Copyright ©The Author(s) 2015. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Rheumatol. Mar 12, 2015; 5(1): 36-44
Published online Mar 12, 2015. doi: 10.5499/wjr.v5.i1.36
Classification, diagnosis and treatment of ANCA-associated vasculitis
Sergey V Moiseev, Pavel I Novikov
Sergey V Moiseev, Pavel I Novikov, Clinic of Nephrology, Internal and Occupational Diseases, The Sechenov First Moscow State Medical University, Moscow 119435, Russia
Author contributions: Both the authors contributed to this paper.
Open-Access: This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See:
Correspondence to: Sergey V Moiseev, MD, Professor, Clinic of Nephrology, Internal and Occupational Diseases, The Sechenov First Moscow State Medical University, Rossolimo, 11/5, Moscow 119435, Russia.
Telephone: +7-495-2482544 Fax: +7-901-5904491
Received: June 25, 2014
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.

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.