping the rheumatologists to revise diagnosis timely and to adjust drug treatment accordingly.

Background:

Ferroptosis is a recently discovered type of regulated necrosis and glutathione peroxidase 4 (GPX4) has been recognized as a key enzyme that protects against ferroptosis. However, the role of platelet GPX4 in systemic lupus erythematosus (SLE) has not been explored. In this study, GPX4 levels in platelets were measured and the correlation with clinical features were analyzed.

Methods:

Platelet GPX4 protein expression was detected by Western blot and immunofluorescence in 37 SLE patients and 23 healthy controls. Clinical data were recorded at time points of blood sampling. Platelet activation was analyzed by flow cytometry, LDH release by LDH release assay and plasma oxidized DNA levels by a general 8-OHdG ELISA kit.

Results:

Patients with SLE had significantly decreased expression of platelet GPX4 and increased percentage of platelet activation as compared with healthy controls. Furthermore, levels of platelet GPX4 were negatively correlated with SLEDAI-2K score, 24-hour urine protein and oxidized DNA. Low GPX4 expression were associated with skin and joint involvements of SLE patients. Interestingly, deferoxamine (DFO), a ferroptosis inhibitor, could protect SLE platelets from death.

Conclusions:

These findings confirmed that downregulated GPX4 protein in platelets of SLE patients negatively correlated with plasma oxidized DNA, SLE disease activity and 24-hour urine protein, suggesting the potential role of platelet ferroptosis in oxidized DNA release and the pathogenesis of SLE.

Background

Nowadays, technological advances enable instant data collection in a secure and confidential manner. Integrating electronic data capture technologies into the online clinical trial design, has been rarely investigated. The aim of this study was to test the feasibility and logistics of the online clinical trial model in osteoarthritis.

Methods

We used Research Electronic Data Capture (REDCap), a versatile and secure database, as the data management platform. Without face-to-face interactions, the majority of the communications among investigators and between investigators and participants were through the REDCap platform. The embedded randomisation procedure was also completed on the same platform.

Results

We developed the online trial model in a 12-week, phase II, placebo-controlled, randomised clinical trial (RCT) investigating the efficacy and safety of a supplement combination in people with hand osteoarthritis (RADIANT study). We replicated the same platform and questionnaire model to a larger hybrid RCT, a phase III, two-year placebo-controlled trial investigating the use of stem cells in the treatment of knee osteoarthritis (SCUlpTOR study).

In the RADIANT study, we screened 301 participants within five months’ timeframe between October 2019 and March 2020 during the pandemic of COVID. 106 eligible participants were randomised, and the adherence rate was 100%. By leveraging advanced REDCap features, including custom record status dashboards, automated alerts and notifications, text messaging integration via Twilio, and comprehensive project dashboards, we significantly enhanced the efficiency of the recruitment process and participant management. These improvements also effectively reduced the survey burden on participants, contributing to the overall success and robustness of our data collection methodology. In the SCUlpTOR study, we successfully completed the recruitment between May 2021 to Nov 2023 in two sites with screening of 10,110 participants and eventually 321 participants were randomised. The visualisation of the process provides an efficient system to adjust the recruitment strategy in a cost-effective and efficient manner (Figure 1).

Conclusion

This online trial implementation and data management system successfully work for RCT recruitment and conduction. The two well-performed trials prove the feasibility of online clinic trial model. This is particularly important in the post-COVID era when face-to-face interactions may be limited. However, the incorporation of technology and secure data management system in the implementation of RCT is still underexplored. Further validation is needed to generalize this method to a broader trial design in other medical disorders.

Background:

Statistics indicate that there are approximately 200 million individuals with hyperuricemia and 20 million people suffering from gout within my country. The general public is lack of awareness of the risks associated with gout. Many primary hospitals lack the capacity to correctly diagnose and treat gout, resulting in the inefficient use of national medical resources and significant distress and economic hardship for patients. Consequently, the expeditious and efficacious dissemination of gout science knowledge to the general public is of considerable social and economic consequence.

Methods:

The project team employs a rational integration of online platforms, newspapers, and other resources, in both online and offline modes, and promotes gout medical science knowledge through the utilization of new integrated media science popularisation models.

Results:

The project leader created the personal IP of “Dr. He Yi” with a cumulative fan base of over 2 million across the entire network. He was awarded one science popularisation project of the Guangdong Province Science and Technology Plan and two science popularisation projects of the Guangzhou City Science and Technology Plan. As the editor-in-chief, he published the popular science book “Dr. Tiantian Talks about Rheumatism-Gout”. As of May 2022, more than 600 popular science articles have been published, more than 200 popular science videos have been released, and 3 training and teaching videos have been recorded; the cumulative number of visits has exceeded 200 million times. Nearly 100 free clinics, science popularisation lectures, and grassroots doctor training have been conducted. The project leader was awarded several honours, including the “2023 Sohu Video Top Ten Broadcasters of the Year” accolade, the “2020 Chinese Medical Doctor Association Health Communication Working Committee n Kwai, Comprehensive Doctors and Science popularisation Contribution Award”, and the “2020 Baidu Health Live Broadcast Top Ten”. Additionally, the project was recognised with the “Guangdong Provincial Medical Science and Technology Award, Popular Science Award”.

Conclusion:

The project team proposed a novel model of “integrated media” for the popularisation of medical science. This model is not merely the integration of newspapers and online media, nor is it simply the integration of popular science articles, videos, and books. It also encompasses the integration of free clinics, popular science lectures, grassroots doctor training, MDT diagnosis and treatment teams, and new “three habitats” medical talent training. This model improves patients’ quality of life, reduces patients’ economic burden, conserves health care resources, and contributes to national health.

Background:

Sacroiliac joints (SIJ) radiography has lower sensitivity and specificity compared to magnetic resonance imaging (MRI) and low-dose CT (ldCT) of the SIJ for detecting axial spondyloarthritis. Whether this is also true for axial psoriatic arthritis (axPsA) remains uncertain. This study aimed to evaluate the performance of ldCT, MRI, and conventional radiography of SIJ and spine in distinguishing axial involvement in PsA patients.

Methods:

Fifty-one consecutive PsA patients (70.6% male, age 41 ± 13 years) meeting the CASPAR classification criteria, regardless of back pain, were included. All patients underwent SIJ and whole-spine radiography, SIJ MRI as well as SIJ ldCT, while 25 (49%) of 51 patients also underwent whole-spine MRI and ldCT. One rheumatologist with expertise in imaging and one trained reader evaluated images. The final diagnosis of axPsA was ascertained by two experienced rheumatologists. The sensitivity, specificity, and diagnostic accuracy of the three modalities for discriminating axPsA were compared.

Results:

AxPsA was diagnosed in 33.3% (17) of 51 patients. In discerning axial involvement in PsA patients, MRI exhibited superior sensitivity (92.9%) compared with ldCT (83.3%) and radiography (67.9%), while ldCT demonstrated higher specificity (97.1%) than MRI (86.4%) and radiography (72.7%). LdCT achieved the highest diagnostic accuracy (92%) among the three modalities, surpassing radiography (71%) and being comparable to MRI (89%). In the subgroup of patients with whole spine imaging, the combination of MRI and ldCT SIJ had the best diagnostic accuracy, reaching 93%. Including SIJ ldCT in addition to SIJ MRI further improved specificity (86.7% vs. 73.3%) with unchanged sensitivity (100%), while incorporating SIJ MRI alongside SIJ ldCT further improved sensitivity from 83% to 100%, with a modest decrease in specificity (86.7% vs. 93.3%). However, incorporating spine MRI/ldCT alongside SIJ MRI/ldCT did not further improve sensitivity or specificity in discriminating axial involvement in PsA patients.

Conclusion:

LdCT demonstrated superior diagnostic performance in distinguishing axial involvement in PsA patients compared to MRI or radiography, although MRI was more sensitive for detecting active sacroiliitis. The highest diagnostic performance was achieved with both MRI and ldCT of the SIJ. Additional spine imaging did not further increase diagnostic performance.

Background:

In patients with axial spondyloarthritis (AxSpA), we have reported that higher inflammatory burden as reflected by a longer disease duration, delay in diagnosis and higher erythrocyte sedimentation rate (ESR) levels were predictors associated with incident hypertension after adjusting for traditional cardiovascular (CV) risk factors (1). Whether this is due to accelerated arterial stiffness progression is worth exploring.

Methods:

One hundred patients with axSpA who fulfilled the ASAS classification criteria and with active disease (ASDAS $\ge$2.1) were recruited from the out-patient clinic of 6 regional hospitals. All participants will receive a 2-year protocolized treatment aiming to achieve ASDAS low disease activity (LDA) in order to address the effects of treating-to-target (T2T) and vascular outcomes (The Hong Kong AxSpA T2T vascular study). Medical history, medication used, disease activity, serum levels of inflammatory markers, cholesterol and fasting glucose were measured/recorded every 6 months. Brachial-ankle pulse wave velocity (baPWV) and carotid ultrasound were assessed at baseline and yearly. In this interim-analysis, we will focus on baPWV.

Results:

Ninety-two patients completed the month 12 follow-up. The patients were stratified into two groups based on the duration from symptom onset to diagnosis: less than 2 years (early axSpA group) and 2 years or more (non-early axSpA group). Table 1 summarized the demographic and clinical characteristics of both groups. The baseline baPWV values were comparable between the two groups (1412.4 ± 230.45 and 1325.19 ± 174.75, p > 0.05). However, at Month 12, there was a significant difference in baPWV between the groups (1470.77 ± 233.70 and 1302.09 ± 198.52, p= 0.001) (Figure 2). The early axSpA group showed a decrease of 23.09 cm/s, while the non-early axSpA group exhibited an increase of 59.85 cm/s (p=0.033). A multivariate linear regression analysis was conducted to predict the change in baPWV based on age, gender, early axSpA, use of csDMARDs, and glucocorticoids at baseline. Early axSpA remained the only significant predictor and was associated with a positive effect in reducing baPWV ($\beta$= -82.939; p= 0.033).

Conclusion:

Early diagnosis may be able to prevent progression of arterial stiffness in patients with axial SpA by lowering the cumulative inflammatory burden before effective suppression of inflammation could be given to the patients.

Background:

To identify contributors to non-traumatic incident fractures on rheumatic disease patients who are on long term glucocorticoids (LTGC)

Methods:

Two hundred and twenty patients on LTGC (110 with vertebral fracture and 110 without vertebral fracture) who participated in a cross-sectional study in 2014-2015 were invited to have repeated assessments on 1) aBMD using dual-energy X-ray absorptiometry (DXA), 2) volumetric BMD (vBMD), microstructure and bone strength assessment of the wrist and tibia using high-resolution peripheral quantitative computed tomography (HR-pQCT) and 3) spine radiographs in the 5th year. Clinical covariates were recorded on questionnaires, and Fracture Risk Assessment Tool (FRAX) score was calculated accordingly. Non-traumatic incident fracture over the 5-year were documented. Receiver operating characteristic curve (ROC) analysis was performed to compare the strength of fracture prediction tools.

Results:

Out of the 140 patients who completed the 5th year assessments, 47 (33.6%) developed incident fractures. History of previous fracture, aBMD at hip and lumbar spine, T-score, trabecular vBMD, trabecular bone volume fraction and estimated bone strength at the tibia at baseline remained significantly different after adjusting for age between the group with and without incident fractures. The area under curve (AUC) of a prediction model comprised of age, history of previous fracture and average trabecular vBMD at tibia was comparable with that of the FRAX score (0.710 vs 0.679-0.702), and slightly outperformed than the AUC of DXA aBMD at hip and lumbar spine (0.628-0.668) under ROC analysis.

Conclusion:

Age, history of previous fracture and average trabecular vBMD at tibia could be the main contributors in building a prediction model for non-traumatic incident fracture in rheumatic disease patients on LTGC.

Background

Data from open label follow-up were merely 5 years for secukinumab as second line treatment. Real world evidence from European and Korean cohort revealed retention rate up to 4 years. No data from China has been published. We aim to evauate the longterm retention rate of secukinumab in axial spondyloarthritis (axSpA) and its predisposing factors in Chinese patients.

Methods

Patients were recruited from retrospective medical records of two tertiary hospitals in China from January 2020 and March 2024. Patients were diagnosed with 2009 ASAS classification criteria. The retention rate of secukinumab was estimated using the Kaplan-Meier method, and the log-rank test was used to compare the retention curves of the two cohorts.

Results

In total, medical records of prescription for secukinumab and relevant clinical information from 445 patients were identified. The mean age was 34 (29$\sim$41) years with male gender of 77.30% and HLA-B27 posititivy of 88.54% in our cohort. Mean disease duration was 8 (5$\sim$13) years with relatively shorter duration in biologics naive compared with biologics experienced patients (7 (3$\sim$12) vs. 9 (6$\sim$14) years, p¡0.05). The secukinumab treatment period was 3-42 months, with majority of 150mg dosage. 16 patients (3.60%) initiated with 300mg loading dose. Smoking status and other clinical characteristics including concomitant treatment and comorbility were not significant between the two groups. Overall retention rate of our cohort during follow-up at 12-month, 24-month and 36-month was 0.750, 0.686 and 0.641, respectively. In subgroup analysis, biologics experience contributed to lower retention rate (Figure 1) and higher dose (300mg) treatment resulted in better retention rate (Figure 2). Discontinuation occurred in 35 patients mainly due to fertility demand. Switching because of inefficient response occurred in 58 patients. Adalimumab (n=24) was the preferred choice, followed by etanercept (n=16)., other DMARDs or NSAIDs (n=13) and JAKi (n=5).

Conclusion

Secukinumab had a good performance of retention in patients with axSpA from China, especially in those with higher dosage and without biologics experience.

Background:

Ankylosing spondylitis (AS) is a chronic disease characterized by inflammatory back pain, with osteoporosis prevalence ranging from 18.7% to 62%

1. Vertebral fractures are four times more likely to occur

2. Turner syndrome (TS) is the most common sex chromosome abnormality in females, with a 1.4- to 2.2-fold increased likelihood of osteoporosis-related fractures

3. Seven cases of AS combined with TS with HLA-B27 positivity have been reported4.

Methods:

We report a case of a female patient with AS and TS who exhibited an inadequate response to adalimumab (ADA) and suffered a thoracic vertebral fracture.

Results:

A 41-year-old woman with HLA-B27 negative was diagnosed with AS in 2012 because of low back pain, and began ADA treatment in 2018. The patient had a past history of TS (45X/47XXX) with ultrasound revealing smaller than normal ovaries and uterus. Body weight was 54kg and height was 145cm (BMI25.68). She had primary amenorrhea, a wrist fracture at a young age, and a thoracic spine fracture in 2018. Bone mineral density (BMD) values for orthotopic lumbar vertebrae L1-L4 were 0.74 g/cm², with Z-score of -2.7, and 0.564 g/cm² at the left femoral neck with Z-score of -2.4. The FRAX tool predicted a 10-year fracture risk of 2.4% for the occurrence of major osteoporotic fracture (MOF), and a fracture risk of 0.9% for hip fracture. She initiated hormone replacement therapy (HRT). In November 2022, she discontinued ADA due to COVID-19 infection. Laboratory tests identified slightly elevated inflammatory markers (Fig. 1), low trough drug level of 0.31ug/ml and positive anti-drug antibody. In February 2023, she slipped in a snowdrift and experienced increased back pain. Spinal MRI revealed osteoporosis with a T7 compression fracture and a new T9 fracture (Figure 2 A,B). Sacroiliac (SI) joints MRI showed significant improvement of inflammation (Fig. 2 C,D). Spine surgery consultation suspected fracture attributed to obvious kyphosis deformity and stress concentration during falls, resulting in buckling compression. Repeated test determined a BMD value of 0.920 g/cm² for orthotopic L3-L4 with a Z-score of -1.4, and a BMD value of 0.775 g/cm² for the left total hip with a Z-score of -1.2, the 10-year probability of MOF was 3.2%, and that of hip fracture was 0.5%. She was diagnosed with severe osteoporosis due to recurrent fracture despite of BMD improvement and anti-osteoporosis medications were postponed.

Conclusion:

This case further proved the dominant role of disease co-morbidity and insufficient treatment in the unfavorable outcome regardless of genetic predisposition.

Background:

Patients with adult-onset idiopathic inflammatory myopathy (IIM) were at risk of cancer. While the recently published international guideline on cancer screening suggested cancer risk stratification in patients with newly diagnosed IIM, the difference in clinical and serological risk profile among patients with cancer diagnosed before and after IIM diagnosis remained uncertain.

Methods:

MyoHK was a longitudinal observational cohort that collected data from patients with rheumatologist diagnosis of IIM from 8 rheumatology centres in Hong Kong. Data were reviewed from 2004-2023 for this study. Patients with unavailable myositis specific antibody (MSA) were excluded from analysis. Occurrence of cancer 3 years before to 3 years after the diagnosis of IIM were documented. Patients with active cancer 3 years before diagnosis of IIM were classified to “Prior cancer” group and patients with cancer diagnosed at or within 3 years after IIM diagnosis were classified to “subsequent cancer” group. Patients were stratified to high, intermediate and standard cancer risks. Risk profile between the “prior cancer” and “subsequent cancer” groups were compared.

Results:

501 patients were included in analysis. 82 patients had cancer within 3 years, in which 21 patients (25.6%) had cancer within 3 years preceding IIM diagnosis and 61 patients (74.4%) developed cancer at or within 3 years after IIM diagnosis. Nasopharyngeal, lung and breast cancers accounted for the top 3 cancers in both groups. In the “Prior cancer” group, patients were more likely to be female (85.7% vs 55.7%, p = 0.014) and were MSA-negative (33.3% vs 9.8%, p = 0.011). No patient in the “Prior cancer” group developed interstitial lung disease (0% vs 18.0%, p = 0.037) or fulfilled criteria for anti-synthetase syndrome (0% vs 13.1%, p = 0.062). Anti-TIF1g antibody was the most identified MSA in the two groups (42.9% vs 54.1%, p = 374). Myositis-associated-antibody (14.3% vs 42.6%, p = 0.019) and anti-Ro52 antibody (14.3% vs 37.7%, p = 0.047) were more common in the “subsequent cancer” group. In the overall cohort, 228 (45.5%), 246 (49.1%) and 27 (5.4%) of IIM patients were stratified into high, intermediate and standard cancer risk. Two-thirds of the “Prior cancer” group were stratified into high-risk while the remaining one-third being intermediate-risk, compared to 73.8% (p = 0.532) and 26.2% (p = 0.532) in the “subsequent cancer” group. Mortality between the two groups were comparable (38.1% vs 42.6%, p = 0.716).

