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Semin Arthritis Rheum. 2014 Feb;43(4):542-57. doi: 10.1016/j.semarthrit.2013.07.010. Epub 2013 Aug 24.

Biological therapy for systemic vasculitis: a systematic review.

Author information

  • 1Rheumatology Department, Hospital Universitario de Guadalajara, Guadalajara, Spain. Electronic address: luciasilva@ser.es.
  • 2Institute for Musculoskeletal Health, Madrid, Spain.
  • 3Rheumatology Department, Hospital Universitario Marqués de Valdecilla, Facultad de Medicina, Universidad de Cantabria, Santander, Spain.
  • 4Rheumatology Department, Hospital Universitario Doctor Negrín, Las Palmas de Gran Canaria, Spain.
  • 5Rheumatology Department, Complejo Hospitalario Universitario de Vigo, Instituto de Investigación Biomédica de Vigo, Vigo, Spain.
  • 6Rheumatology Department, Hospital Universitario Infanta Sofía, San Sebastián de los Reyes, Spain.



Relapses and failure are frequent in systemic vasculitis (SV) patients. Biological agents have been prescribed as rescue therapies. The aim of this systematic review is to analyze the current evidence on the therapeutic use of biological agents for SV.


MEDLINE, EMBASE, the Cochrane Database of Systematic Reviews, and the Cochrane Central Register of Controlled Trials were searched up to the end of April 2013. Systematic reviews and meta-analysis, clinical trials, cohort studies, and case series with >3 patients were included. Independent article review and study quality assessment was done by 2 investigators with consensus resolution of discrepancies.


Of 3447 citations, abstracts, and hand-searched studies screened, 90 were included. Most of the studies included ANCA-associated vasculitis (AAV) patients and only a few included large vessel vasculitis (LVV) patients. Rituximab was the most used agent, having demonstrated efficacy for remission induction in patients with AAV. A number of studies used different anti-TNFα agents with contrasting results. A few uncontrolled studies on the use of abatacept, alemtuzumab, mepolizumab, and tocilizumab were found.


Current evidence on the use of biological therapies for SV is mainly based on uncontrolled, observational data. Rituximab is not inferior to cyclophosphamide for remission induction in AAV and might be superior in relapsing disease. Infliximab and adalimumab are effective as steroid-sparing agents. Etanercept is not effective to maintain remission in patients with granulomatosis with polyangiitis, and serious adverse events have been reported. For LVV, both infliximab and etanercept had a role as steroid-sparing agents, and tocilizumab might be effective also for remission induction in LVV.

Copyright © 2014 Elsevier Inc. All rights reserved.


ANCA-associated vasculitis; Biological therapy; Large vessel vasculitis; Systematic review

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