Understanding the burden of ANCA-associated vasculitis.

Hellmich, Bernhard · Rheumatology (Oxford) · 2026

review · Level V

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Abstract

Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a group of frequently relapsing systemic autoimmune disorders characterized by vasculitis-related organ damage and multiple comorbidities related to chronic inflammation. Advances in immunosuppression-based therapies for AAV have considerably improved remission rates, reduced the risk of relapse, and improved survival in patients with AAV. However, mortality remains high compared with the general population and the benefits of treatment are often offset by treatment-related comorbidities, organ damage and adverse effects, particularly infections. The aim of this review is to investigate the key contributors to disease burden in patients with AAV and to describe strategies for improving health-related quality of life.

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