Conclusion:

Compared to IIM patients with cancer detected at or after IIM diagnosis, patients with prior cancer were more likely to be female, negative for MSA and absence for ILD.

Background

Osteoarthritis (OA) is a leading cause of pain and disability in middle-aged or older adults worldwide. The prevalence of symptomatic knee OA has been increasing over the past several decades, indicating the need for the deployment of innovative prevention and treatment strategies for knee OA. The aim of this study is to investigate the efficacy and safety of an oral complementary medicine formulation in people with symptomatic knee OA.

Methods

This is a randomised, placebo-controlled, double-blinded (participants and investigators), two-arm, superiority, parallel, phase II trial. The study will recruit 82 ($\sim$41 per arm) community-based participants with knee OA living in Australia. Participants aged $\ge$ 40 years with a clinical diagnosis of symptomatic knee OA and radiographic change on X-ray (Kellgren-Lawrence Grade $\ge$ 2) will be recruited. Participants will be randomly allocated to receive either a complementary medicine formulation containing a daily dose of Boswellia serrata extract (Boswellin$Ⓡ$Super, 250 mg/day), pine bark extract (Fenoprolic™70 Organic 100mg/day), curcumin (500mg/day), piperine (5mg/day), and methylsulfonylmethane (MSM, 1500mg/day), or placebo, for 12 weeks. The primary endpoint is change from baseline in average index knee pain at 12 weeks (visual analogue scale). Secondary endpoints include change in knee pain at other timepoints, the Knee Injury and Osteoarthritis Outcome Score (KOOS), global assessment of disease activity, global rating of change, health-related quality of life (AQoL-8D), and adverse events.

Ethics, trial registration and dissemination:

The study protocol has been approved by the University of Sydney Human Research Ethics Committee (#2021/877). The trial has been prospectively registered on the Australian New Zealand Clinical Trials Registry (ACTRN12623000380695, 14/04/2023). Dissemination will occur through lay summaries, scientific publications, conference abstracts, poster or oral presentations, infographics, and theses.

Conclusion

The outcomes of this study will provide high-quality evidence guiding the clinical use of the complementary medicine combination and will inform updates on international OA clinical practice standards on the use of complementary medicines.

Introduction:

The potential association between Human papillomavirus (HPV) vaccination and the onset of rheumatoid arthritis (RA) and juvenile idiopathic arthritis (JIA) remains a pivotal point of exploration in immunization safety discourse.

Method:

This retrospective cohort study aims to ascertain the risk of RA and JIA following HPV vaccination, utilizing the TriNetX database, tracking the health trajectories of 32,165,650 females, from January 1, 2013, to December 31, 2022. By applying inclusion and exclusion criteria, we formed a balanced cohort of 344,165 HPV-vaccinated individuals, the propensity score-matched 1:1 with unvaccinated controls based on a range of demographic and clinical parameters. This study used the Cox proportional hazards model to analyze the risk of RA and JIA after HPV vaccination, expressed the results by adjusted hazard ratio (aHR) and 95% confidence interval (CI), and used Kaplan-Meier survival analysis.

Results:

The analysis showed that over a follow-up period of up to 10 years after vaccination, the risks of RA and JIA were significantly reduced in the HPV vaccination group, with aHR of 0.428 (95% CI: 0.372-0.492) and 0.310 (95% CI: 0.262-0.367) respectively. This trend persisted in a sensitivity analysis that replaced the control group with influenza-vaccinated individuals. Subgroup analyses showed that the risk of RA and JIA was significantly reduced in the 9-18 and 19-26 age groups, across ethnic backgrounds, and in the obesity and asthma subgroups, but still among those with a smoking history, the risk reduction was not statistically significant.

Conclusion:

Our findings highlight the significant protective effect of the HPV vaccine against RA and JIA. Further research is needed to delve deeper into this molecular mechanism.

Background

During the COVID-19 pandemic, 2 types of COVID-19 vaccines were made available in Hong Kong - the mRNA-based vaccine and the inactivated virus vaccine. All residents were required to complete 3 vaccinations. There is concern that immunogenicity acquired from COVID-19 vaccinations could be unsustainable in patients with inflammatory rheumatic disease (IRD) due to the underlying condition and immunosuppressive medication used. Therefore, this study aims to investigate the immunogenicity of COVID-19 vaccines and its sustainability in IRD patients.

Methods

This single-centre cohort was assembled prospectively from 8/2021 - 2/2022 in Hong Kong. Peripheral blood samples were collected from the patients before, 28 (± 3) days and 12 months after the third dose of COVID-19 vaccination. The neutralising antibody titre against the SARS-CoV-2 virus was quantified by ELISA.

Results

A total of 83 patients (age: 51.4± 11.7 years; 72.3% female; 17 rheumatoid arthritis, 17 psoriatic arthritis, 12 axial spondylarthritis, 37 systematic lupus erythematosus) attended all 3 visits. The vaccine distribution is summarized in Fig. 1A. Majority (96.4%) of patients had positive neutralising antibodies before the 3rd vaccine dose despite a low mean titre of 38.2± 29.7%. The antibody titre improved significantly after the 3rd dose (85.9± 23.8%) and was maintained at the 12-month visit (87.5± 22.6%). The mean antibody levels pre- and post-3rd dose were higher in patients who took mRNA-based vaccine (Fig. 1B). The presence of COVID-19 infection during the follow-up period led to significantly higher antibody titre at the 12-month visit (Table 1). There were no significant associations between immunogenicity and age, type of IRD, biologic/targeted Disease Modifying Anti-Rheumatic Drugs usage as well as additional booster doses.

Conclusion

3 doses of COVID-19 vaccine produced sustained humoral response in IRD patients. The immunogenicity appeared to be dependent on the type of vaccines received in the short term and breakthrough infection in longer term.

Background

Psoriatic arthritis (PsA) is characterized by a higher prevalence of metabolic syndrome and is associated with chronic inflammation, which increases the likelihood of developing cardiovascular disease. The aim of our study was to assess the differences of cardiovascular risk and clinical characteristics among psoriatic arthritis between the gender.

Methods

In this cross-sectional study, PsA patients who attended a single rheumatology referral center without cardiovascular disease (CVD) were recruited. CVD was defined as the occurrence of coronary artery disease or cerebrovascular ischemic disease events. This study utilized the atherosclerotic cardiovascular disease (ASCVD) score to predict the 10-year risk of atherosclerotic cardiovascular events, analyzing any differences or similarities in risk between male and female patients.

Results

We enrolled a total of 246 patients, comprising 108 males and 138 females. The mean age of the participants was 57 years (± 13.8) for males and 55 years (± 13.3) for females. Female patients exhibited significantly higher erythrocyte sedimentation rates and were more likely to be using two or more conventional synthetic disease-modifying antirheumatic drugs. Additionally, a significantly higher proportion of females had scalp involvement in their psoriasis compared to males (p $\le$ 0.05). Male patients with PsA had a lower ASCVD score (B=-1.008, 95% CI: -1.489, -0.526) compared to female PsA patients. However, there was no statistically significant association between gender and multiple domains of PsA.

Conclusion

The higher ASCVD scores in female PsA patients underscore the need for proactive cardiovascular risk management, including lifestyle interventions and pharmacological treatments, to mitigate the risk of future cardiovascular events. Further research is required to explore the underlying mechanisms driving these gender differences and to develop tailored strategies for the prevention and management of comorbidities in PsA patients.

Background

Herpes zoster (HZ), commonly known as shingles, is caused by the reactivation of the Varicella-zoster virus (VZV), the same virus that causes chickenpox. Approximately one-third of the population experiences shingles at least once in their lifetime. While age is the primary risk factor, immunocompromised individuals, such as patients with rheumatic diseases are particularly susceptible, especially those treated with Janus kinase inhibitors (JAKi). Real-world data from the Hong Kong Biologics Registry 2023 reported a herpes zoster infection rate of 3.49 per 100 patient-years among rheumatoid arthritis patients. This study aims to determine the rate of HZ infection among these patients and in particular, to assess their self-management strategies for HZ infection in a regional hospital.

Methods

This retrospective study involved patients diagnosed with rheumatoid arthritis, Spondyloarthritis and Psoriatic Arthritis, who were receiving JAKi treatments (Tofacitinib, Baricitinib and Upadacitinib). Participants were interviewed at the Rheumatology nurse clinic or by phone. A questionnaire gathered data on patient age, HZ vaccination status, history of HZ post JAKi initiation, and self-management plans for HZ infections.

Results

As of July 2024, a total number of 52 patients were interviewed. 33 (63.5%) had received the HZ vaccine prior to starting of JAKi, while 19 (36.5%) had not. 4 (7.7%) of them developed HZ after initiating treatment, with ages ranging from 46-84. 19 (36.5%) of them would seek medical advice if they have HZ infection, 7 (13.5%) would consult a doctor and consider withholding JAKi treatment. 3 (5.8%) would only stop the medication, 23 (44.2%) of them were uncertain about their actions.

Conclusion

This study demonstrates an increased risk of HZ infection among these patients and most importantly, highlights a critical gap in patient knowledge about HZ prevention and management. There is a clear need to enhance patient education regarding raising awareness of HZ risks, promoting vaccination, and providing effective self-management strategies.

Background:

In systemic lupus erythematosus (SLE), retinopathy is connected to severe disease and poorer prognosis. This study aimed to investigate changes in retinal vascular density (VD) in SLE and lupus nephritis (LN) patients and to examine the effects of Belimumab and hydroxychloroquine (HCQ) on retinal VD in these patients.

Methods:

This cross-sectional study enrolled 54 SLE patients (21 with LN and 33 without LN). A comprehensive ophthalmological evaluation using optical coherence tomography angiography (OCTA) was conducted on SLE patients. Rheumatological evaluations were performed through the collection of relevant clinical and laboratory data, as well as the assessment of rheumatological treatment.

Results:

We found that the mean VD in the superficial capillary plexus (SCP-VD) of most regions showed a significant decrease in the LN group. Further analysis revealed that LN patients using Belimumab exhibited a reduction in SCP-VD and deep capillary plexus VD (DCP-VD) (P<0.05). Furthermore, LN patients who had been using HCQ for more than 5 years exhibited a notable decrease in DCP-VD. Notably, SCP-VD and the cumulative dose of HCQ demonstrated a significant negative correlation.

Conclusion:

This study provides insights into the changes in retinal vascular density in LN patients and highlights the potential impact of long-term Belimumab and HCQ treatment on retinal microvascular damage. These findings contribute to the development of effective strategies for preventing and managing retinal complications in LN patients.

Background: There is a substantial expansion in the number of biologics available for the treatment of JIA and childhood rheumatic disease over the past decades. However, there is no report on their use and safety profile in the local paediatric population.

Method of study:

This is a multi-centred retrospective cohort study, recruiting patients followed up in three Paediatric departments in Hong Kong. Subjects who were followed up for childhood-onset rheumatic diseases and was treated with any of the biologic DMARs on or before the 31st December 2022, were reviewed.

Results:

A total of 96 patients who received biologic DMARs in the studied period were reviewed. Sixty patients were diagnosed to have JIA, cSLE (10), uveitis (8), refractory KD (7), JDM (2), COVID-MISC (3), and others (6). Concerning the JIA group, male: female is 1.4:1. 38% of the patients had polyarticular JIA and 50% had ERA who failed conventional treatment with various cDMARS. Concerning the nature of biologics, 85% of JIA patients used different types of TNF-blockers, including etanercept (38.3%), adalimumab (40%), golimumab (5%) and infliximab (3.3%). Six JIA patients used tocilizumab and one used abatacept as second-line treatment after fail response to TNF-blockers. One JIA patient was on tofacitinib to avoid subcutaneous injection. For other biologics, infliximab for refractory KD (8), rituximab for cSLE and JDM (7 and 1), belimumab in cSLE (2) and anakinra in COVID-MISC (4). One JIA-ERA patient developed lymphoma six months after stopping adalimumab and recovered after chemotherapy. One cSLE patient received rituximab and developed EBV-related CNS lymphoma with repeated infections that required hospitalization. Most patients tolerated the biologic DMARs well without complications. There was no tuberculosis infection or activation. Two cSLE patients developed herpes zoster after rituximab infusion and required hospital treatment. Two patients on adalimumab developed recurrent folliculitis and one had to stop treatment. One patient developed bacteria endophthalmitis while on adalimumab. Four patients on adalimumab developed injection site reactions and one patient need to change to another biologic. One patient on adalimumab developed positive lupus serology without clinical features.

Conclusion:

A broad spectrum of biologics has been used in children with various rheumatic diseases without major complications. Although rare, 2 patients developed lymphoma during the study period. Although there is no causal relationship identified with the use of biologics, continuous monitoring of the long-term safety of these drugs in the paediatric cohort is recommended.

Background

Cardiovascular disease is a significant complication of SLE patients, leading to high risk of morbidity and mortality. Atherosclerosis is a major contributor. Belimumab can reduce disease relapses and indicated to be atheroprotective. As a new biologic agent telitacicept targets both BLys and APRIL and inhibits both B cells and plasma cells. It may become a better option.

Methods

This case report describes a male patient with SLE combined with refractory lupus nephritis. Belimumab was given on the basis of corticosteroids and MMF. His condition was partially improved but recurrent palpitation occurred. Coronary artery atherosclerosis was confirmed. After switching from belimumab to telitacicept, palpitation disappeared and the patient’s condition gradually improved.

Results

A 40-year-old man presented recurrent fatigue for 8 years. He is a non-smoker, BMI 24.07kg/m², without family history of CAD. In October 2015, pancytopenia was found, with ANA, anti-dsDNA and U1-nRNP/Sm antibody positive. C3 decreased obviously. Coombs test was positive. 24h proteinuria was 11895 mg. SLE complicated with lupus nephritis was diagose and he was treated with pulse steroid, IVIG and CTX. Condition was improved, and he maitained steroid, MMF and hydroxychloroquine.

In May 2023, he stopped MMF himself for fear of COVID-19 infection. Soon lower limbs edema developed. C3 0.37g/L, C4 0.02g/L, dsDNA 675.3 IU/ml, 24h-P 8444 mg was documented. SLEDAI score was 12. Renal pathology indicated ISN/RPS 2003 Class IV-G(A)+V Lupus Nephritis. He received pulse steroid (MP 500 mg per day) for 3 days, belimumab 720mg once and with MMF 750mg twice per day. Belimumab was regularly given after disease was controlled. Proteinuria decreased and remained about 3g.

In August,2023, the patient began to feel palpitation repeatedly. LDH, HBDH and LDL increased. Holter showed sinus rhythm, accidental supraventricular premature beat, but no ST-T change. Coronary CTA showed non-calcified plaques in local section of RCA and the proximal section of branch of LAD, with slight stenosis. Rosuvastatin was prescribed. But palpitation reoccured after belimumab was given. Belimumab was stopped and changed to telitacicept 160mg every 4 weeks from 12 November, 2023. Till now, palpitation doesn’t occure. Prednisolone was tapered down to 7.5mg per day and MMF maintained 750mg twice a day. Complements rised. Proteinuria decreased to 1164.34mg.

Conclusion

Telitacicept is supposed to be a choice for refractory active SLE patient with atherosclerosis who has poor response to conventional therapy.

Background There is a increasing suggestion that upadacitinib may elevate the risk of infections in patients with rheumatoid arthritis (RA). To better understand this potential infection risk, we conducted a meta-analysis using data from randomized clinical trials (RCTs) to evaluate the overall risk of infections in these patients.

Methods We conducted a comprehensive systematic search in EMBASE, MEDLINE, and CENTRAL from their inception until 31st December 2023 to identify relevant RCTs reporting the occurrence of infections in patients with RA who were treated with monotherapy upadacitinib (15mg once daily orally) regardless of the background RA therapy used in these patients. The primary outcomes were the incidence of total infections, serious infections, non-serious infections, and opportunistic infections (including herpes zoster and tuberculosis). The secondary outcomes were the incidence of various sites of infections, including the upper respiratory tract, lower respiratory tract, gastrointestinal tract and others. In anticipation of study heterogeneity, the effect estimate was pooled with a random-effects model, to obtain the risk ratios (RR) and 95% confidence intervals (CI), using Mantel-Haenszel statistical method.

Results Nine RCTs were included, involving a total of 4,575 RA patients. Compared to the control group (placebo and other active treatments), patients treated with upadacitinib exhibited a significant increase in the risk of total infections (RR, 1.18; 95% CI, 1.02-1.36; I2, 49%), and non-serious infections (RR, 1.16; 95% CI, 1.01-1.34; I2, 46%). Patients who were on upadacitinib were significantly at an increased risk of gastrointestinal infections (RR, 3.10; 95% CI, 1.47-6.56; I2, 0%) but not on other types of infections.

Conclusion Compared with placebo and other active treatments, upadacitinib signi?cantly increased the overall risk of infections (total infections and non-serious infections), particularly gastrointestinal infections. These ?ndings may assist clinicians to weigh the benefit-risk balance of upadacitinib prior to their administration to patients with RA.

Background Rheumatological disease induced shoulder pain could affect most of the ADLs and work requiring shoulder height and upper arm rotation such as combing hair, pulling pants up, and reaching for something in a cabinet. Pain affects the stability of hands making difficulties in using chopsticks and make-up. Pain also affects sleep quality and psychological factors such as stress and depression.

Methods Occupational therapy (OT) aimed to alleviate pain, prevent further deterioration, promote independence in ADL, work, and leisure by means of fascia therapy, mobilization and strengthening; training on proper body mechanics; fabricating splinting to enhance structural support; prescription of aids and gadgets; and self-management strategies.

