Systemic vasculitis refers to a condition of blood vessel inflammation, of which the causes are various. In a substantial number of cases, autoantibodies against neutrophil cytoplasm constituents (ANCAs) are present. The authors then refer to the systemic vasculitis as ANCA-associated systemic vasculitis. Renal disease is an unfavorable component, leading to dialysis dependency in a considerable number of patients. This review aims to summarize in brief what was reported about ANCA-associated vasculitis in the recent past. What the exact pathogenic role of ANCAs in the development of systemic vasculitis is remains uncertain, and it is still not clear how their presence leads to the histopathologic lesions called vasculitis.