Abstract: As a group of systemic autoimmune diseases, anti-neutrophil cytoplasmic antibody-associated vasculitis （AAV） is characterized by necrotizing inflammation and fibrinoid necrosis of small blood vessels in multiple systems and positive serum antibodies to anti-neutrophil cytoplasmic antibody. Renal injury is common in this disease. Major clinical manifestations are hematuria, proteinuria and varying degrees of renal impairment. Glucocorticoids plus immunosuppressive agents are a conventional treatment for AAV. However, this regimen has a high relapse rate and numerous adverse events. Seeking a safer, more effective and comprehensive treatment, this review summarized the latest advances in the diagnosis and treatment of AAV.