Results

During flare up, OT educate patient to minimize stress on shoulder joint, such as inflammatory self-management and gentle stretch of surrounding muscles, mobilization, and proper body mechanics in ADLs. Aggressive stretching should be avoided. Specific joint protection techniques to avoid extra stress on shoulder during their ADL and work includes proper head and neck position with supporting devices; avoid side lying on the painful side when sleeping etc. In addition, avoid over generalization of basic joint protection techniques, e.g., carrying heavy bags on forearm may increase loading to the shoulder; while using small wheeled-trolley to carry heavy things in damping road may cause impact to shoulder. Instead, using small towel to minimize rotational force on shoulder; and use upper body part to push heavy door in shopping centre to minimize the impact loading on shoulder joint, etc. On the other hand, training on basic relaxation techniques like breathing focus or progressive muscle relaxation could allow relieve pain. For muscle strengthening, muscle balance must be the basic consideration by means of pendulum exercise, and progress to isometric and eccentric training. Strengthen core and upper back muscles should be included to provide basic stability.

Conclusion Patient with severe shoulder instability but not fit to receive surgery, could result in pain and functional deficit. Thus, patient is encouraged to compile on different bracing, e.g., AC joint brace or shoulder sling for certain period to optimize joint stability and pain. Alternative ADL and work strategies e.g., lowering the working area or using safety ladder; and utilizing some aids e.g., long-handle reacher, and electrical screw driver, could promote their independence. Advanced technology could also be employed like exo-skeleton device promote patient’s function independence ADLs and work independence.

Systemic lupus erythematosus (SLE) is often related to a lower rate of live birth and a higher incidence of adverse pregnancy outcomes (APOs), and pregnancy can lead to an increased risk of SLE flares. Comprehensive preconception assessment is crucial, but global consensus regarding the criteria the patients should meet before pregnancy has not been formed yet. This study, conducted retrospectively based on data from Chinese SLE Treatment and Research group (CSTAR) registry, a multicenter Chinese SLE cohort, aimed to investigate the optimal criteria. Analysis included 347 singleton pregnancies from 333 patients with SLE in total. The criteria that patients with SLE should meet before pregnancy were identified by univariate logistic regression analysis, which were: 1) disease stable for at least 6 months; 2) absent of vital organ damage; 3) on nonfluorinated corticosteroids no more than the dose equivalent to prednisone 7.5mg per day; 4) on hydroxychloroquine. The proportion of live birth was significantly higher in the group meeting the criteria (86.1% vs 73.7%, p=0.004). Furthermore, the gestational week at delivery (38.4 vs 37.6, p<0.001) and the birthweight (2956.7g vs 2810.2g, p=0.004) were significantly larger. Then, APOs occurred less frequently in the group meeting the criteria (29.4% vs 52.1%, p<0.001). For each type of APOs, the incidences of therapeutic abortion (5.0% vs 12.0%, p=0.019), preterm delivery (14.2% vs 33.3%, p<0.001), and preeclampsia (1.1% vs 5.4%, p=0.023) were remarkably lower. When it comes to disease flares, patients in group meeting the criteria experienced flares during pregnancy less frequently (14.7% vs 28.1%, p=0.005), as well as for severe flares (5.8% vs 15.6%, p=0.006). Results were similar when taking both gestation period and one year postpartum into consideration. The results of this study stress the importance of comprehensive preconception assessment and warrant the implementation of such criteria among patients with SLE in clinical practice.

A 60-year-old married male farmer with a significant medical history, including STElevation Myocardial Infarction (STEMI), coronary artery bypass grafting (CABG), and Raynaud’s disease, presented to the emergency department with sudden-onset syncope and progressive shortness of breath on exertion. The syncope episode involved an abrupt loss of consciousness, with spontaneous recovery and no associated symptoms like jerky movements or tongue biting. The shortness of breath had been gradually worsening over several weeks, becoming noticeable even during minimal exertion.

On examination, the patient appeared chronically ill but had stable vital signs cardiovascular examination revealed bilateral pedal edema, while respiratory assessment and Neurological evaluation were normal. Blood tests indicated normal full blood count (FBC) and urea and electrolytes (U+E), but slight elevations in CRP 29 mg/L and ESR 20 mm/hr. Troponin levels remained stable, but brain natriuretic peptide (BNP) was markedly elevated at 2061 pg/mL. The electrocardiogram (ECG) showed right axis deviation and incomplete right bundle branch block (RBBB). A CT scan of the brain ruled out any acute pathology, such as stroke or hemorrhage. A previous echocardiogram (ECHO) had shown a left ventricular ejection fraction (LVEF) of 50-55%, indicating good left ventricular function. Given the findings, the patient was started on Furosemide 40 mg daily and advised to follow a fluid restriction regimen to prevent worsening of overload symptoms.

A repeat echocardiogram revealed deterioration in right ventricular (RV) function with a significantly (RVSP) of 80 mmHg, suggesting severe pulmonary hypertension (PHTN). A D-dimer test was negative for pulmonary embolism (PE). Cardiology consultation raised concerns about possible chronic thromboembolic disease, leading to further testing, including a computed tomography pulmonary angiogram (CTPA) and an autoimmune screen, along with potential right heart catheterization (RHC). The CTPA was negative for PE, but the autoimmune screen returned positive results for antinuclear antibodies (ANA >1200) and anticentromere antibodies. Right heart catheterization confirmed pre-capillary pulmonary hypertension (PAH) with pulmonary artery pressure exceeding 44 mmHg and no nitrate response. A rheumatology review, in light of the patient’s history of Raynaud’s disease, positive ANA, and confirmed PAH, led to the diagnosis of Systemic Sclerosis Sine Scleroderma–a form of systemic sclerosis without skin involvement. The patient was initiated on Tadalafil, (PDE-5) for PAH management and prescribed home oxygen therapy, particularly during exertion. He was discharged with a plan for multidisciplinary followup involving rheumatology, cardiology, and pulmonology to manage his complex condition.

Conclusion: Multidisciplinary is essential for optimizing outcomes in SSc and PAH.

Background Takayasu arteritis (TA) is an idiopathic, granulomatous, large-vessel arteritis that predominantly involves the aorta, its major branch arteries. Then objective is to evaluate the clinical features, angiographic findings and response of treatment

Method 12 patients with TA were studied at the NCRD between 2018 and 2023 and were followed for 3 months to 5 years. Data on clinical features, angiographic and laboratory findings, disease course, and response to therapy were all recorded and stored in a computer-based retrieval system.

Result TA was more common in females (54%). The mean age at disease onset was 37.08± 16.91 years. The clinical presentation ranged from asymptomatic to catastrophic with uncontrolled hypertension, gradual loss of vision. The most common clinical finding was a fatigue and dizziness. Hypertension was most often associated with renal artery stenosis (4 out of 12). Almost 50% of the patients had systemic symptoms on presentation. Medical therapy was required for all of the patients. Immunosuppressive treatment with glucocorticoids helped achieve remission (ITAS-0) whereas remaining had decrease in disease activity with one reported mortality.

Conclusion In Nepal, Takayasu arteritis is a rare disease. It is heterogeneous in presentation, progression, and response to therapy. Although mortality was low, substantial morbidity occurred in most patients.

Background:

Systemic lupus erythematosus (SLE) is a multisystem autoimmune disorder mainly affecting women of childbearing age. Mortality and organ damage of SLE is mainly due to uncontrolled disease activity and treatment-related toxicities. Thus, assessment of disease activity is important during routine clinical practice. In recent years, neutrophil-to-lymphocyte ratio (NLR) has been explored as a marker of disease in many autoimmune disorders including SLE. As such, our study objective is to investigate the correlation between neutrophil-to-lymphocyte ratio (NLR) and disease activity of SLE using the SLE disease activity score (SLE-DAS) in our local population.

Methods:

Consecutive adult patients who fulfilled the ACR or SLICC criteria for SLE were recruited between March 2023 and February 2024. SLE activity was assessed by SLE-DAS, physicians’ global assessment (PGA) and SLE disease activity index-2000 (SLEDAI-2K). The calculated NLR was correlated with disease activity indices and serological parameters (anti-dsDNA, C3/4) by Spearman’s rank correlation. Patients were stratified into remission, low and moderate/severe disease activity as defined by the SLE-DAS and comparison of the NLR was performed among these subgroups by one-way ANOVA.

Results:

A total of 420 SLE patients were studied (96.2% women, age 46.0± 11.0 years, SLE duration 16.4± 8.3 years). Moderate/severe, low disease activity and SLE-DAS remission was present in 70(16.7%), 66(15.7%), and 284(67.6%) patients, respectively. SLE-DAS correlated significantly with SLEDAI-2K (rho 0.9; p<0.001) and PGA (Rho 0.60; p<0.001). The mean NLR of all patients was 3.54± 4.0 and NLR correlated significantly with SLE-DAS (Rho 0.17; p<0.001). The NLR was significantly higher in active renal (urine P/Cr >0.5, active sediments or histological features) than inactive SLE (4.62 vs 3.27; p=0.02). The NLR in patients with moderate/severe SLE-DAS activity was significantly higher than those in remission (4.87 vs 3.27; p=0.007). ROC analysis showed that a NLR cut-off of 3.11 showed a sensitivity of 55.6% and specificity of 68.7% in detecting moderate/severe SLE-DAS (AUC 0.67[0.6-0.7]; p<0.001).

Conclusion:

The NLR is a satisfactory and convenient marker that correlates significantly with disease activity in SLE.

Background

Conventional disease modifying anti-rheumatic drugs (DMARDs) are the first line drugs in a conventional DMARDs in Pakistan.

Methods and materials

This prospective cohort study was conducted in rheumatology department of FFH Rawalpindi from October 2023 till March 2024. 224 cases of treatment naïve early RA, diagnosed on the basis of ACR2010 criteria, were included. Patients were started on Methotrexate or other conventional. Baseline daa were collected for demographic, clinical parameters including age, gender, smoking, BMI, RA duration and serology. Disease activity was assessed by DAS28 CRP at 3 months for assessing the response to treatment. Correlations and comparisons were performed by Pearson’s correlation coefficient and independent sample t-test. Statistically significance p value was 0.05.

Results

Females constituted19six (87.5%) and males 22(12.5%) patients. 130 patients were seropositive, 94 were seronegative. 13 patients were smokers(..%). Radiographic damage was present in 67 patients. Mean DAS28 of the study population was 3.05± 89. RA duration was 11.07± 10.70 years. BMI was 27.70± 22.48kg/m². Table I and II show the results of correlation and comparison of the clinical features with respect to disease activity score.

Conclusion

Patients with higher BMI has poorer response to conventional DMARDs. Weight optimization may be important step in improving the response to DMARDs in patients with RA.

Background:

LUNAR trial failed in lupus nephritis. However case series across the globe have shown good outcome with rituximab in lupus nephritis. So we evaluated the efficacy and safety of rituximab in lupus nephritis in Indian patients

Methods:

This was single centre, retrospective study done in patients of lupus nephritis receiving rituximab between 2013 to 2022. All patients fulfilled the SLICC criteria. Rituximab was given if there was a flare with background immunosuppression or upfront for early steroid tapering. Patients were followed up at 3, 6 and 12 months. Complete kidney Response (CR) is defined as return of serum creatinine(Scr) to previous level with a decline in 24 hour urine protein to <500 mg at 12 months. (1) Partial kidney response (PR) was defined as an improvement of Scr, but not to the standardized normal, with at least 50% decline in the 24 hour urine protein achieved preferably by 6 months and no later than 12 months following treatment initiation.

Results:

Out of 125 patients who were included for analysis, CR and PR were achieved in 79(63%) and 18(14%) patients respectively at 12 months. Mean steroid dose in prednisolone equivalent was less than 10 mg at 6 months in all patients. Improvement in parameters like dsDNA, c3, c4 were observed. In follow up 14 patients (11%) developed Chronic kidney disease (CKD), 6 patients (4.8%) required dialysis and 8 patients (6.4%) expired. Subgroup analysis between patients who received rituximab upfront and those after treatment failure or relapse didn’t show statistical difference in results including CR,PR,CKD, dialysis requirement or death.

Conclusions:

These results indicate the possible efficacy and safety of rituximab based regimen with its potential to reduce the steroid requirement as well as achieving remission in treatment naïve and in patients who are refractory or relapse with standard agents in Indian Subcontinent.

Background

This study aimed to ascertain the effect of secukinumab on erosion and enthesiophyte progression in psoriatic arthritis (PsA) by high-resolution peripheral quantitative computed tomography (HR-pQCT).

Methods

This was a one-year double-blind, randomized, placebo-controlled trial (NCT0362386740). Patients with erosion in the metacarpophalangeal joints (MCPJ) 2-4 were randomised in a 1:1 ratio to either the secukinumab or placebo group. HR-pQCT of the MCPJ 2-4 were performed at baseline, week-24 and 1-year. Progression of enthesiophyte were defined as changes in enthesiophyte volume exceeding smallest detectable change (SDC) (0.12mm³) or the identification of any new enthesiophyte. Partial repair of erosion was defined as a reduction in erosion volume greater than SDC (0.1mm³).

Results

Forty patients (age: 51.9± 13.4 years, 20 [50%] male) were recruited. Thirty-four patients who completed study treatment were included in the per-protocol analysis. The erosion volume at baseline, week-24 and week-48 revealed significant reduction in the secukinumab group while no differences in the placebo group (Figure-3A). There was a trend suggesting that fewer patients developed new-erosions in the secukinumab group (one-erosion in one-patient) compared to the placebo group (six-erosions in five-patients) (p=0.078) (Figure-3B). A significantly higher proportion of erosions with partial healing was observed in the secukinumab group compared with the placebo group [51%vs30%, p=0.029] (Figure-3C). Regarding enthesiophyte, a total of 25-enthesiophytes were identified in both the secukinumab and placebo groups at baseline. The enthesiophyte volume at baseline, week-24 and week-48 revealed significant differences in the secukinumab group while no differences in the placebo group (Figure-3D). While one (one-enthesiophyte in one-patient) and four-enthesiophytes (four-enthesiophytes in three-patients) were newly identified in the secukinumab group and placebo group respectively (Figure-3E), the proportion of enthesiophyte progression was numerically higher in the placebo group than the secukinumab group [40%vs16%, p=0.114] at week 48 (Figure-3F). GEE results showed that the odds ratio (OR) for enthesiophyte progression in the secukinumab group was 0.264 (95% CI: 0.080-0.878, p=0.030), while the OR for partial erosion healing in the secukinumab group was 2.816 (95% CI: 1.109 to 7.153, p=0.029), adjusting for tender-joint-counts.

Conclusions

Secukinumab demonstrates a potential benefit in facilitating partial erosion repair and preventing enthesiophyte progression in PsA.

Introduction

Rheumatoid Arthritis (RA) is a chronic autoimmune disease that causes joint inflammation and potential disability. (1). The relationship between comorbidities and RA is well-known and is associated with poor outcomes. (3,4, 5) Furthermore, patients with RA are at an increased risk of cardiovascular disease (CVD). (6) This study investigates the epidemiology of RA in Kuwait, focusing on the prevalence of comorbidities such as hypertension, type 2 diabetes, hyperlipidemia and obesity using the Kuwait Rheumatoid Arthritis registry.

Methods

This retrospective study analyzes data from 2239 RA patients in Kuwait, enrolled from February 2012 to May 2024

Results

As shown in Table 1, (71.0%) are females with a mean age of 55.6 years and a mean disease duration of 11.1 years. The average body mass index (BMI) is 29.2, with 39.2%% having a BMIˆ3 of 30 and 11.3% are active smokers.

Analysis of comorbidities in RA patients shows that hypertension (23%) and diabetes Type 2 (20%) are the most prevalent conditions, followed by thyroid disease and hyperlipidemia (13%), as demonstrated in Figure 1.

Conclusion

This study reveals a high prevalence of hypertension, diabetes, dyslipidemia and obesity, all of which are modifiable risk factors for CVD. This finding emphasizes the importance of pre-emptive screening for these comorbid conditions, establishing a comprehensive care plan, and optimizing treatment protocol to improve patient outcome.

Introduction

Rheumatoid arthritis (RA) is a chronic autoimmune disease affecting females. In RA, the treat to target strategy necessitates an early and intensive approach to achieve disease control. (1, 2) However, the therapeutic options can adversely affect reproductive health and pregnancy. Hence, the age of female patients at presentation might be a factor in decision making; although, there is a scarcity of evidence to support this hypothesis. We analyzed prescribing trends in Kuwait to examine the effect of female age on initial treatment decision.

Methods

Retrospective analysis of patients enrolled in the Kuwait RA Registry from 2012 to 2024. Baseline characteristics and medication prescribed at the initial visit were analyzed based on age group.

Results

1101 female patients were included and stratified into two age groups $\le$49 years and >49 years. 30.8% were $\le$49 years and had a higher proportion of rheumatoid factor (RF) n Anti-CCP positivity (82.4% vs 75.5%, p=0.087). There was no significant difference in the mean DAS-28 at baseline (3.2 vs 3.1, p=0.32).

A numerically higher proportion of patients started methotrexate (MTX) in the >49 years group (59.4% vs 54%, p=0.090). No statistically significant difference was found between the groups in sulfasalazine (SSZ), hydroxychloroquine (HCQ), or leflunomide (Lef) use. Rituximab was the most prescribed biologic with no statistical difference between the groups in prescription rate (p=0.822); TNF inhibitors (TNFi) were prescribed more often in the >49 years group (16.5% vs 11%, p=0.022); adalimumab, highest TNFi, prescription was not statistically different between the groups; Etanercept was prescribed more often the >49 years group (7.7% vs 4.7% and 5% vs 1.2%) respectively. There was no statistical difference between the groups in monotherapy vs. combination therapy treatment strategy.

Discussion & Conclusion

This study showed that age at presentation does not significantly influence the initial choice of Disease-Modifying Anti-Rheumatic Drugs in female RA patients in Kuwait. Other factors, such as completion of family and patient preferences, likely play a more critical role in treatment decisions. Further research is needed to explore these influences and optimize management strategies for RA in women, particularly concerning reproductive health.

