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Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) consists of autoimmune diseases characterized by necrotizing inflammation of small vessels and presence of ANCA. As a kind of life-threatening disease, AAV tends to involve a plenty of systems, and is subject to relapse even after complete remission. In this review, we focus on the latest progress of epidemiology, etiology, pathogenesis and clinical diagnosis of AAV. [ABSTRACT FROM AUTHOR]