Abstract
Vasculitis is defined as the infiltration of inflammatory cells and necrosis of the vessel wall. Vasculitis related to anti-neutrophil cytoplasm antibody (ANCA) is called “ANCA-associated vasculitis (AAV)”, which is consisted of microscopic polyangiitis (MPA), granulomatosis with polyangiitis (GPA) (formerly Wegener's granulomatosis), and Churg-Strauss syndrome. It is clinically important that AAV is a life-threatening and /or debilitating disease and also serious organ dysfunction have been associated not only with vasculitis but also with treatment for vasculitis. In addition, the diagnosis will be experienced a variety of difficult problems. For this reason, the process of exclusion diagnosis is important, such as for infectious diseases, malignant tumors, and rheumatic diseases. As for AAV is systemic disease, it is very important to cooperate with doctors from many clinical fields.