Background:

Antinuclear antibodies (ANA) are often used in diagnosing autoimmune rheumatological diseases, but their behavior in response to biological therapies, particularly in Asian patients, is not well-documented. The impact of these therapies on ANA status is crucial for understanding disease management and treatment efficacy.

This study aims to evaluate the prevalence and significance of ANA positivity following the administration of biological agents in patients with autoimmune rheumatological diseases.

Methods:

This pilot study was a prospective cohort study involving 100 patients with autoimmune rheumatological diseases who were initially ANA negative at baseline. Predominantly included patients were axial spondyloarthritis (AxSpA) (n=95) and ANCA vasculitis (n=5). Patients with AxSpA were diagnosed according to the 2009 ASAS criteria, and treated with biological agents (e.g., TNF inhibitors such as adalimumab, etanercept, or IL-17 inhibitors such as secukinumab) and followed at a tertiary care center in India from January 2021 to December 2023. ANA levels were measured before and after 3, 6, 9, or 12 months of biological therapy. Changes in ANA positivity, clinical outcomes, and correlations with disease activity were analyzed.

Results:

Out of the 100 patients enrolled, 67 (67%) completed the study. All participants were ANA negative at baseline. After biological therapy, ANA positivity developed in 4 patients (5.9%). Despite this increase in Ana positivity, there was no significant correlation between ANA positivity and clinical disease activity, as measured by the Ankylosing Spondylitis Disease Activity Score (ASDAS) and the visual analog scale (VAS) for pain, BVAS suggesting effective therapeutic response despite changes in ANA status. Additionally, 30 patients were lost to follow-up, highlighting challenges related to the financial sustainability of biological therapy in the resource limited countries.

Conclusion:

Biological therapies for AxSpA are associated with an increase in ANA positivity although this change does not appear to impact clinical disease activity. The rise in ANA titres may reflect disease-related changes or treatment effects rather than a direct correlation with therapeutic efficacy. Further longitudinal studies are needed to understand the clinical significance of these findings and to refine the interpretation of ANA results during biological therapy.

Background: SLE is a chronic heterogenous systemic autoimmune disease associated with significant morbidity and poor health related quality of life. Chronic sleep disturbances have been found to have detrimental effects on health and functioning. In this study we aim to evaluate and compare the sleep quality in SLE patients vs. healthy controls using validated sleep questionnaires in the Indian population.

Methods: We assessed sleep quality in enrolled SLE patients and age-sex matched controls using Pittsburgh Sleep Quality Index (PSQI) and Epworth Sleepiness score. Data on demographics, quality of life (QoL) and functional disability was collected using the WHO QoL Brief version and Health assessment Questionnaire (HAQ)-CRD version. Disease severity i.e. activity and damage scores were recorded during clinical examination.

Results: 90 cases and 90 controls were enrolled in this study. In cases, the mean age was 37.11 ± 13.01 years with 90% females, average disease duration of 7.12 ± 6.65 years, SLEDAI-2k of 4.01± 3.72 (i.e. mild disease) and SLICC-DI of 0.40± 0.76 (Table 1). 72.2% cases had Musculo-skeletal involvement, 50% has mucocutaneous, 30% had nephritis and 25.6% had hematological abnormalities as primary complaints. Few cases had serositis (5.6%), gangrene (2.2%), anti-phospholipid syndrome (3.3%) and pulmonary involvement (3.3%). Cases demonstrated worse sleep quality than controls with a mean PSQI of 7.59± 3.33 vs 4.52± 2.90 respectively (p$<$0.001). 82.2% (n=74) cases had PSQI score $>$4. Cases also had poorer PSQI component scores i.e. quality, latency, disturbances, medication use and daytime dysfunction (p$<$0.001). Epworth sleepiness score was significantly higher in the SLE group (4.68± 3.31 vs 2.19± 2.55) (Table 2). Poor sleep quality was associated with lower WHO QoL domain scores and higher HAQ scores (p$<$0.001). Significant association was found between disease activity (SLEDAI-2K) and PSQI total score (p=0.027), but no such correlation was found with organ involvement or total damage.

Conclusion: Sleep quality was significantly worse in SLE patients than controls. This was associated with overall poor quality of life and physical function. Given that sleep is multifactorial; symptoms such as fatigue, myalgia, joint pains and rashes significantly affect sleep but maybe missed on standard disease indices. Poor sleep translates into poor general health which the patient may experience despite controlled disease.

Background

Inflammatory bowel disease (IBD) is a significant component of the global disease burden, particularly among adolescents and young adults. This study aimed to investigate the patterns and trends of IBD among adolescents from 1990 to 2021.

Methods

We retrieved data on the incidence and disability-adjusted life years (DALYs) of IBD in adolescents (aged 15-39 years) from the Global Burden of Disease (GBD) 2021 study, Temporal trends of incidence and DALYs were calculated using the average annual percent change (AAPC). The correlation between age-specific incidence rates and DALYs rates with the Sociodemographic Index (SDI) was performed using Spearman’s correlation analysis.

Results

From 1990 to 2021, age-specific incidence rates significantly increased while age-specific DALYs rates declined [AAPC: 0.22vs-0.16]. All SDI regions saw a decline in age-specific incidence rates while age-specific DALYs rates in adolescent showed an increasing trend in low and middle SDI regions but a-decrease in high, high-middle and low-middle SDI regions. Incidence rates rose from 1990 to 2021 in 21-GBD regions except for high-income North America. In contrast, DALYs rates increased in regions such as Western Sub-Saharan Africa, Central Latin America, Tropical Latin America, Australasia, Eastern Sub-Saharan Africa, Central Sub-Saharan Africa, Central Asia, and North Africa and the Middle East, while they declined in the remaining 21-GBD regions. Among the 204-countries, China exhibited the fastest-growing age-specific incidence rate among adolescents [AAPC: 2.60], followed by Libya (AAPC: 2.59) and Oman (AAPC: 1.56), while Italy showed the fastest decline [AAPC: -0.76] (Figure-1A). Mauritius experienced the fastest growth in age-specific DALYs rates among adolescents (AAPC: 1.93), followed by Libya (AAPC: 1.79) and Mexico (AAPC: 1.45), while Northern Mariana Islands saw the fastest decline (AAPC: -3.28) (Figure-1B). Moreover, the AAPC of age-specific incidence rates for adolescent IBD exhibited a slight decreasing trend with increasing SDI (Figure-1C), and the AAPC of DALYs rates significantly declined with increasing SDI (Figure-1D).

Conclusion

The regional disparities in the incidence and DALYs rates of adolescent IBD highlight the urgent need for innovative prevention and healthcare strategies to alleviate the global burden of IBD among adolescents. Early screening could be crucial in mitigating the impact of IBD on adolescents.

Objective:

1. To evaluate the frequency of the various lesions in distal interphalangeal joints of patients with psoriatic arthritis by clinical and ultrasound assessment. 2. To determine the correlation between grayscale and Power Doppler ultrasonography score with disease activity score. Methods: This cross-sectional study was performed on 41 patients, who were diagnosed with psoriatic arthritis by CASPAR 2006 criteria. Results: Ultrasound detected more joint lesions than clinical examination. The proportion of patients with clinical distal interphalangeal arthritis was 12,2%. 100% of patients had at least one distal interphalangeal arthritis by ultrasound, and 27,6% of patients had periarticular soft tissue lesions. The frequency of various ultrasound abnormalities was as follows: Synovial hypertrophy was seen in 55,9%, Power Doppler abnormality suggesting hypervascularity in 52,5%. The periarticular lesion frequency: extensor tendonitis in 6,1%; loss of normal fibrillary architecture of extensor tendon erosions was seen in 14,4%; flexor tenositis in 7,1%; and other lesions were seen in 32,8% of patients. Significant correlation was found between DAPSA score and grayscale joint count (GSJC) (Spearman’s R=0,55; p$<$0,001), grayscale joint score (GSJS) (R=0,62; P<0,001), Power Doppler joint count (PDJC) (R=0.68; p$<$0,001), and Power Doppler joint score (PDJS) (R=78; p$<$0,001). Conclusion: Ultrasound can detect more joint lesions than clinical assessment. The severity of lesions on ultrasound correlates with the disease activity score.

Background: Eosinophilic granulomatosis with polyangiitis is a rare clinical condition with multiple organ involvement. We report a case of a 64-year-old male patient who presented with symptoms of chronic rhinosinusitis, numbness, and pain in both lower limbs, weight loss of 6 kg/3 months, and subcutaneous vasculitis lesions along the forearms and legs on both sides. The patient had peripheral eosinophilia with an eosinophil count of 11.6%, eosinophil count of 1.56G/l, positive pANCA test, and electromyography results showing damage to the left calf nerve, bilateral superficial peroneal nerve, and left deep peroneal nerve with axonal loss. Biopsy results of calf muscle showed chronic granulomatous vasculitis. The patient was diagnosed with eosinophilic granulomatosis with polyangiitis (EGPA) according to the ACR/EULAR 2022 criteria. The patient was given intravenous Methylprednisolone, followed by maintenance treatment with reduced doses of Corticosteroids and Methotrexate. The patient’s leg pain was significantly reduced, numbness in both lower limbs was reduced, and the patient’s quality of life was improved. Methods: clinical case report. Results: The patient’s peripheral polyneuropathy and subcutaneous vasculitis were significantly reduced after treatment with Corticosteroids and Methotrexate. Conclusion: Eosinophilic granulomatosis with polyangiitis is a rare clinical condition with manifestations of multiple organ damage. Timely detection and diagnosis help increase treatment effectiveness and improve clinical symptoms for patients.

Background: Axial Spondyloarthritis (AxSpA) is a chronic inflammatory condition which impacts various aspects of patients’ lives, including work productivity. This study aims to identify the impact of AxSpA on key work outcomes including sick leave rate, disease related unemployment rate, work productivity loss and its association with disease activity.

Methods:

62 Adult patients aged ¿20 years who met the Assessment in Spondyloarthritis International Society (ASAS) 2009 criteria for AxSpA were enrolled. Work productivity was assessed by the Work Productivity and Activity Impairment scale (WPAI:SpA). The validated Sinhala version of WPAI questionnaire was used to obtain data and calculate absenteeism, presenteeism and work productivity loss (WPL). Higher scores for presenteeism, absenteeism, or WPL indicate greater work impairment due to illness. Disease activity was assessed by Ankylosing Spondylitis Disease Activity Score (ASDAS). Multivariate linear regression was utilized to assess the association between work productivity and disease activity.

Results:

Among 62 patients who were on paid employment, permanent loss of employment rate due to disease was 20.9% and disease related sick leave rate for $>$ 2 months was 58.06%. Out of them 48% engaged in heavy labor jobs and 65% had high or very high disease activity. (Figure 1) Among employed patients, mean Absenteeism, Presenteeism and WPL were 22%, 51.04% and 56% respectively. A decrease in work productivity was related to an increase in disease activity. Disease activity was strongly correlated with absenteeism (p $<$ 0.01), presenteeism (p $<$ 0.01) and work productivity loss (p $<$ 0.001). (Figure 2)

Conclusion:

The impact of AxSpA on work outcomes is substantial and necessitates effective intervention, like controlling disease activity. The significantly elevated levels of presenteeism, absenteeism, and WPL when compared to global data may be linked to suboptimal disease management due to limited availability of biological treatments as well as labor-intensive jobs among the population.

Background

The durability of immune responses after vaccination against SARS CoV-2 in patients with autoimmune rheumatic diseases (AIRD) is not well studied.

Objective

We aimed to determine the longevity of humoral immune response to homologous vaccination with 2 doses of ChAdOx1 nCoV-19 and BBV152 in adult patients with AIRD.

Methods

In this prospective observational study, patients aged 18 to 60 years, diagnosed with SLE, primary systemic vasculitis and inflammatory arthritis (IA) who were scheduled to or had received vaccination with either ChAdOx1 nCoV-19 or BBV152 were recruited between June 2021 to March 2023. IgG antibodies against SARS CoV-2 spike protein (anti-S) were estimated in serum or plasma of patients prior to vaccination if feasible (T0), 15 to 28 days (T1), 75-100 days (T2), 170-190 days (T3), 240-290 days (T4), 340-370 days (T5) after receiving Vx2 by Diasorin chemiluminescence assay. The association of antibody titers with baseline parameters is estimated using chi-square or Mann Whitney tests using SPSS 16.

Results

Overall, 118 patients (93 females; age: 37.1, IQR: 28- 45 years; disease duration: 60 ((IQR) 29-103) months) with AIRD (SLE:48(40.6%), IA:53(44.9%), systemic vasculitis:7(5.9%) and spondyloarthropathy: 9(7.6%)) were recruited. Baseline demographic data has been depicted in (Table 1). The median antibody titer did not differ significantly between the two vaccines at T1 while the titers at T2, T3 and T4 were significantly lower for those vaccinated with BBV 152 (Table 2). The steroid dose prior to T1 and T2, intake of DMARDs in peri-vaccination period, type of AIRD (IA vs CTD) was not associated with significant difference in antibody titers at T1 and T2.

Conclusion

The anti-S humoral immune response persisted beyond 1 year for most of our patients with AIRD. After 3 months of vaccination, these responses were higher for ChAdOx1 nCoV-19 as compared with BBV152 in our patients with AIRD.

Introduction

Sarcoidosis is a complex, multisystem disorder characterized by the formation of non-caseating granulomas in various organs, most commonly the lungs and lymph nodes. Ocular involvement is frequent, occurring in up to 78% of patients, while skin manifestations are seen in approximately 25–35% of patients. Here we present a case of a 13-year-old boy with generalized body pain that later evolved to oculocutaneous sarcoidosis.

Case Report

A 13-year-old Sri Lankan boy presented in 2021 with severe generalized body pain, bilateral cervical lymph node swelling, occasional fever, and elevated inflammatory markers. Initial investigations for infections, malignancies, and autoimmune diseases were inconclusive. Lymph node biopsy suggested toxoplasmosis, but serological tests were normal. He later developed left eye visual blurring and periorbital swelling, with MRI showing left optic neuritis and orbital soft tissue oedema. Treatment with IV Methylprednisolone and oral prednisolone resolved his symptoms but was lost to follow-up. A year later, he presented with transient self-resolving subcutaneous nodules over trunk and limbs. due to its transient nature. ANA, RF, ACE level, serum Calcium, serum protein electrophoresis and IgG4 levels were negative. He was started on steroids, Methotrexate, and Infliximab assuming the possibility of autoinflammatory syndrome or sarcoidosis. In 2024 he again developed bilateral submandibular and cervical lymphadenopathy with acute right side visual blurring. MRI showed right optic neuritis and lacrimal gland swelling. (Figure 1) Lymph node Biopsy confirmed non-caseating granulomas. (Figure 2). Based on the findings, a diagnosis of oculocutaneous sarcoidosis was made. Treatment with steroids, methotrexate and Adalimumab was initiated, resulting in a remarkable response.

Conclusion

Sarcoidosis should be considered as a differential diagnosis in cases of optic neuritis with lymphadenopathy and cutaneous manifestations. Diagnosis of oculocutaneous sarcoidosis is challenging and often requires a multidisciplinary approach involving dermatologists, ophthalmologists, and rheumatologists.

Background

Knee osteoarthritis is one of the most commonly encountered joint disease with prevalence ranging upto 7.9%. There are various radiologic instruments to measure the disease severity of knee osteoarthritis. Kellgren Lawrence (KL grading) classification is commonly used radiographic tool to assess severity. High frequency musculoskeletal ultrasound is being used now-a-days as a reliable tool for assessment of osteoarthritis

Objective

To compare musculoskeletal findings with X-ray findings in knee osteoarthritis

Method

A prospective crosssectional study was conducted in patients with knee osteoarthritis in national centre for rheumatologic disease. An AP view standing X-ray was done for the involved knee and K-L grading was done. High frequency musculoskeletal ultrasound was done for the same knee. Synovial hypertrophy, Doppler activity, cartilage thickness (medial, lateral, intercondylar), osteophytes were noted. Pearson’s coefficient of correlation was calculated to compare the correlation between musculoskeletal ultrasound and radiographic findings

Result

A total of 138 patients with knee osteoarthritis were enrolled in the study with female predominance of 83% and mean age of 56.87± 10.61 years. A negative correlation was observed between cartilage thickness (medial) with joint space narrowing (KL grading)

Conclusion

Musculoskeletal ultrasound may be used in clinical practice to diagnose and monitor cases of knee osteoarthritis. It is potential imaging technique which might help in therapeutic interventions as well as disease monitoring.

Background:

Anti-Ro antibody positivity (anti-Ro+) can have possible association with the clinical features and serology in patients with systemic lupus erythematosus (SLE).

Methods:

This retrospective observational study enrolled 158 SLE patients at the Rheumatology department, Fauji Foundation Hospital, from 1st May to 31st October 2023. Demographic and clinical data were collected. Patients were stratified by anti-Ro (positive vs negative), concomitant anti-La (anti-Ro+/ anti-La+; anti-Ro- / anti-La-; anti-Ro+/anti-La- and anti-Ro-/anti-La+), and ANA status (positive vs negative). Mann-Whitney U and Chi-square tests were employed for comparisons, with statistical significance set at p$<$0.05

Results:

Study population consisted mainly of females (98.7%), with median age of 30± 20 years. Anti-Ro antibodies were positive in 74(46.8%) patients. This group had significantly higher discoid rash (16.2% vs 6%, p-value 0.04), sicca symptoms (41.1% vs 31%, p-value 0.005), anemia (71.6% vs 56%, p-value 0.04) compared to anti-Ro negative group. No significant differences were observed in nephritis (29.7% vs 34.5%, p=0.52) or neurological disease (9.5% vs 11.9%, p=0.69) between the two groups. Anti-Ro+ /anti-La+ group had higher myositis (20.6%), sicca symptoms (55.9%) and fever (70.6%) compared to other groups. ANA+/anti-Ro+ group (48.6%) had significant association with sicca symptoms (50.7%) and fever (64.2%).

