Email updates

Keep up to date with the latest news and content from Arthritis Research & Therapy and BioMed Central.

Review

Progress in treatment of ANCA-associated vasculitis

Rona M Smith*, Rachel B Jones and David RW Jayne

Author Affiliations

Box 57, Department of Renal Medicine, Addenbrooke's Hospital, Hills Road, Cambridge CB20QQ, UK

For all author emails, please log on.

Arthritis Research & Therapy 2012, 14:210  doi:10.1186/ar3797

Published: 30 April 2012

Abstract

Autoantibodies to neutrophil cytoplasmic antigen-associated vasculitis (AAV) is characterised by inflammation of blood vessels. The introduction of immunosuppressive therapy with glucocorticoids and cyclophosphamide transformed AAV from a fatal condition to a largely treatable condition. Over the past 30 years, considerable progress has been made refining immunosuppressive regimens with a focus on minimising toxicity. There is, however, a high unmet need in the treatment of AAV. A proportion of patients are refractory to current therapies; 50% experience a relapse within 5 years and treatment toxicity contributes to mortality and chronic disability. As knowledge of the pathogenesis of vasculitis grows, it is mirrored by the availability of biological agents, which herald a revolution in the treatment of vasculitis. Lymphocyte-targeted and cytokine-targeted agents have been evaluated for the treatment of AAV and are entering the routine therapeutic arena with the potential to improve patient outcomes. As rare diseases, treatment advances in vasculitis depend on international collaborative research networks both to establish an evidence base for newer agents and to develop recommendations for patient management.