Abstract:

Anti-neutrophil cytoplasmic antibodies (ANCA) are a class of autoantibodies that target the cytoplasmic components of neutrophils and monocytes. ANCA includes cytoplasmic ANCA (c-ANCA), perinuclear ANCA (p-ANCA) and atypical ANCA (a-ANCA). The target antigens of c-ANCA and p-ANCA are proteinase 3 and myeloperoxidase, respectively. At present, the ANCA detection assay mainly includes indirect immunofluorescence, enzyme-linked immunosorbent assay, chemiluminescent immunoassays and fluorescent-enzyme immunoassays. ANCA associated vasculitis (AAV) is a necrotizing vasculitis mainly involving small and medium sized arteries with pauci-immune deposits. The incidence of AAV is low in children, but the onset of the disease is urgent, the progression is rapid, the clinical manifestations are diverse, and the disease is easy to relapse after drug withdrawal. ANCA has become an important marker for the diagnosis of granulomatous vasculitis, microscopic vasculitis and eosinophilic granulomatous vasculitis. ANCA detection is helpful for the early diagnosis and treatment of AAV, thus improving the prognosis of the disease.

Key words: anti-neutrophil cytoplasmic antibody, proteinase 3, myeloperoxidase, ANCA-associated vasculitis