Granulomatous polyangiosis (GPA) is a systemic necrotizing granulomatous vasculitis effected the upper-lower airways and the kidney. Small and middle diameter arteries are involved, especially. It is the one of the ANCA associated vasculitis and its characteristic pathological finding is the formation of granulomas. Female and male are affected approximately equally and the average diagnosis is around 40-50 years old. The main symptoms are fever, weakness, sweating, weight loss, myalgia, arthralgia. Respiratory tract, lung, kidney, skin and peripheral nerve involvement are observed. Pulmonary-renal failure, infection and treatment side effects are causes of mortality.