[Treatment of anti-neutrophil cytoplasmic antibody related vasculitis]

ANCA-associated vasculitides are a well-known clinico-pathological group of systemic diseases comprising microscopic poliangiitis, granulomatosis with poliangiitis and eosinophilic granulomatosis with poliangiitis. This article shows contemporary treatment of this diseases with extensive literature review. Stepwise treatment of ANCA-associated vasculitides is divided into induction therapy and remission maintenance therapy. Standard induction therapy is a combination of glucocorticoids and cyclophosphamide, and in maintenance therapy, combination of low-dose glucocorticoids and azathioprine or methotrexate is used. Leading rheumatology and nephrology associations developed treatment guidelines. Since ANCA-associated vasculitides are relatively rare diseases, there are only few randomized controlled studies to provide high level of evidence and treatment recommendations. Most patients achieve remission, but relapses often occur. The main treatment considerations, apart from frequently relapsing disease, are disease refractory to treatment and potentially harmful effects of immunosuppressants, especially cyclophosphamide. Future studies are needed to determine the effects of less toxic immunosuppressants, mainly biological agents.