Published online Mar 12, 2015. doi: 10.5499/wjr.v5.i1.36
Peer-review started: June 26, 2014
First decision: August 14, 2014
Revised: October 29, 2014
Accepted: November 7, 2014
Article in press: November 10, 2014
Published online: March 12, 2015
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.
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.