Classification, diagnosis and treatment of ANCA-associated vasculitis

Abstract

Diagnosis of anti-neutrophil cytoplasmic antibodies (ANCA)-associated vasculitis is usually not difficult in patient with systemic disease, including lung and kidneys involvement, and laboratory signs of inflammation. The presence of ANCA and the results of histological investigation confirm diagnosis of ANCA-associated vasculitis. Cyclophosphamide/azathioprine in combination with high dose steroids are used to induce and maintain remission of systemic vasculitis. The clinical trials also showed efficacy of rituximab that induces depletion of B-cells. Our understanding and management of ANCA-associated vasculitis improved significantly over the last decades but there is still a lot of debate over its classification, diagnostic criteria, assessment of activity and optimum treatment.

Keywords: Systemic vasculitis; Anti-neutrophil cytoplasmic antibodies; Granulomatosis with polyangiitis; Microscopic polyangiitis

Core tip: The diagnosis and treatment of anti-neutrophil cytoplasmic antibodies-associated vasculitis are a challenge for physicians. This article presents an updated information about these uncommon diseases.