Conclusion:

Anti-Ro positivity in SLE is associated with muco-cutaneous manifestations and anemia. Concomitant anti-La positivity is linked to increased xerophthalmia, myositis, and fever. While anti-Ro status is independent of ANA, the combination of both autoantibodies is associated with increased sicca symptoms and fever in SLE patients.

Background:

Musculoskeletal ultrasound (MSUS) is commonly used to detect double contour sign (DCS) to diagnose gout. MSUS is subjective and needs user training and experience for optimal visualisation. Machine learning (ML) models can be used for automatic detection of DCS. We sought to identify the optimal algorithm for detection of DCS on ultrasound images of the fist metatarsophalangeal joint (MTPJ1) using candidate ML algorithms.

Methods:

MSUS images of MTPJ1 of male patients acquired between January, 2020 and January, 2023 were included in this study. Diagnosis of gout was based on imaging, joint aspiration, serum uric acid level, and clinical evaluation. Images were acquired with a high frequency linear transducer (18 MHz) with a dedicated MSUS machine (Toshiba Xario 200). Patients diagnosed with calcium pyrophosphate crystal disease or gross deformities of MTPJ1 were excluded. Selected images were pre-processed using Keras-OCR. The following candidate ML algorithms were trained: Support Vector machine, tree-based algorithms like Random Forest and Decision Tree and boosting-based algorithms like Gradient Boosting and XGBoost and these were compared with a deep neural network (2D-CNN). Classification outcomes (DCS positive or negative) were compared before and after image augmentation. Classification performance was measured using sensitivity, specificity, and area under the receiver operating curve (AUROC) along with accuracy, precision and F1-score.

Results:

We used 410 high resolution ultrasound images of MTPJ1 of male patients, 311 patients with gout and positive DCS and 99 patients without gout and no DCS. On average, the final model using XGBoost for detection of DCS showed sensitivity of 94% and specificity of 93.7% and AUROC of 85%. Using XGBoost accuracy and precision were 84.74% and 87% respectively and recall and F1-scores were 94% and 90% respectively. Comparison of different models is given in Table 1. Classification performance improved after augmentation in almost all the models.

Conclusions:

The classification model based on a boosting based ML model (XGBoost) was trained with a relatively small dataset; however, it showed good accuracy. This proposed system was useful in correct classification of DCS in MSUS images with potential to aid imaging diagnostics of gout in a wider clinical setting.

Objective:

To find out correlation between disease activity scores of Rheumatoid arthritis (RA), shedding light on significance of each disease activity score.

Patients and methods:

It is a cross sectional comparative study. The study was carried out at Rheumatology department, Fauji Foundation Hospital Rawalpindi, Pakistan over a period of six months. 240 patients (33 males, 203 females) aged $\ge$18 years diagnosed as RA according to ACR 2010 criteria, were selected. We recorded demographics, details of RA, laboratory value (baseline chemistry, acute phase reactants, and RA serology), comorbidities, DAS 28 ESR, DAS 28 CRP, CDAI, SDAI and RAPID 3 of all the patients. Analysis was performed using Statistical Package for Social Sciences (SPSS) version 23. Pairwise correlation between the disease activity indices was assessed by Spearman’s log-rank analysis.

Results:

Median age of the study population was 53 years (range 20-87 years). Duration of RA in study population was 8 years (IQR= 4-15). Twenty-two patients (9.6%) had early RA, whereas 208 patients (90.4%) had established RA. ESR was raised in 163 (70.9%) of the RA patients. Methotrexate was the most common disease modifying drug, which was being used by 180(77.9%) patients.

All the disease activity scores showed a significant correlation on bivariate spearman’s analysis. Although the strength of the correlation varied. Disease activity by DAS-28 ESR was most strongly correlated with CDAI. Disease activity assessment via RAPID-3 had the highest correlation with CDAI. There was a fair correlation of RAPID-3 with DAS-28 and a weak correlation with SDAI. Disease activity, as assessed by CDAI and SDAI had a moderate correlation with each other.

Conclusion:

While all disease activity indices demonstrate agreement in clinical use, CDAI and RAPID-3 offer suitable alternatives to DAS28 in resource-limited settings.

Background

ANCA-associated vasculitis (AAV) is a rare disease with significant mortality and morbidity, exhibiting different spectrums among ethnic groups. Chinese patients are more likely to be diagnosed with microscopic polyangiitis (MPA), be MPO-positive, and have renal involvement. However, reliable epidemiological data on AAV in China is scarce. This study aims to provide detailed information on the epidemiology, clinical features, treatment, and outcomes of AAV patients in Hong Kong.

Methods

Patients diagnosed with Granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), or eosinophilic granulomatosis with polyangiitis (EGPA) and followed up in 8 public hospitals from 2013-2023 in Hong Kong were identified using the Hospital Authority Clinical Data Retrieval System (CDARS) with ICD-10 diagnostic codes. Diagnoses were confirmed by designated rheumatologist specialists at each center. Data on demographics, clinical presentations, laboratory results, biopsy findings, treatments, and outcomes were recorded. Overall survival, relapse-free survival, and dialysis-free survival, along with their associated risk factors, were analyzed using the Kaplan-Meier method and Cox Regression. Data of GPA and MPA patients are presented here.

Results

A total of 35 GPA and 127 MPA patients were identified, with 91 (56.2%) being female. The mean age of onset was 67.73 years. Forty-six patients (28.4%) were PR3 positive, and 134 patients (82.7%) were MPO positive. Most patients had organ-threatening or life-threatening disease, with 127 patients (78.4%) having an FFS $\ge$2. The mean BVAS score was 15.7. Pulmonary haemorrhage occurred in 35 patients (21.6%), and advanced renal insufficiency (Cr > 4g/L or dialysis) occurred in 49 patients at presentation (30.2%). Thirty-four patients received rituximab, and 83 received cyclophosphamide for induction. PLEX was performed in 30 patients. Over a median follow-up period of 39 months, mortality occurred in 64 patients (39.5%). The 1-year mortality rate was 22.2%, and the 5-year mortality rate was 33.3%. Infections accounted for most of the mortality (46.7%), followed by active vasculitis (15%), renal failure (11.7%), cardiovascular/cerebrovascular events (10%) and malignancy (1.9%). Factors associated with mortality included MPO positivity (OR 4.25, p=0.01), PR3 positivity (OR 3.87, p=0.01), FFS $\ge$2 (OR 2.54, p=0.04) and pulmonary haemorrhage (OR 2.13, p=0.01) in multivariate analysis. Relapse occurred in 30 patients (18.5%), and 30 patients (18.5%) required dialysis.

Conclusion

AAV patients in Hong Kong, predominantly MPA and MPO positive, often present with severe organ or life-threatening manifestations. There is a high mortality risk among these patients, with MPO or PR3 positivity, FFS$>$=2 and pulmonary haemorrhage being significant predictors of mortality.

Introduction:

Acute Pancreatitis in systemic lupus Erythematous is rare but has high mortality. Incidence varies from 1-5% and reported mortality is 3-37.04% with mortality being higher in juvenile SLE.

Objective:

Study of Clinical and serological profile of SLE patients presenting with acute pancreatitis.

Method:

Retrospective data of SLE patients visiting our institution was collected. Diagnosis was considered if patients had clinical + biochemical and radiological evidence of pancreatitis. Patients with diagnosis of gallstone, history of alcohol intake, serum Triglycerides>500 mg/dl, serum calcium>10.5 mg/dl and taking Azathioprine at the time of diagnosis were excluded.

Results:

9 patients fulfilled inclusion and exclusion criteria. 7 had severe and 2 had moderately severe acute pancreatitis. Female: Male ratio was 8:1, Median age was 25(15-50) years, Median disease duration 12 months (3-48), 4(44.45%) presented within 1 year of disease onset. Mean SELENA-SLEDAI at the time of presentation was 26(19-41). 4(44.45%) were on corticosteroids at the time of presentation. Other active domains at the time of diagnosis were Nephritis in 7(77.78%), NPLE in 3(33.34%), Myositis in 4(44.45%), Autoimmune haemolytic anaemia in 2 (22.22%), Macrophage activation syndrome in 4 (44.45%) enteritis in 1 (11.11%) Myocarditis in 2 (22.22%) and Mucocutaneous in 9 (100%) patients.

All patients had elevated Serum Amylase and Lipase level with Median serum Amylase being 545 Units/Litre (normal$<$176) and median serum Lipase being 511 ($<$160) Units/litre. 6(66.67%) had hypocalcemia and 5 (55.56%) had hypertriglyceridemia. Raised dsDNA was seen in 9(100%, median 277 IU/ml) and hypocomplementemia was seen in 7(77.78%, Median C3 value 49 mg/dL)). None of the patients had associated antiphospholipid syndrome but persistent antiphospholipid antibodies were present in 5(55.56%)-4 had lupus anticoagulant and 1 had Anticardiolipin antibody.

6(66.67%) patients had microbiologically confirmed infection the time of diagnosis of acute pancreatitis (4 bacterial and 1 fungal) among which 3 had lower respiratory tract infection, 1 had urinary tract infection, 1 had pseudomembranous colitis and 1 had peri-pancreatic abscess. 7 patients received only corticosteroids, and 2 patients received IVIG with corticosteroids. 3 out of 9 patients (33.34%) expired due to multi-organ failure. Out of 6 surviving patients all received IV cyclophosphamide (NIH protocol) and are doing fine.

Conclusion:

Acute pancreatitis in SLE has high mortality probably due to associated high clinical and serological disease activity and high incidence of concomitant infection. Extensive investigation for underlying infection (bacterial, viral and fungal) should be done in SLE patients presenting with acute pancreatitis for comprehensive management.

Background

Juvenile idiopathic arthritis (JIA) is the most common chronic inflammatory joint disease in children and adolescents. Historically, moderate to severe psoriatic arthritis were treated with disease-modifying anti-rheumatic drugs, such as methotrexate. Since the 2000s, biologics such as TNF-inhibitors had been used for severe or refractory disease. More recently, Secukinumab, an anti-IL-17 monoclonal antibody, was approved in the US for treatment of psoriatic JIA since 2021.

Methods

We describe a case of psoriatic JIA, who was to our knowledge the first paediatric patient to be treated with Secukinumab in Hong Kong.

Results

A 12-years-old Chinese girl presented with 6-months history of plaque psoriasis associated with left hip pain. (Figure 1) Her JADAS10 score was 16.5 and her PASI score was 10.4. Naproxen, topical steroids and oral methotrexate were used but did not significantly improve her symptoms. An MRI scan continued to show evidence of active sacroiliitis. The patient was treated with Secukinumab at a loading dose of 150mg weekly SC for five weeks, followed by a maintenance dose of 150mg monthly. After one month, her inflammatory markers normalised and her JADAS10 score was 0, indicating inactive arthritis. After 3 months, her PASI score was 0.6. Follow-up of her skin condition showed complete solution of psoriasis at 6 months. (Figure 2)

Conclusion

Secukinumab induced rapid disease remission in a Chinese paediatric patient with PsJIA. It helped prevent progressive joint damage and greatly improved her skin’s cosmetic appearance. Our case demonstrated the remarkable efficacy of Secukinumab in the treatment of PsJIA.

Background:

In developing nations, rheumatic diseases bear significant health issues, affecting millions of people and placing a substantial burden on healthcare systems. Education of the patient is crucial for the management and prevention of rheumatic diseases. Objectives: To determine the level of patient knowledge regarding rheumatology and rheumatic diseases in a developing nation.

Methods:

Patients attending rheumatology clinics of Mayo Hospital, Lahore, in 2021-23, participated in this cross-sectional study. A structured questionnaire was used to collect data on demographic characteristics and rheumatology and rheumatic disease knowledge of patients. Questions pertaining to the definition, symptoms, causes, and treatment of rheumatic diseases were included in the questionnaire.

Results:

The study enrolled a total of 1306 patients. Demographic characteristics of rheumatic disease patients comprised 377 males (28.9%) and 929 females (71.1%), whereby the proportion of female patients was significantly high (p$<$0.05). The mean age of the participants was 43.22+10.98 years. Patients’ locations were categorized as either rural or urban with statistically significant number (p$<$0.05) of 757 (57.96%) and 549 (42.03%), respectively. Majority of patients were educated (p$<$0.05), with 706 females (74.5%) and 248 males (65.7%). Patients’ overall knowledge of rheumatology and rheumatic diseases was inadequate, with majority of the patients (56.4%) had scores between 0 and 2, indicating a lack of understanding of these conditions. Rheumatic disease causes, symptoms, and treatment options were poorly understood by patients.

Conclusion:

In developing nations, patient education regarding rheumatology and rheumatic diseases appears to be deficient. Providers of healthcare should address this deficiency by providing patients with adequate education and resources. Improved patient knowledge will ultimately improve patient outcomes and lessen the burden of rheumatic diseases on the healthcare systems of developing nations.

Background:

Tuberculous arthritis presents a diagnostic difficulty due to its modest clinical indications and vague imaging data, frequently resulting in a delayed or wrong diagnosis. Pigmented villonodular synovitis (PVNS), which might seem like tuberculous arthritis, complicates the diagnosis.

Case Report:

This case study features a young child whose knee arthritis was first misdiagnosed as PVNS. Magnetic resonance imaging (MRI) revealed nodular masses and hemosiderin deposits around the knee joint, indicating widespread PVNS. This error caused a delay in accurate diagnosis and treatment.

Conclusion:

These findings underscore the relevance of investigating TB in patients who appear with PVNS-like characteristics, emphasizing the necessity for a high index of suspicion and quick examination to achieve fast and correct diagnosis for successful care.

Background:

Ankylosing spondylitis (AS) is a chronic inflammatory disease primarily affecting the spine and pelvis. While studies have extensively explored the physical manifestations of AS, the prevalence of sexual dysfunction in male patients and its association with disease activity remains under-explored.

The study aims to determine the prevalence of sexual dysfunction in AS and its association with the disease activity.

Methods:

This comparative cross-sectional observational study was conducted with sexually active male patients diagnosed with AS. Disease activity was quantified using the Bath Ankylosing Spondylitis Disease Activity Index (BASDAI) and the Ankylosing Spondylitis Disease Activity Score-C-reactive protein (ASDAS-CRP). Functional limitations and the extent of spinal and pelvic involvement were assessed through the Bath Ankylosing Spondylitis Functional Index (BASFI) and the Bath Ankylosing Spondylitis Metrology Index (BASMI), respectively. Sexual function was evaluated using the International Index of Erectile Function-15 (IIEF-15), and psychological status was measured via the Hospital Anxiety and Depression Scale (HADS).

Results:

The study reveals a significantly higher prevalence of sexual dysfunction among male patients with AS compared to control subjects, with p-values of < 0.0001 across all five domains of IIEF-15 (Fig.1). Notably, domains such as erectile function, orgasmic function, and overall sexual satisfaction were markedly impaired and exhibited a strong negative correlation with increased disease activity, as measured by BASDAI and the ASDAS-CRP. The erectile function scores demonstrated a pronounced negative correlation with disease activity indices, with correlation coefficients of -0.9840 for BASDAI and -0.9669 for ASDAS-CRP (Fig.2).

Conclusion:

AS is associated with a high prevalence of sexual dysfunction and correlates with disease severity. The results underscore the importance of integrating sexual health assessments into routine clinical evaluations of AS patients and addressing sexual dysfunction as a critical component of comprehensive disease management.

Background:

Scleroderma associated pulmonary arterial hypertension and interstitial lung disease (ILD) with pulmonary hypertension are considered to be separate pathological processes with different approach to management. In this study, we looked at the demographics, clinical features, and outcome of patients with both type of pulmonary hypertension in systemic sclerosis

Methods:

Patients diagnosed with scleroderma and with pulmonary hypertension as per transthoracic ECHO in the time period between 2010 and 2023 were retrospectively identified enrolled in the study. They were divided into two groups-isolated PAH and ILD-PH. ECHO parameters, clinical data were recorded

Results:

There were 800 patients with a diagnosis of systemic sclerosis during this period. In total, 49 incident scleroderma PH cases were identified (6.13%); 25 with diffuse cutaneous SSc (51.02%), 19 with limited cutaneous SSc (38.78%), and 5 with sine scleroderma (10.2%). Among the study group, 39 (79.59%) patients had ILD with PH and 10 (20.4%) had isolated PH (Table 1). Though the baseline ECHO parameters were similar in both the groups, features of severe PH - Right atrial and ventricular dilatation with Right ventricular dysfunction was more in Isolated PH than ILD-PH (50% vs 23%). Other clinical features were similar in both the groups (Table 2). Isolated PH was treated with vasodilatory therapy and all patients with ILD -PAH were treated with immunosuppression along with vasodilatory therapy. Follow up of ECHO parameters of the two groups at the end of 2 years showed better improvement in the ILD-PH group. Unsatisfactory long-term outcome as defined by clinical NYHA progression, hospitalization and need for long term oxygen therapy was similar in both isolated PH and PH-ILD (40% vs 41%). 1 year survival was 95.9% (2/49 lost to follow up).

Conclusions:

The prevalence of Pulmonary Hypertension in our scleroderma cohort of a tertiary care center was 6.13%. ILD-PH was 4 times more prevalent than isolated PH.46.94% of all our PH patients had it at presentation. The one-year survival of our cohort was 95.9% almost equivalent to other international cohorts

Background:

Pachydermoperiostosis (PDP), or primary hypertrophic osteoarthropathy (PHO), also known as the Touraine–Solente–Gole syndrome, is a genetic disorder that is uncommon and is identified by finger clubbing, skin thickening, and periosteal growth.

Case Report:

This case study details the case of a 21-year-old man with PDP to raise awareness, improve diagnosis, and enhance management strategies for the condition. The individual showed common signs like digital clubbing, pachydermia, and periostosis, as well as related symptoms like hyperhidrosis. Radiological imaging supported the diagnosis by revealing periosteal reactions and cortical thickening in multiple bones. Other conditions with comparable clinical characteristics were considered in the differential diagnosis, however, the diagnosis of PHO was confirmed by the specific radiological results and normal hormonal levels. The treatment primarily targets alleviating symptoms with drugs like NSAIDs and corticosteroids, along with newer options such as bisphosphonates.

