Journal of Clinical Rheumatology and Immunology

Nonsteroidal anti-inflammatory drugs (NSAIDs) are widely used for their analgesic, antipyretic, and anti-inflammatory properties. However, they are also associated with a broad spectrum of hypersensitivity reactions, ranging from mild cutaneous manifestations to severe systemic responses. The complex nature of these reactions and their underlying mechanisms pose challenges in diagnosis and management.

We review the clinical evidence to six distinct clinical phenotypes of NSAID hypersensitivity, including: NSAID-induced urticaria/angioedema or anaphylaxis (SNIUAA), NSAID-exacerbated respiratory disease (NERD), NSAID-exacerbated cutaneous disease (NECD), NSAID-induced urticaria/angioedema (NIUA), food-dependent NSAID-induced hypersensitivity reactions (FDNIH), and selective NSAID-induced delayed reactions (SNIDR). This review aims to provide a comprehensive review of NSAID hypersensitivity reactions based on clinical phenotyping, which can aid in understanding and managing these adverse events.

Objective: To describe the clinical profile and predictors of mortality of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) patients in Hong Kong. To compare the accuracy of the latest Five-Factor Score (FFS-2009) and the Birmingham Vasculitis Activity Score (BVAS) in prediction of survival with this local cohort.

Methods: A retrospective observational study on newly diagnosed AAV patients, from January 1, 2011 to March 31, 2022, managed in the Kowloon West Cluster (KWC) hospitals in Hong Kong. Demographic and baseline characteristics, clinical profile, and treatment profile were reviewed. Factors associated with mortality were analyzed with the Cox proportional hazards model. The performances of FFS and BVAS in mortality prediction were analyzed by receiver operating characteristic (ROC) curves.

Results: A total of 83 AAV patients were included in the study. The median age was 70.5 years at diagnosis. Microscopic polyangiitis (MPA; 69.9%) was the most common AAV subtype. The median FFS and BVAS were 2 and 20, respectively. The overall mortality was 45.6% across the study period. Multivariate Cox regression identified age at diagnosis (HR 1.043, $P=0.027$), stabilized peak serum creatinine (HR 1.002, $P=0.001$), hemoglobin level (HR 0.754, $P=0.006$), cardiac involvement (HR 3.862, $P=0.008$), and use of maintenance therapy (HR 0.261, $P=0.002$) as independent predictors of overall survival. Both FFS and BVAS were significant predictors of overall survival. The areas under the curve (AUC) of ROC curves suggested FFS was a good prediction tool for early mortality in 1 year, with an AUC value of 0.874.

Conclusion: Despite the advances in treatment, AAV still carried significant morbidities with high mortality. Clinical predictors and existing scoring systems showed good predictive power on mortality.

Idiopathic inflammatory myopathy (IIM) poses elevated risk of cardiovascular event and mortality, similar to other autoimmune rheumatic diseases. We conducted a cross-sectional study to examine the prevalence and risk factor of subclinical coronary artery disease (CAD) in patients with IIM, using CT coronary angiogram (CTCA). The prevalence of obstructive CAD and CAD in IIM (13.3% and 66.7%, respectively) were significantly higher than age and sex-matched controls (0% and 30%, respectively, both $p<0.05$). Diabetes mellitus and calcium calcification score $>100$ units were found to be the independent predictors of obstructive CAD. Screening of high-risk patients with aggressive treatment of cardiovascular risk factors should be considered in managing IIM patients.

Objective: To assess the predictive accuracy of the EULAR definition of clinically suspected arthralgia (CSA) for progression to rheumatoid arthritis (RA) in patients presenting with arthralgia without arthritis.

Methods: This prospective study recruited 100 patients with arthralgia but no clinical arthritis. Patients were evaluated at baseline and followed up every 6 months for 2 years. The association of the presence of $\ge 3$ and $\ge 4$ out of seven CSA parameters at baseline with RA development was investigated. The association of individual CSA parameters with RA development was also evaluated.

Results: Twenty-one of 100 patients (21%) developed RA. The proportion of patients meeting $\ge 3$ CSA criteria was higher in those who developed RA (66.7%) compared to those who did not (39.2%, $p=0.025$). Similarly, a higher proportion of patients who developed RA met $\ge 4$ CSA criteria (47.6%) compared to those who did not (20.3%, $p=0.011$). The sensitivity and specificity of $\ge 3$ CSA parameters were 71.4% and 69.6%, and for $\ge 4$ CSA parameters were 42.9% and 87.3%, respectively. “Symptom duration $<1$ year” and “difficulty holding a fist” were significantly associated with RA development. A simplified criteria using these two items provided a similar area under receiver operating characteristic (ROC) curve (0.747 vs. 0.676) compared to the original criteria.

Conclusion: Meeting EULAR CSA criteria was significantly associated with progression to RA in patients with arthralgia. Symptom duration $<1$ year and difficulty holding a fist were the most predictive individual parameters. Continuous monitoring using the CSA criteria, and probably a simplified version, could help identify high-risk individuals for RA development.

Myocarditis is an uncommon presentation in systemic sclerosis. We report the case of a patient who developed myocarditis on the initial diagnosis of systemic sclerosis and was successfully managed with optimal medical treatment, including immunosuppression. This case was able to raise awareness in recognizing the heart as an organ of involvement in systemic sclerosis and showed the importance of screening for heart involvement in the management of systemic sclerosis. The management of primary myocardial involvement in systemic sclerosis was also reviewed.

A 38-year-old lady was diagnosed with systemic lupus erythematosus (SLE) and alpha-amino-3-hydroxy-5-methyl-4-isoxazole propionic acid receptor (AMPAR) autoimmune encephalitis, fulfilling both classification criteria during her index admission. She was subsequently diagnosed with seropositive myasthenia gravis (MG) with a typical clinical presentation. Her SLE and AMPAR encephalitis were successfully treated with immunosuppressive therapy. Her MG responded well to anti-cholinesterase inhibitors. We report a distinctive case with the association of three rare autoimmune diseases in an individual.