The pathogenesis and treatment in antineutrophil cytoplasmic antibody associated vasculitis

Anti-neutrophil cytoplasmic antibody (ANCA) associated vasculitis (AAV) is a systemic autoimmune disease characterized by leukocytoclastic inflammation of small blood vessels. Commonly detected autoantibodies include anti-protease 3 (PR3) and anti-myeloperoxidase (MPO). Although cell necrosis plays an important role in the production of autoantibodies and the pathogenesis of AAV, the correlation between their titers and disease activity remains elusive. As improved detection techniques facilitate early diagnosis, a satisfactory efficacy can be achieved in patients with mild to medium severe AAV treated with glucocorticoids and immunosuppressants. However, resistant and relapsing AAV, sometimes life-threatening, do exist in clinical practice. In-depth understanding of pathogenesis of AAV may lend novel insight into the mechanism responsible for its formation and help find effective targeted therapies for refractory patients.