Conclusion:

Timely detection and correct treatment are essential to enhance the well-being of people with PHO. This case study emphasizes the significance of tracking symptoms and offering thorough care to those with PDP/PHO.

Background

Consensus on anti-SRP myositis shows that early rituximab (RTX) is favoured over conventional immunosuppression (cIS), but the data is derived from case series without comparative studies. This study aims to a) explore poor prognostic features, and b) assess and compare the efficacy and safety of RTX with cIS in individuals with anti-SRP myositis.

Methods

This is an observational study in a tertiary care centre in India. Data was obtained from a prospectively maintained database comprising consecutive adult (> 16 years) patients diagnosed myositis. A total of 1033 myositis patients were screened from January 2019 to January 2024. Fourty IIM patients with confirmed anti-SRP positivity fulfilling inclusion criteria with more than 3 months of follow-up were selected for the study and response analysis. Details were obtained at baseline, 3 months, and 6 months, as well as subsequent follow-up. Treatment response was categorised as improved, worsening, or stable disease. The characteristics of patients between “RTX” and “cIS” groups were compared using t-test and Chi-square test as appropriate. Survival analysis curves were plotted for improvement and death between the two groups.

Results

The mean age of onset of anti-SRP myositis was 44.2 (SD13.14) years; 71.4% of patients were female. Baseline characteristics are described in Table 1. For response analysis, 35 patients into two groups: RTX and cIS. Fifteen (42.85%) patients were given RTX. While 20 received cIS (57.14%, 12/20 (60%) received mycophenolate mofetil, 8/20 (40%) received methotrexate, and 5/20 (25%) received cyclophosphamide). The median follow-up was 12 (IQR 6-24) months. There was no significant difference in improvement between RTX and cIS groups (11/15 (73.3%) RTX group and 9/20(45%) cIS group (p-value -0.465)). Time to improvement was also not significantly different (Figure 1). However, the Mean change in MMT8 from baseline to 6 months was more in the RTX group (p-value 0.001). The average monthly steroid dose significantly decreased six months after RTX with a p-value of 0.001(1600.13mg (SD 271.53) in RTX versus 826.6 mg (SD 370.35) in cIS). Mortality rates stood at 1/15 (6.67%) for RTX and 4/20 (20%) for cIS. Unravelling predictors of mortality revealed significant associations with cardiac involvement (p-value - 0.02) and severe non-ambulant muscle weakness (p-value - 0.03).

Conclusions

Both cIS and RTX are effective and safe in anti-SRP myositis for muscle affection and time for response. Cardiac involvement and severe muscle weakness are associated with mortality. RTX use decreases steroid requirement in anti-SRP myositis.

Background:

Antiphospholipid syndrome (APLA) is an autoimmune disease with autoantibodies and hypercoagulability. APLA commonly presents vascular thrombosis and obstetrical complications. Stroke in young adults is rare, and unusual causes must be considered.

Case Report:

Here we present a case of 26 year-old male patient with sudden onset of hemiparesis marked on right side without evidence of atherothrombosis or other vascular pathology. However extensive workup revealed primary Antiphospholipid Antibody Syndrome (APLA) as the underlying cause. MRI brain showing acute infarcts involving both cerebellar hemispheres more on left side, bilateral periventricular and deep white matter infarcts involving bilateral frontal and parietal lobes, Periventricular T2WI hyperintensities likely suggestive of Fazekas II change. Prompt treatment with anticoagulation led to significant neurological recovery.

Conclusion:

This case highlights the importance of considering APLA in the differential diagnosis of stroke in young adults, and the need for early recognition and treatment to improve outcomes.

Background:

Gastrointestinal manifestations are common in Systemic Sclerosis, but studies in the Indian population are sparse.

Methods:

This is a retrospective study of 100 patients who presented to the Department of Clinical Immunology and Rheumatology between 2014 to 2019. The charts and investigations were reviewed for clinical and serological profile, and the investigations done were recorded.

Results:

100 patients diagnosed with systemic sclerosis (SSc) who fulfilled the ACR/EULAR 2013 criteria were included in the study-81 patients were classified as diffuse SSc, 15 as limited SSc and 4 as Sine Scleroderma. In total, 72 out of 100 patients reported reflux symptoms and 4 reported chronic diarrhoea. The imaging studies done for the patients and the findings are summarised in Table 1; the indications for endoscopy and the findings are summarised in Table 2. 2 patients were diagnosed with Chronic liver disease. 90 out of 100 patients were treated with a combination of proton pump inhibitors (PPIs) and prokinetics and 10 patients with PPIs alone. H Pylori eradication and antifungal therapy as per scopy findings were administered. 3 patients with chronic diarrhoea were empirically treated with Rifaximin for probable SIBO with symptomatic improvement.

Conclusions:

GI involvement is common in Scleroderma patients (93%- symptomatic and subclinical combined); however, the symptoms are under reported. Complications of GERD are less common in our popular, probably due to early initiation of PPIs and prokinetics. HRCT identified esophageal dilatation in 79% of the patients. Patients with refractory symptoms need to undergo scopies to rule out infections and malignancies. Further studies are required for better assessment and management of GI symptoms.

Background:

ASA syndrome is a rare and complex immune-mediated entity with heterogeneously diverse presentations diagnosed by the presence of antibodies directed against aminoacyl tRNA synthetase (most common anti-JO-1). The clinical manifestations range from myositis, interstitial lung disease (NSIP PATTERN), non-erosive arthritis, unexplained recurrent fever, Raynaud’s phenomenon, mechanics hands involving mainly skin, respiratory and musculoskeletal systems.

Case Report:

We report a case of 37-year-old female who had multiple admissions for polyarthritis which was symmetrical, progressive, involving large and small joints associated with morning stiffness, relieved by medication temporarily, with subsequent development of respiratory symptoms like cough, fever off and on, shortness of breath, exertional dyspnea, along with cracks on fingers of hands (mechanics hands). Symptoms worsened gradually with time, affecting her daily routine activities like combing hair, changing clothes, exertional dyspnea on taking few steps, debilitating her quality of life. Extensive and detailed workup was done, Anti-Jo-1 antibodies and Anti-SSA/Ro were positive, CPK level raised, serum aldolase raised, HRCT chest showed interstitial lung disease most likely NSIP, ANA negative, ACE levels normal, Bronchial biopsy showed benign respiratory mucosa, no evidence of malignancy was found. Patient was diagnosed with anti-synthetase antibody syndrome and started on pulse therapy with methylprednisolone.

Conclusion:

This article explores the clinical manifestations, diagnostic criteria, treatment, progression and prognosis of the disease course.

Background:

Interstitial lung disease (ILD) is a significant cause of mortality in patients with Rheumatoid Arthritis (RA). Existing studies on the efficacy of Mycophenolate (MPA) for RA-associated ILD are limited, with the largest study including only 26 patients. This study aims to evaluate the efficacy of MPA in stabilizing or improving lung function in a larger cohort of RA-ILD patients over an extended follow-up period.

Methods:

Patients diagnosed with RA-ILD and treated with MPA for $\ge$ 1year, attending our clinics between 2017 and 2023 were included. Data regarding demography, spirometry, HRCT and treatment were noted retrospectively at the diagnosis of ILD (baseline) and 6 monthly visits thereafter till last follow up. Data were collected on patient demographics, spirometry, high-resolution CT (HRCT) scans, and treatment regimens. The primary outcome was the change in predicted forced vital capacity (FVC) over the follow-up period, with a $\ge$ 10% decline considered as progression. Clinical improvements in cough, dyspnea, and HRCT findings were recorded as binary outcomes.

Results:

Of the 73 patients (58 females), 70% showed stability or improvement in FVC, while 18% experienced a decline. Clinically, 86% of patients improved or remained stable, while 12% worsened. HRCT results showed stable or improved disease in 34% of patients, 30% had worsening of fibrosis, while 36% did not undergo follow-up HRCT due to clinical stability. Physiologically - 52(70%) met the primary outcome of stability/ improvement in % FVC while 18% had worsening in % FVC. Use of rituximab was not associated with better outcomes. None of the baseline clinical parameters predicted better outcomes in univariate. The most common adverse events were dyspepsia and serious infections, occurring in 12% of patients

Conclusion:

MPA is an effective and relatively safe treatment option for RA-ILD, with 70% of patients achieving stabilization or improvement in lung function.

Background

Rheumatoid arthritis (RA) is a chronic inflammatory disease with progressive joint destruction. High-resolution peripheral quantitative CT (HR-pQCT) is a novel technique for detailed bone microstructure analysis allowing volumetric assessment of bone erosions. While most patients demonstrating little baseline joint damage on radiography in the early stage of RA, whether achieving simple disease activity index (SDAI) remission can lead to better structural outcome is to be confirmed.

Methods

Patients with ERA were recruited and treated to the target of simple disease activity index (SDAI) remission using a standardized algorithm. One hundred patients who received the one-year tight-control treatment were grouped by achieving SDAI sustained LDA [SDAI $\le 11]$ at 6, 9 and 12 months (sLDA group) or not (non-sLDA group). HR-pQCT of the second to fourth metacarpophalangeal joints were performed at baseline, month 6 and one-year. Erosion volume and marginal bone mineral density (BMD) were measured. On an individual patient basis, erosion progression was defined as at least one erosion showing (1) an increase in erosion volume exceeding the smallest detectable change [SDC] (0.08 mm ³) AND (2) a decrease in marginal BMD exceeding SDC (7.8 mmHA/cm ³). Erosion healing was defined as at least one erosion showing (1) a reduction in erosion volume greater than SDC AND an increase in BMD exceeding SDC OR complete disappearance of the lesion AND (2) the absence of any erosion progression.

Results

Fourteen patients (12.8%) achieved sustained SDAI remission from 6-12 months (SDI group). After 12 months, a significant reduction in erosion volume and marginal osteosclerosis was observed in both groups. The SDI group showed a lower incidence of erosion progression (increase in volume $>$0.08mm ³ and decrease in marginal osteosclerosis $>$7.8mg HA/cm ³ : 0% vs 16%, p=0.135) and exhibited a higher rate of erosion healing (56% vs 29%, p=0.083) compared with the non-SDI group. In the GEE model, patients in the SDI group showed a higher likelihood of erosion healing (OR: 2.976, 95% CI: 1.0 - 8.9, p=0.050). The changes in erosion depth and width were similar between two groups.

Conclusion

Achieving sustained SDAI remission could improve erosion healing and limit erosion progression in patients with ERA.

Background:

Systemic sclerosis (SSc) is a chronic disease presenting in the age group of 20-50yrs and females are more affected than males. Osteoporosis is common in many rheumatological diseases and an association has been well established in Rheumatoid Arthritis and Systemic Lupus Erythematosus. However, there is no such robust data in patients affected with SSc. Our study aimed to measure Bone Mineral Density and assess the risk of fracture in patients of systemic sclerosis using the Fracture Risk Assessment using (FRAX) algorithm.

Methodology:

The case control study conducted in a tertiary care hospital included 24 patients of age 18-45years with systemic sclerosis and 24 age and sex matched healthy controls. 62.5% cases were antiScl-70 antibody positive, 62.5% had interstitial lung disease and 37.5% had pulmonary arterial hypertension. The BMD of Scl-70 antibody positive patients was 0.64± 0.08 gm/sq.cm at neck of femur, 0.70± 0.08 gm/sq cm at lumbar spine and 0.37± 0.06 gm/sq. cm at forearm. A significant lower T score at neck of femur was noted in cases as compared to controls (-1.91± 10 vs -0.32± 1.06, p=0.001). The mean FRAX score value for major osteoporotic fracture in cases, was 1.46± 0.61% with maximum value of 3% and minimum value of 0.9%. Whereas, for the hip fracture mean FRAX was 0.83± 0.07% with range of 0.7% to 1.9%. A significant difference was noted in risk of major osteoporotic fractures between cases and controls (p=0.017). On multivariate regression models computed to analyse the covariates of BMD for neck of femur, lumbar spine and forearm, it was observed that serum vitamin D levels, presence of pulmonary hypertension and/or ILD were significant covariates of BMD neck of femur.

Conclusion:

Systemic sclerosis is associated with significantly low bone mineral density compared to controls. The bone mass was negatively correlated with involvement of internal organs and serum iPTH levels. SSc may be considered as an independent risk factor for osteoporosis and patients of SSc should be evaluated for BMD with DEXA scan to prevent osteoporotic fractures.

Background

Non-steroidal anti-inflammatory drugs (NSAIDs) are amongst the most frequently prescribed medications, their associated hypersensitivity reactions can not only lead to potentially severe reactions, but also limit analgesic and anti-inflammation options. This is especially true for those who are ailed with rheumatological or orthopaedic diseases leading to chronic pain and inflammatory conditions. This highlights the importance of identifying different types of NSAID hypersensitivity, and to find safe alternatives, namely selective COX-2 inhibitors, to groups of patients that may benefit the most from its utility.

Objective

To assess and categorise patients with suspected NSAID hypersensitivity, and to successfully provide alternatives to those with chronic rheumatologic and orthopedic ailments.

Methods

115 patients with suspected NSAID hypersensitivity were referred to Queen Mary Hospital for specialist evaluation between Jan 2023 and March 2024, those suitable were later challenged and delabelled. Remaining patients were assessed as clinically compatible with NSAID hypersensitivity, and later separated into whether they had background of orthopedic or rheumatological conditions, hence strong indications for NSAIDs, and without, to which selective COX-2 inhibitors tolerance tests were offered.

Results

Of 115 patients, 20% (23/115) were assessed as unlikely to have NSAID hypersensitivity, and later challenged. 18.2% (21/115) had negative challenges and were delabelled; only 2 patients had positive reactions, confirming NSAID hypersensitivity, of which symptoms were mild and did not require hospitalization. 82% of the remaining patients (94/115) were then later stratified into different NSAID hypersensitivity reactions according to European Academy of Allergy & Clinical Immunology (EAACI) guidelines. 57.3% (66/115) of patients were able to resume use of NSAIDs either by finding an alternative NSAID, namely a selective COX2 inhibitor (45/115, 39.1%), or through a negative drug challenge (21/115, 18.2%). Overall there was no significant difference in the proportion of patients with successful COX2 inhibitor challenges between the group with rheumatological and orthopedic diagnoses against those without (62% vs 54% p=0.414). 29 patients are still pending workup, likely underestimating the amount of patients that are able to resume NSAIDs.

Conclusion

NSAID hypersensitivity is amongst the most commonly reported drug allergies, and may present with many different clinical phenotypes. Patients with suspected NSAID hypersensitivity require allergist evaluation, and if not clinically suggestive of allergy, would benefit from drug challenges, especially in cases with underlying rheumatological or orthopedic conditions, where NSAIDs are essential. In situations where NSAID hypersensitivity is considered likely, selective COX-2 inhibitors can often be considered as a suitable alternative.

Introduction

The paradigm of managing JIA has been changing. There is ongoing discussion whether using bDMARDs early in the treatment course is superior to sequential use after cDMARDs¹. On the other hand, with a treat to target approach, it is shown both approaches achieve comparable outcome².

We aim to investigate the real life experience of a single-centre JIA cohort regarding treatment escalation, including the timing and the role of disease activity index.

Method

A chart review on all the JIA attending the Paediatric Rheumatology Clinic at the Prince of Wales Hospital between JUL 2023 to AUG 2024 was performed. SJIA was excluded as it follows different treatment algorithm. Outcome of interests are time to cDMARDs or bDMARDs from first clinic visit, time to escalation to cDMARDs and bDMARDs, disease activity indexes (cJADAS, BASDAI and BASFI for ERA) at the time of treatment escalation. Results are presented in descriptive analysis.

Result

Total 17 cases with 30 treatment escalation events were recorded. The median age at last FU was 15 years old (IQR 9.5-18.5), with a median duration of FU of 51 months (IQR 25-79.5). For the whole group the median time from first clinic visit to cDMARDs was 1 month (IQR 0-26), to bDMARDs was 12 months (IQR 5-33). 3 cases required further switch.

The median time to 1st treatment escalation was 1 month (IQR 0-26). The median time between 1st and 2nd treatment escalation was 4.5 months (IQR 3-17). These usually involved change from cDMARDs to bDMARDs.

cJADAS scores were recorded at the time of switch in 23 events. The median score at each treatment escalation was 14 (IQR 8.9-18). It signified high or moderate disease activity in oligoarticular or polyarticular course JIA, respectively.

For the eight ERA, the median time to first cDMARDs was 6 months (IQR 0-28). It was 17 months (IQR 3-29) to next treatment, mostly bDMARDs. Regarding disease activity index at the time of switch, the median cJADAS of 13 events was 11.2 (IQR 6.5-14), ie moderate disease activity, while the median BASDAI of 14 events was 2.55 (IQR2.07-4.4), ie not active disease.

Conclusion

Overall the timing of cDMARDs use, treatment switch and performance of cJADAS were inline with other centres. In this small cohort BASDAI is not optimal in terms of indicating treatment switch in ERA.

1 Kimura Y et al. Arthritis Rheumatol. 2021 Oct;73(10):1898-1909.

2 Hissink Muller P et a1. Ann. Rheum. Dis. 2019;78:51–59

Background

Coronary vasculitis is a rare cause of myocardial infarction through varied mechanisms. We report the case of a 32-year-old female found to have diffuse coronary microaneurysms during treatment for an Inferior ST Elevation Myocardial Infarction (STEMI), with challenges in pinpointing the underlying aetiology.

Results

Our patient has been diagnosed with a seronegative arthritis and had been initiated on regular Golimumab 4 months prior. She presented with prolonged fever for 4 weeks and inflammatory oligoarthritis with left sacroiliac joint pain. During this admission, Antinuclear antibody was 1:2560 and anti ds DNA was 1:80. She had highly positive Rickettsia conorii IgG titres of 1:128, suggestive of acute infection with Orientia tsustsugamushi. Meropenem and doxycyclin for presumed typhus had no impact on her fever spikes. In the 10th week of the illness, she developed ischaemic chest pain with electrocardiographic changes of inferior STEMI and was thrombolysed. Invasive coronary angiography revealed numerous coronary microaneurysms affecting all major myocardial vessels, with minimal coronary artery disease. Following multidisciplinary discussion, she was treated with intravenous methylprednisolone, antiplatelets, a statin and warfarin for a diagnosis of coronary vasculitis.

