title: Biological therapy for systemic vasculitis: a systematic review.
abstract: OBJECTIVE
  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.
  
  
  METHODS
  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.
  
  
  RESULTS
  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.
  
  
  CONCLUSIONS
  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.
authors: Rheumatology Department, Hospital Universitario de Guadalajara, Guadalajara, Spain. Electronic address: luciasilva@ser.es.
authors: Lucía
authors: L
authors: Silva-Fernández
authors: Institute for Musculoskeletal Health, Madrid, Spain.
authors: Estíbaliz
authors: E
authors: Loza
authors: Rheumatology Department, Hospital Universitario Marqués de Valdecilla, Facultad de Medicina, Universidad de Cantabria, Santander, Spain.
authors: Víctor M
authors: VM
authors: Martínez-Taboada
authors: Rheumatology Department, Hospital Universitario Marqués de Valdecilla, Facultad de Medicina, Universidad de Cantabria, Santander, Spain.
authors: Ricardo
authors: R
authors: Blanco
authors: Rheumatology Department, Hospital Universitario Doctor Negrín, Las Palmas de Gran Canaria, Spain.
authors: Iñigo
authors: I
authors: Rúa-Figueroa
authors: Rheumatology Department, Complejo Hospitalario Universitario de Vigo, Instituto de Investigación Biomédica de Vigo, Vigo, Spain.
authors: José María
authors: JM
authors: Pego-Reigosa
authors: Rheumatology Department, Hospital Universitario Infanta Sofía, San Sebastián de los Reyes, Spain.
authors: Santiago
authors: S
authors: Muñoz-Fernández
mesh_terms: D000068879:Adalimumab; D000911:Antibodies, Monoclonal / Q000627:therapeutic use; D061067:Antibodies, Monoclonal, Humanized / Q000627:therapeutic use; D058846:Antibodies, Monoclonal, Murine-Derived / Q000627:therapeutic use; D001688:Biological Products / Q000627:therapeutic use*; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D000069285:Infliximab; D012074:Remission Induction; D000069283:Rituximab; D056647:Systemic Vasculitis / Q000188:drug therapy*; D016896:Treatment Outcome
chemical_list: D000911:Antibodies, Monoclonal; D061067:Antibodies, Monoclonal, Humanized; D058846:Antibodies, Monoclonal, Murine-Derived; D001688:Biological Products; D007166:Immunosuppressive Agents; D000069283:Rituximab; D000069285:Infliximab; D000068879:Adalimumab; C502936:tocilizumab
keywords: ANCA-associated vasculitis; Biological therapy; Large vessel vasculitis; Systematic review