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Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is associated with significant morbidity and mortality. Management of AAV is divided into induction of remission and maintenance of remission. Management of AAV has evolved with the aim of improving treatment outcomes and minimizing treatment toxicities. In this article, we will review the latest evidence on the treatment of AAV.

The clinical studies of Takayasu’s arteritis and antineutrophil cytoplasm antibodies (ANCA)-associated vasculitis are very active in the Asia Pacific region. The results of these studies give us a more comprehensive understanding of these vasculitis, which may not only change the management and long-term prognosis of the disease, but also reduce complications and prolonged survival time. Therefore, we summarize the results of these studies that were led or actively participated by investigators in the Asia Pacific region from 2020 to 2021, mainly focusing on the clinical characteristics and management.

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.