Repeat angiography showed complete resolution of the microaneurysms. She continues on anticoagulation and has since had no coronary events.

Conclusion

Typhus and medium vessel vasculitis such as Kawasaki and Polyarteritis Nodosa were potential diagnoses for this presentation. A diagnosis of SLE was established due to the highly specific immunological profile. As there are reports of high Rickettsial titres in autoimmune disease, which alongside the lack of response to doxycyclin makes Typhus unlikely. The possibility of golimumab induced SLE was briefly entertained due to similar reports, but the positivity of ds DNA and major organ involvement made this possibility unlikely.

We highlight the cardiac involvement in this case due to its high morbidity on young patients, and the current paucity of knowledge on SLE-related endothelial dysfunction and immune-mediated vascular damage. The role of anticoagulation in this inflammatory milieu too, is controversial.

This case highlights a rare presentation of SLE, with an emphasis on coronary vasculitis. It adds to the current knowledge on the complex cardiovascular risk in SLE and highlights ambiguous areas in current management strategies to prompt further research.

Background:

Immunodeficiency 70 is a rare disease and there are only very few cases in the literature.

Case History:

Young boy in his early 20’s from Indian subcontinent presented with recurrent episodes of fever, oral ulcers, loss of weight for past 3 years. Four episodes (july 2019, april 2020, APRIL 2021, Jan 2022) of fever, each high grade associated with chills, rigor, nausea, myalgia, throat pain, holo-cranial headache, painful oral ulcers over the tongue and painless over lips. Initial episode of fever was associated with leucopenia (3200) and thrombocytopenia (122000), acute kidney injury (creatinie-3.0) and pyelonephritis, transaminitis. He received 2 weeks of parenteral antibiotics and improved. Subsequent episodes of fever is not associated with any localizing symptoms or signs. No history Suggestive of connective tissue disease. Family history was non contributory.

Physical examination:

He was febrile (101 degree F), pulse rate 100/minute, respiratory rate 18/minute, tongue ulceration in lateral border, no lymphadenopathy, no organomegaly, normal sytemic examination.

Evaluation:

The patient was evaluated for chronic infections, hematalogical malignancy, Behcet’s disease, auto inflammatory syndromes like Haplo insufficiency of A20.

His complete blood count, electrolytes, renal function test, liver function test urine microscopy, blood culture were normal. CRP was elevated (58 mg/l). Tzanck smear from buccal mucosa was negative. CT thorax and abdomen showed no significant lymphadenopathy, organomegaly, evidence of infections or malignancy.

In view of oral ulcers & fever Behcet’s disease was considered and HLA b51 was sent and came negative. Exome genome sequencing was done to diagnose immunodeficiency or autoinflammatory syndromes which came positive for immunodeficiency 70.

Treatment:

He was treated with colchicine and fever subsided and leucopenia improved

Discussion:

Immunodeficiency-70 (IMD70) is an autosomal dominant immunologic disorder characterized by severe cutaneous warts on the hands, feet, and face, suggesting increased susceptibility to human papillomavirus (HPV) infection. Affected individuals may also have recurrent bacterial infections like pneumonia, boils, sinusitis, as well as feature of autoinflammation, such as colitis, celiac disease, and retinal vasculitis. Laboratory studies show decreased CD4+ T cells and decreased CD19+ B cells; hypogammaglobulinemia, combined T cell and B cell immunodeficiency characterized by decreased CD4+ T cells, decreased CD19+ B cells, recurrent bacterial infections, and severe cutaneous warts on the hands, feet, and face that has material basis in heterozygous mutation in IVNS1ABP on chromosome 1q25.3.

Background:

TAFRO SYNDROME mimics SLE. Differentiating both is essential for correct manangement.

Case report:

40 year old lady came with complaints of fever, insidious onset of painless abdominal distension which was gradually progressive to form tense ascities in a span of 2 months. On examination she was pallor, having pedal edema, tense ascities and cervical and axillary lymph node enlargement (mazimum 1^* 1 cm). Lymph nodes were firm, non matted, not fixed. On evaluation found to have pancytopenia. (Hb: 5.3 gm%, platelet count, Wbc-3600/cu mm, 90,000/cu mm), Her LFT -alkaline phosphate -639 U/L, Hyperbilirubinemia with total Bil of 1.73 mg/dl and direct fraction of 1 mg/dl and LDH of 139 U/L. Renal - 24 hour urine protien = 301 mg.

She had malena at presentation and her Hb dropped to 3.2 gm% platelets count of 10,000. Ct abdomen showed -Mild to moderate ascites, mild hepatomegaly. Ascitic Tap of 3 litre was done which showed high SAAG and High protein. Ugi endoscopy was done which showed Antral gastritis and no feature of porat hypertension. Infective workup including tuberculosis came negative.

Autoimmune work

ANA - 2+ speckled. (1:100 dilution)

Coombs test direct - 3+

C3 = 115 (90 to 180)

C4 = 39.8 (10 to 40)

Ds DNA = 121 (< 100 IU/ ml)

Urine UP/UC = 0.48

24 hour urine protien = 301 mg

Anti SSA ELISA test - 63 (< 20 RU/ml)

Anti SM ELISA test 1 (< 20 Ru/ml)

ELISA Anti RNP - 2 Ru/ml (< 20)

APLA work up was within normal limit and lupus anticoagulant was mildy positve.

She was satifying the ACR EULAR 2019 classification criteria for SLE and SLE can have lymphadenopathy features suggestive of castle man disease. She was intially diagnosed as SLE and treated with Sle protocol and was not reposnding. Lymphnode biopsy later was done suggestive of Hypervascular type of castle man disease and intiated on R - CEP (Rituximab, Cyclophosphamide, etosposide, prednsiolone)

LEARNING POINT

1) SLE is a great mimicker of variety of other diseases and rheumatologist should be aware of the disease that mimics SLE.

2) TAFRO syndrome is variety of castleman disease.

 T-Thrombocytopenia

 A-Ascities

 F-Fever

 R-renal dysfunction

 O-Organomegaly

3) TAFRO syndrome has autoimmune manifestations that mimics SLE and it an aggressive variety of castleman disease

Background:

Neuropsychiatric systemic lupus erythematosus (NPSLE) is a serious complication in SLE, and distinguishing these manifestations from other neuropsychiatric disorders is challenging.

Method:

This was a retrospective single-center study. NP manifestations were attributed to SLE according to clinical experts’ opinions. Demographic and clinical data were obtained in patients with and without NP manifestations attributed to SLE. And antibodies profiles were included for hierarchical cluster analysis, analyze the correlation between different antibody clusters and clinical manifestations of SLE.

Results:

The final analysis group included 176 patients, of which 151 (85.8%) had NP manifestations attributed by SLE. 1, The positive rates of anti-nuclear antibodies (ANA) and anti-SSB of NPSLE patients and the levels of immunoglobulin G (IgG) were significantly higher in comparison with those NP symptoms not attributed to SLE (non-NPSLE) patients [145 (96.0%) vs 18 (72.0%), P $<$ 0.001; 23 (15.2%) vs 0 (0.0%), P=0.048; median 23.5 vs 9.6 points, IQR 10.00, 48.25 vs 7.58,15.83 points, P $<$ 0.01; respectively]. The levels of C4 and the proportion of patients with concomitant hemolytic anemia were lower than non-NPSLE [median 0.09 vs 0.15 points, IQR 0.05, 0.14 vs 0.07, 0.17 points, P $<$ 0.01; 9 (6.0%) vs 7 (28.0%), P $<$ 0.005; respectively]; 2, NP manifestations in the central nervous system were the most frequent (86.9%). In patients with NPSLE, cerebrovascular disease, seizures, headche occurred most often; Among non-NPSLE, common causes included infection, non-specific cerebrovascular disease, hematological disease; 3, Four clusters among 151 NPSLE patients were identified. Cluster 1 comprised patients with triple aPLs (antiphospholipid antibodies) positivity and demonstrates a high incidence of cerebrovascular disease, seizure, Cluster 2 was characterized by high positive rates of anti-SSA and anti-SSB, with high risk of system damage such as mucocutaneous, renal. Cluster 3 was characterized by a high positive rate of anti Sm, mainly present with aseptic meningitis and polyneuropathy, often accompanied by renal and hematological involvement. Cluster 4 was characterized by high positive rates of anti Ro52, anti RNP (ribosome P protein), includes seizure and demyelinating syndrome.

Conclusion:

Four characterized antibody clusters are identified in NPSLE patients in this study. Different clusters are associated with certain clinical features and complications of NPSLE. However, the correlations found in this study need to be investigated further in larger populations.

Introduction

Azathioprine (AZA), an immunosuppressant and mercaptopurine analog, is used to manage rheumatological and immunological conditions. Although effective, AZA can cause myelosuppression, sometimes necessitating dose adjustments or discontinuation. While severe pancytopenia or alopecia is rare, these issues can arise. This report describes a case of severe myelosuppression and alopecia totalis following AZA treatment in a patient with systemic lupus erythematosus (SLE).

Case Report

A 27-year-old healthy female presented with an 8-month history of fever, cough with pleuritic chest pain, and hemoptysis. Clinical and biochemical evaluations diagnosed her with SLE and secondary pulmonary vasculitis. She was initially treated with systemic steroids, followed by AZA. AZA was started at 25 mg daily (0.8 mg/kg) and increased to 75 mg daily (2 mg/kg) over three months. After dose escalation, she developed increased hair loss, feverishness, and lethargy, prompting her admission to a medical facility. Examination revealed significant non-scarring alopecia, (Figure 1) but no clinical signs of an SLE flare.

Laboratory tests showed pancytopenia: hemoglobin 8.4 g/dL, white blood cells 880/$\mu$L, platelets 130K/$\mu$L, and absolute neutrophil count 38/$\mu$L. Inflammatory markers, complement levels, and septic screening were normal. AZA was discontinued, and she was treated for neutropenic sepsis with high-dose steroids and GM-CSF. Despite these measures, blood parameters did not improve as expected. After 25 days of treatment, hair loss ceased, and cytopenias began to recover. Bone marrow analysis was not performed during the acute phase. Upon discharge, her blood counts were WBC 6,130/$\mu$L, hemoglobin 10.9 g/dL, and platelets 85K/$\mu$L.

Given her SLE and lung pathology, she continued immunosuppressive therapy but was switched from AZA to IV Rituximab. Six weeks later, her blood counts had improved (WBC 10K/$\mu$L, hemoglobin 10.9 g/dL, platelets 289K/$\mu$L), and hair regrowth was observed. (Figure 2)

Conclusion

This case underscores the need for clinicians to be vigilant for early signs of severe myelosuppression, such as alopecia, in patients receiving azathioprine. Awareness of these uncommon side effects is crucial for effective management.

Rheumatoid arthritis (RA) and spondyloarthritis(SpA) are chronic inflammatory disorders with unique symptoms and pathologies. Misclassification of these two entities is prevalent due to overlapping clinical features and the presence of typical symptoms for both disorders. Rheumatoid nodules are one of the most common extra articular manifestations in rheumatoid arthritis, usually associated with severe disease activity. Herein, we describe a case of rheumatoid arthritis with bilateral sacroiliitis, an uncommon joint involvement of rheumatoid arthritis, and accelerated subcutaneous nodulosis after treatment with adalimumab.

Introduction

RA is a chronic, autoimmune disorder that primarily affects peripheral synovial joints [1]. The axial skeleton is usually spared other than the cervical spine, particularly C1 to C2 [1]. Though sacroiliitis is a paramount sign of SpA, it can be rarely observed in RA [2]. The commonest extra-articular manifestation of RA is subcutaneous nodules, which can be accelerated with RA treatments [3]. Herein, we describe a patient presented with bilateral sacroiliitis, along with the diagnostic and therapeutic challenges we surmounted.

Case presentation

A-45-years-old female presented with a one-year history of inflammatory arthritis of bilateral metacarpophalangeal, wrist, and ankle joints. She also had inflammatory-type back pain with buttock pain and plantar fasciitis. The Left-sided Faber test was positive. The laboratory investigations revealed elevated inflammatory markers, negative RF and HLA-B27. MRI-SI joints revealed bilateral active sacroiliitis. She was treated as SpA with NSAIDS at first, followed by subcutaneous adalimumab due to inadequate response. Though arthritis improved following adalimumab, multiple firm subcutaneous nodules occurred in the extensor surface of the elbow and hands after six-doses of adalimumab. Biopsy was compatible with rheumatoid nodule and anti-cyclic citrullinated peptide was positive. Based on the presence of rheumatoid nodules and positive anti-CCP antibodies, the patient was diagnosed with seropositive RA and bilateral sacroiliitis, an unusual joint involvement in RA.

Discussion

Sacroiliitis is the paramount clinical sign in SpA [2]. However, it can be seen in a wide range of disease conditions [4]. Misclassification between SpA and RA can occur due to overlapping clinical manifestations [4].

 The Rheumatoid nodule is the most common cutaneous manifestation of RA and often seen in seropositive RA and more severe disease [3]. There have been case reports of accelerated subcutaneous nodulosis in patient with RA, treated with MTX, leflunomide, azathioprine, TNF alfa inhibitors and tocilizumab [3].

 Though Inflammatory back pain, active sacroiliitis, and plantar fasciitis mislead the diagnosis, rheumatoid nodules and positive anti-CCP antibodies aid the classification of RA.

Background –

Juvenile Dermatomyositis can have a unpredictable course after diagnosis, with no tools to prognosticate near-term outcomes. We explored a Short Tau Inversion Recovery (STIR) sequence Magnetic Resonance Imaging (MRI) protocol as a non-invasive baseline investigation to assess the present outcome in a cohort of JDM patients.

Objectives –

To determine the difference in baseline STIR-MRI parameters between children with JDM, who are currently in remission with medication and children in sustained remission off medications.

Methods –

We retrospectively reviewed the baseline MRI findings of 28 consecutive patients, evaluated and followed up for at least one year, by a senior paediatric rheumatologist. A radiologist reviewed the images of each child’s MRI thigh STIR Sequences, scored on four domains - STIR hyper-intensities, fatty infiltration of muscle, presence of skin and subcutaneous tissue involvement and muscle atrophy. We compared the median (inter quartile range) scores between children in remission with and without medication.

Results –

Median baseline age was 7 (7.5) years. At baseline, they reported a median CMAS of 17 (21), with STIR signal intensity of 2 (2), fatty infiltration grade of 0 (1). Twelve (42.9%) and four children (14.3%) showed evidence of skin and subcutaneous involvement and muscle atrophy, respectively. After being followed up for a median of 6 (6) years, eight children (28.6%) were in remission off medication. There was evidence that fatty infiltration differed between children in remission with medication vs off medication (p = 0.043). MRI Fatty infiltration grades predicted delayed treatment initiation ($>$ 6 months since symptom onset) in children, fairly accurately (AUC 0.731 ± 0.11, p = 0.036).

Conclusions –

Our study indicates that fatty infiltration in baseline STIR-MRI protocol may suggest a chronic disease course. Fatty infiltration, also suggests a history of inadequate/delayed diagnosis and treatment since symptom onset. Thus, early MRI may prognosticate the disease course in children with JDM. Further research is needed to exclude potential effect modifiers and confounders in the analysis.

Background:

Tumor necrosis factor (TNF) inhibitors have been extensively utilized to treat various autoimmune diseases. However, there are ongoing debates about the risk of inflammatory central nervous system (CNS) diseases events following TNF inhibitors therapy, as well as uncertainty about how this risk varies across different autoimmune diseases or TNF blocking agents. We aimed to evaluate the risk of inflammatory CNS diseases after anti-TNF initiation and assess the difference in risk among different types of underlying autoimmune diseases, or TNF inhibitors.

Method:

Separate searches were conducted across PubMed, EMBASE, and the Cochrane Library from inception until March 1, 2024. Observational studies assessing the association between anti-TNF therapy and inflammatory CNS diseases relative to a comparator group. Study eligibility assessment and data extraction were independently conducted by two investigators following PRISMA guidelines. The risk ratio (RR) was used as the effect measure of the pooled analysis. The primary outcome was the risk of incident inflammatory CNS events after anti-TNF therapy for autoimmune diseases. Secondary analyses were performed based on different types of underlying autoimmune diseases and TNF inhibitors.

Results:

Eighteen studies involving 1,118,428 patients with autoimmune diseases contributing over 5,698,532 person-years of follow-up were analyzed. The incidence rates of new-onset inflammatory CNS events after initiating TNF inhibitors ranged from 2.0 to 13.4 per 10,000 person-years. Overall, exposure to TNF inhibitors was associated with a 33% increased risk of any inflammatory CNS diseases compared to conventional therapies (RR = 1.36, 95%CI 1.01-1.84; I2 = 49%), mainly attributed to demyelinating diseases (RR = 1.38, 95% CI 1.04-1.81; I2 = 31%). Secondary analyses revealed a similar risk of inflammatory CNS diseases across different types of underlying autoimmune diseases (rheumatic diseases: RR 1.36, 95%CI 0.84-2.21; inflammatory bowel disease 1.49, 95%CI 0.93-2.40; P for subgroup = 0.74) and TNF inhibitors (anti-TNF monoclonal antibodies versus etanercept: RR 1.04, 95%CI 0.93-1.15, I2 = 0%).

Conclusions:

Compared to conventional therapies, exposure to TNF inhibitors exposure was associated with a 36% increased risk of inflammatory CNS diseases, irrespective of background autoimmune diseases or TNF inhibitor types.

Background:

Cardiovascular and cerebrovascular outcomes in patients with anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV) remain elusive, especially for some specific cardiovascular and cerebrovascular disease (CCVD). We aimed to quantify the magnitude of the risk of total and type-specific CCVD in AAV population.

Method:

Electronic database searches of PubMed, EMBASE and Cochrane Library plus a hand search of conference proceedings were performed. Observational studies reporting data on CCVD in AAV patients were eligible. Pooled risk ratios (RR) with 95% confidence intervals were calculated. The primary outcome was the risk of CCVD, CAD and CVA in AAV population. The secondary outcomes were the risk of cause-specific CCVD, including myocardial infraction, angina pectoris, heart failure, stroke, ischemic stroke.

