Since antineutrophil cytoplasmic autoantibody (ANCA) was found, the researches in the pathogenesis of vasculitis syndrome have been developing. Proinflammatory cytokines including tumor necrosis factor-alpha are induced by certain infections, by which ANCA that binds to granule components of cytoplasm adhesions to endothelial cells. These endothelial cells produce proteolytic enzymes and oxygen radicals, which leads to vasculitis. It might be contributed in the production of ANCA that bacterial organisms are mimicry to the peptide sequences of granule components. The multicentric randomized clinical trials undertaken by the European Vasculitis Study Group (EUVAS) optimized that cyclophosphamide-corticosteroid combination therapy was useful to ANCA-associated vasculitis. It is increasing evidences that biologics are useful to ANCA-associated vasculitis, although it is necessary to take care of severe adverse effects.