antineutrophil cytoplasmic antibody (ANCA) -associated vasculitis (ANCA-associated vasculitis, AAV) is a group of systemic small vessel vasculitis characterized by the ability to detect ANCA in serum. 1982 Davies et al.[1]This new IgG class antibody was unexpectedly discovered during the study of autoantibodies in the serum of patients with segmental necrotizing glomerulonephritis, which can react with normal human neutrophils. 1985 Van der Wonder et al.[2]This similar autoantibody was found in the sera of patients with Wegener's granulomatosis (WG), and ANCA was gradually widely recognized. 1988 Falk et al.[3]It is found that there are two types of ANCA positive fluorescence staining models, one is the perinuclear pattern (P-ANCA) that produces yellow-green fluorescence around the cell nucleus, and the other is the cytoplasmic pattern (C-ANCA) that produces scattered granular fluorescence in the cytoplasm. It is also found that myeloperoxidase (MPO) is the target antigen of P-ANCA. In 1990, proteinase 3 (PR3) was confirmed as a target antigen of C-ANCA[4]。 Since then, more ANCA target antigens have been gradually discovered, and ANCA has also been found to be closely related to the pathogenesis, clinical manifestations and prognosis of some small vessel vasculitis. The 2012 Chapel Hill Consensus Conference (CHCC) proposed a new classification of vasculitis[5]Encompasses granulomatosis with polyangiitis (GPA; formerly known as WG), microscopic polyangiitis (MPA), eosinophilic granulomatosis with polyangiitis (EGPA; formerly known as Churg-Strauss syndrome) and other small vessel vasculitis are classified as AAV, further proving the important role of ANCA in the pathogenesis and diagnosis and treatment of AAV. With the deepening of the research on AAV, the call for classifying AAV according to the different target antigens of ANCA is also increasing. Therefore, it is more important to detect ANCA in a timely and accurate manner.