Results:

Fourteen studies met the inclusion criteria, comprising 20,096 AAV patients (over 46,495 person-years) with 5,757 CCVD events. Compared with non-vasculitis population, AAV patients showed an 83% increased risk of incident CCVD (1.83 [1.37-2.45]; n=10), 48% for coronary artery disease (1.48 [1.26-1.75]; n=9), and 56% for cerebrovascular accident (1.56 [1.22-1.99]; n=9). For type-specific CCVD, the risks of myocardial infarction, stroke, heart failure were increased by 67% (1.67 [1.29-2.15]; n=6), 97% (1.97 [1.19-3.25]; n=8) and 72% (1.72 [1.28-2.32]; n=4), whereas there was only a trend toward a higher risk of angina pectoris (1.46 [0.90-2.39]; n=2), and ischemic stroke (1.88 [0.86-4.12]; n=4). Subgroup analyses by AAV type found significantly increased CCVD risk in both granulomatosis with polyangiitis (1.87 [1.29-2.73]; n=7) and microscopic polyangiitis (2.93 [1.58-5.43]; n=3). In three studies reporting impact of follow-up period after AAV diagnosis, the CCVD risk was significantly higher in the first two years after diagnosis than the subsequent follow-up (2.23 [2.00-2.48] vs. 1.48 [1.40-1.56]; p < 0.01). Significant heterogeneity existed in the main analyses.

Conclusions:

This meta-analysis demonstrates that AAV is associated with increased risks of overall and type-specific CCVD, especially within two years after AAV diagnosis.

Background:

Individuals with systemic lupus erythematosus (SLE) patients confer substantial increased risks of cardiovascular complications that have become the primary cause of premature mortality worldwide. We aimed to investigate the association between triglyceride-glucose (TyG) index and carotid atherosclerosis in patients with systemic lupus erythematosus (SLE).

Methods:

This cross-sectional study in a tertiary hospital included 333 consecutive SLE patients receiving carotid ultrasonography with integrated TyG index, calculated as ln [fasting triglycerides (mg/dL) × fasting glucose (mg/dL)/2]. The subjects were categorized into three groups according to the TyG index tertiles. Logistic regression models were used to assess the association of TyG index with carotid atherosclerosis and carotid artery plaque. Fully adjusted model included age, gender, body mass index, comorbidities, low-density lipoprotein cholesterol and SLE-related variables.

Results:

There were 10.5% (35/333) of participants identified as having carotid atherosclerosis. Patients with carotid atherosclerosis had significantly higher TyG index as compared with those without (8.77 ± 0.45 vs. 8.45 ± 0.49, p $<$ 0.001). The frequency of carotid atherosclerosis was increased with increases in TyG index tertiles (3.6% for tertile 1, 10.0% for tertile 2, 17.7% for tertile 3; p = 0.003). Multivariate logistic analyses showed that each 1-unit increase in TyG index was significantly associated with prevalent carotid atherosclerosis (unadjusted OR 4.07 (1.87-8.87); fully-adjusted OR 4.88 (1.74-13.69)). The unadjusted and fully-adjusted OR for occurrence of carotid atherosclerosis were 5.70 (1.88-17.28) and 5.88 (1.66-20.74) in patients with tertile 3 compared to patients with tertile 1 of TyG index (P for trend 0.006 and 0.019, respectively). Similar findings were noticeable for the outcome of carotid artery plaque.

Conclusions:

The present study suggested that an elevated TyG index was associated with a higher risk of carotid atherosclerosis, independent of traditional cardiovascular risk factors and SLE-related factors. TyG index may be a promising and accessible atherosclerotic indictor for SLE population.

Background:

There were mixed practices and attitude towards glucocorticoids (GC) use for rheumatoid arthritis (RA). We aimed to investigate current practices, changes, and perceptions of rheumatologists regarding GC use in RA patients.

Methods:

A cross-sectional survey was conducted using a structured questionnaire between April and August 2023. Rheumatologists from 31 province-level regions of Mainland China were invited to participate. Chi-squared tests were adopted to investigate the differences by sociodemographic characteristics.

Results:

1,717 rheumatologists from 598 hospitals completed the survey with a response rate of 92%. Up to 60% of participants expressed currently infrequent initiation of GC co-therapy with csDMARDs (hardly ever 7.0%; occasionally 24.6%; sometimes 29.1%), accompanied by a decline of frequency over time reported in 64.2%. Regarding attitudes towards bridging therapy with GC, 604 (35.2%) participants supported this approach, 468 (27.3%) opposed it, and 645 (37.6%) remained inconclusive. Time to GC discontinuation in context of csDMARDs was commonly reported within 6 months in current practice which has been narrowed over time. Reasons for chronic GC use were mostly reported due to suboptimal disease control, followed by the need of RA complications, and pre-existing comorbidities. After failure of GC cessation, majority of respondents (84.4%) would escalate RA therapy (commonly by addition of JAK inhibitors, TNF inhibitors), which usually or often facilitated the GC cessation. The most frequently reported advantages and weaknesses of GC were rapid and strong efficacy, adverse events, respectively. Regarding long-term low-dose GC use for RA, the percentage of respondents who supported, opposed, or depended on the situation were 15.9%, 17.2%, and 66.9%, respectively.

Conclusion:

The current data demonstrate that GC initiation for RA treatment is not as frequent as before and the awareness of GC discontinuation is growing in current practice. Attitudes towards GC co-therapy with csDMARDs vary considerably and long-term low-dose GC use remain situation dependent.

Background

The side effect of allopurinol was severe allopurinol hypersensitivity (SAH). There are two major causes including genetic risk factor, HLA-B^*58:01 and non-genetic risk factors including female, old age, renal impairment, inadequate starting dosage of allopurinol and received diuretic. Although HLA-B^*58:01 testing is recommended in Thai, the several problems including cost, limitation of the testing and the delay of the result. Therefore, we aimed to use these non-genetic risk factors for developing the model to predict SAH.

Method

The study was a retrospective observational incidence density sampling. SAH cases were collected from tertiary care medical center which some cases were referred from primary care medical center; where most non-SAH cases were collected from primary care hospitals and tertiary care medical centers. The data of non-genetic factors particularly sex, age, renal function, co-diuresis, starting dosage of allopurinol and serum uric acid (SUA) of non-SAH cases selected the first description of allopurinol. The binary prevalence-weighted logistic regression statistical method was used to develop the prediction models. Three models were developed following general practice.

Result

Totally, there were 209 cases of SAH and 23,068 cases of non-SAH. Factors that were associated with the development of SAH within 90 days were female, old age (¿ 65 years old), renal impairment, inadequate starting dosage of allopurinol, co-medication(s) with diuretic and high SUA before prescription of allopurinol. Model 1a and model 1b were applied for patients who did not have and have SUA when starting allopurinol, respectively. Model 2 was applied for patients who had all non-genetic risk factors and started allopurinol within 60 days but have not SAH. The area under the receiver operating characteristic curve for model 1a, model 1b and model 2 were 0.72, 0.81 and 0.82, respectively (Figure 1). The performance for each predictions SAH were good.

Conclusion

Model 1a and model 1b predict SAH for the patients who had their first prescribed allopurinol, model 2 predicts SAH for the patients who had been taken allopurinol within 60 days but no SAH. The scoring system of each model helps clinician to prescribe allopurinol in real clinical practice before the patients develop SAH. The score of 0-1%, 1-2% and 2-100% indicates the low, moderate and high risk, respectively. The low-risk group can start allopurinol. The moderate-risk group considers to start allopurinol with closed monitoring of SAH. The high-risk group suggests to change to other urate lowering agents for preventing SAH (Table 1).

Background:

Although EuroQol 5-domain (EQ-5D) of the 5-level version (5L) instrument has been used to determine quality of life and health utility in gout, the health utility among patients with gout flare (GF) and non-gout flare (non-GF) is still limited. This study aimed to measure the utility values among GF and non-GF patients in Thailand.

Methods:

A multi-center cross-sectional survey study was performed. Patients with GF and non-GF were face-to-face interviewed by trained research staffs using the EQ-5D-5L (Thai version) and EQ-Visual Analog Scale (VAS) instrument. Those with GF were subdivided into those who received no treatment and those who received treatment less than 48 hours after GF.

Results:

Two hundred and sixteen patients (108 GF and 108 non-GF patients), male in 90.28%, were included. The disease duration was significantly longer in the non-GF than in the GF groups (median: 10 vs 5 years, p = 0.004). There was no difference in the tophi present between the two groups. When compared with non-GF group, the GF patients significantly had low health utility (0.34 ± 0.36 vs. 0.89 ± 0.15, p $\le$ 0.001), and EQ-VAS score (54.73 ± 24.14 vs. 84.06 ± 13.38, p $\le$ 0.001). In a subgroup analysis of the non-GF group, there was not significant health utility and EQ-VAS score between those with tophi and those without tophi (0.87 ± 0.14 vs. 0.90 ± 0.15, p = 0.351 and 83.37 ± 14.92 vs. 84.33 ± 12.84, p = 0.738, respectively).

Conclusion:

This study highlights the importance of assessing quality of life in managing gouty patients. Patients with GF had lower health utility and EQ-VAS status than the non-GF patients.

Background:

Since Thailand have high prevalence of Human Leukocyte Antigen Type-B^* 58:01 (HLA-B^*58:01) genotype. This genetic has strongly associated with allopurinol hypersensitivity syndrome (AHS). The national health security guideline of Thailand has accepted the association of HLA-B^*58:01 genotype with prevention of AHS. The present study aims to investigate willingness to pay (WTP) of gouty patients for HLA-B^*58:01 testing prior to starting allopurinol and factors influencing their decision for the testing.

Methods:

This study is a cross-sectional survey. The contingent valuation by open-ended questionnaire interview on WTP of gouty patients for HLA-B^*58:01. The descriptive statistics are used. Participants who are age $>$ 20 years, diagnosed based on the American College of Rheumatology/European League Against Rheumatism 2015 gout classification criteria at Naresuan university hospital (NUH), and three sub-district health promoting hospitals of NUH, Phitsanulok, Thailand are included.

Results:

Two hundred and fifty gouty patients are included. The majority of them are male, 214 patients (85.60%). The average age of the participants is 64.5 (± 12.8) years. Male patients are more WTP than female patients. The gouty patients with a monthly income between 10,001-30,000 Thai Baht (THB) (288.43-865.30 United states dollar (USD)) are the WTP patient group. The results show the majority of patients (64.2%) have WTP between 101-500 THB (2.88-14.42 USD) or average of median (IQR) is 500 (300, 800) THB or 14.37 8.62, 22.99) USD for the testing. The reason for decline the testing are the cost of the testing is too expensive. Other urate lowering agents, gouty patients have WTP for febuxostat, sulfinpyrazone and benzbromarone in 68%, 67.60% and 71.60%, respectively. Average WTP for febuxostat, sulfinpyrazone and benzbromarone 1,000 THB (28.74 USD), 1,000 THB (28.74 USD) and 285 THB (8.19 USD), respectively.

Conclusion:

The majority of gouty patients are willing to pay for the genetic testing even they have to pay for the testing cost themselves which is 1,000 THB (28.74 USD). They are willing to pay if they have to bear the cost of the testing themselves. For those un-WTP, the main reason is the cost of the genetic testing too expensive, followed by lack of income/low income. The maximum amount most participants are willing to pay for the testing is average of median (IQR) is 500 (300, 800) THB or 14.37 (8.62, 22.99) USD. This study may has affected to change a health policy for the genetic testing price or to switch from allopurinol to other urate lowering agents.

Background:

The common causes of Dry eye syndrome (DES) were autoimmune or non-autoimmune disorders. DES is common but lack of data in quality of life (QoL) of DES patients in Thailand. The primary outcome of this study was to determine QoL and health utility in patients of DES by EuroQol 5-domain (EQ-5D) of the 5-level version (5L) instrument. The secondary outcome was comparison of the utility in the patients of DES classified by severity and causes including the autoimmune and non-autoimmune diseases.

Methods:

The study was a cross-sectional study at a hospital in the northern part of Thailand. The inclusions DES patients were followed by Tear Film and Ocular surface Society the Dry Eye WorkShop II definition. The EQ-5D-5L (Thai version) descriptive system and the EQ visual analogue scale (VAS) was instrument for QoL evaluation.

Results:

Total patients of DES were fifty-six. The most patients were female. The mean age was 57.7(± 13.9) years. Most patients (94.6%) were female. The mean age was 59.7 (± 13.9) years old. The median time from diagnosis of DES was 4.7 years (IQR = 2.2 to 6.8 years). The common cause of DES associated was autoimmune disease. Primary Sjogren syndrome and idiopathic causes were the most common causes in autoimmune diseases and non- autoimmune diseases, respectively. In autoimmune disease, Primary Sjogren syndrome, was 41.18%. The most common causes of non-autoimmune diseases were idiopathic. The mean of EQ-5D-utility and EQ-VAS of DES were 0.76 (± 0.18) and 72.86 (± 15.19), respectively. The mean of EQ-5D-utility and EQ-VAS in these patients who were classified by severity including mild, moderate and severe were 0.84 (± 0.16) and 67.31(± 15.76), 0.78 (± 0.14) and 74.47 (± 15.71), 0.71 (± 0.22) and 74.58 (± 14.36), respectively. The mean of EQ-5D-utility and EQ-VAS in these patients who were classified by autoimmune or non-autoimmune disorders were 0.75 (± 0.21) and 75.78 (± 14.21), 0.78 (± 0.15) and 68.96 (± 15.88), respectively. There is no statistic significant in the EQ-5D-utility and EQ-VAS among severity and the causes including autoimmune or non-autoimmune disorders of these patients.

Conclusions:

This study demonstrated the importance of assessing QoL in DES. Primary Sjogren syndrome and idiopathic causes were the most common causes in autoimmune diseases and non-autoimmune diseases, respectively. The EQ-5D-utility was accorded with the severity of DES. However, no statistic significant was showed in the mean of EQ-5D-utility and EQ-VAS between the severity and between the causes including the autoimmune and non-autoimmune diseases of these patients.

Background

Skin involvement in PsA is associated with worsened overall health related quality of life, increased health care utilisation. Skin disease is assessed objectively through PASI score. As per BSR guidelines for treatment of PsA, skin and quality of life (QOL) assessment is an important domain in deciding escalation of treatment for biologics. Assessment of skin aids in selection of the type of biologic.

Methods

20 patients with psoriatic arthritis were assessed in rheumatology outpatient clinics of East Lancashire Hospitals NHS trust, UK. The last letter to GP of each patient was reviewed. Data on objective assessment (PASI Score) and subjective assessment (mild, moderate or severe) or no assessment of skin disease along with QOL assessment was recorded. Staff were educated to assess PASI score, using mobile application “GRAPPA”, calculate and document. All patients with psoriatic arthritis were given the Dermatology Life Quality Index (DLQI) questionnaire. BSR 2022 guidance on PsA was considered as the standard reference.

 After 3 months of intervention, data were analysed the effectiveness of the intervention.

Results

Pre intervention results showed a subjective assessment of 10 (50%), Objective assessment of skin disease 0(0%), and DLQI assessment of 0 (0%). Post intervention results showed an improvement of objective assessment of skin via PASI score to 10 (50%). Another 8 (40%) of patients had subjective assessment of skin. DLQI assessment improved to 4(20%).

Conclusion

Introduction of convenient mobile application to assess skin involvement of PsA had shown remarkable improvement of PASI score. DQOLI patient questionnaire improved QOL assessment in PsA patients.

 Change of practice through objective skin assessment through PASI score and assessment of DQOLI lead to escalation of treatment to biologics. In addition, PASI score helped in selection of most appropriate biologic that targets both skin and joints.

Background:

There is currently a limited offer of automated multi-analyte tests for autoantibody testing in autoimmune connective tissue diseases (CTD). We assessed the analytical performance of a novel, single-use, multiplexed microarray immunoassay prototype (MosaiQ AiPlex CTDplus), used with the fully automated MosaiQ^Ⓡ system, for the simultaneous detection of fifteen autoantibodies associated with CTD, compared with selected CE-marked devices.

Methods:

Banked human serum samples, characterized as reactive to ≥ 1 autoantibody or non-reactive were included. Reactive samples were characterized with FIDIS^™ Connective Profile (Theradiag) for CENP B, Jo-1, Ribosomal P, Scl-70, Sm, Sm/RNP, SS-A 60, TRIM21 (SS-A 52), SS-B and U1RNP; EliA CCP (Phadia) for CCP2, QUANTA Lite^Ⓡ Chromatin (Werfen) for Chromatin, QUANTA Flash^Ⓡ DFS70^* (Werfen) for DFS70, Anti-dsDNA-IgG-ELISA (DRG) for dsDNA, and Immunodot (Euroimmun) for RNA polymerase III (RNAP III). For CCP2, only reactive samples were included. For the remaining analytes, non-reactive samples were characterized using immunofluorescence (ANA-Ro IgG FLUORESCENT HEp-2000^Ⓡ; Immuno Concepts, Germany) and ANAscreen ELISA (ORGENTEC-Diagnostika-GmbH, Germany). Each individual non-reactive sample was tested with the investigational device once; reactive samples were tested in duplicates. Positive percentage agreement (PPA) and negative percentage agreement (NPA) for individual analytes were calculated. For CCP2 only PPA is presented as no negative samples were included.

Results:

Compared with relevant devices, the investigational prototype showed PPA ranging from 75% for chromatin to 99% for SS-A 60 and TRIM21. NPA ranged from 95% for chromatin, RNAP III, Scl-70 and Sm to 100% for DFS70, SS-A 60 and TRIM21. Performance details are shown in the Table.

Conclusions:

The investigational prototype demonstrated substantial agreement with the compared CE-marked devices. Further studies will allow for expanded performance assessment of the investigational device. This fully-automated multiplexed platform has the potential to contribute to optimizing CTD evaluation by simplifying complex testing pathways and analyzing large number of samples per day.