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ANCA-Associated Vasculitides The according to their immunoserological markers (anti-neutrophil cytoplasmic antibodies - ANCA) named ANCA-associated vasculitides (AAV) are classified following the Chapel Hill nomenclature (2012). Microscopic polyangiitis (MPA), granulomatosis with polyangiitis (GPA), and eosinophilic granulomatosis with polyangiitis (EGPA) are categorized according to clinical, histological, and imaging findings. GPA and EGPA mainly differ from MPA in the presence of granulomatous inflammation within the airways. All three are rare autoimmune diseases and their prevalences show geographical differences. Despite their rarity, recognition of the typical symptoms is very important. A timely diagnosis is crucial, as without immunosuppressive treatment the prognosis with respect to preservation of organ function and survival is poor. New treatments with potentially fewer side effects have been introduced in recent years. Amongst those Rituximab plays an important role and has largely replaced cyclophosphamide. Now the aim of therapeutic approaches is to reduce patient exposure to steroids. Because the side effects of therapy and especially steroids are the main causes of AAV morbidity besides the disease itself.