Updates in Pathogenesis and Management of Antineutrophil Cytoplasmic Antibody

AbstractThe ANCA associated vasculitis (AAV) are a group of multisystem diseases characterized by a predominantly primary small vessel vasculitis and the occurrence of ANCA in most, but not all, cases. ANCA are auto antibodies directed against either myeloperoxidase (MPO) or proteinase 3 (PR3) of neutrophils (enzymes found in the primary granules of neutrophils). In general PR3-ANCA &MPO-ANCA are detected in three main diseases {Wegener’s granulomatosis(WG), Microscopic Polyangiitis(MPO), and Churg Strauss Syndrome(CSS) }, plus the renal limited form of vasculitis; idiopathic necrotizing crescentic glomerulonephritis.There was great controversy about the pathogenesis of AAV, whether ANCA were pathogenic in their own right, or it is a matter of autoimmune disease of cellular immune origin (T and B cells).The diagnosis of AAV is made on the basis of the clinical findings, the biopsy of a relevant involved organ and the presence of ANCA in patients’serums.There are rapidly progressive information about the pathogenesis &management of AAV,which help better understanding, what is the nature of the disease , how it occurs & accordingly how it is better managed.of Antineutrophil Cytoplasmic Antibody