Abstract: Anti-neutrophil cytoplasm antibody-associated vasculitis (ANCA) is an autoimmune disease with multi organ involvement characterized by vascular wall inflammation and fibrinoid necrosis, including microscopic polyangitis (MPA), granuloma polyangitis (GPA), and eosinophilic granuloma polyangitis (EGPA). Because its clinical manifestations are complicated and non-specific, it is difficult to make early diagnose. In recent years, some new progress has been made in diagnosis and treatment of this disease. The article will review the related information.