title: B-cell-targeted therapy in systemic vasculitis.
abstract: OBJECTIVE
  The present review discusses the evidence supporting the use of B-cell-targeted therapy in the treatment of various forms of systemic vasculitis with a focus on the use of rituximab (RTX), in the antineutrophil cytoplasmic autoantibody (ANCA)-associated vasculitides (AAV).
  
  
  RESULTS
  Long-term follow-up of the two studies establishing the efficacy of a RTX-based induction regimen for severe AAV have demonstrated noninferiority of a single course of RTX compared with conventional therapy for remission maintenance. In addition, these observations highlight an association between relapse and B-cell reconstitution in patients treated with RTX. The maintenance of remission using rituximab in systemic ANCA-associated vasculitis trial compared a regimen of RTX infusions every 6 months to azathioprine for remission maintenance and concluded that serial RTX lead to higher rates of sustained remission.RTX is also an established therapy for cryoglobulinemic vasculitis associated with hepatitis C viral (HCV) infection. Recently published data support the long-term efficacy and safety of RTX for cryoglobulinemic vasculitis.
  
  
  CONCLUSIONS
  B-cell depletion with RTX is an established therapy for both remission induction and maintenance in severe AAV and in HCV-related cryoglobulinemic vasculitis. There are limited data to support use of B-cell-targeted therapy in refractory cases of other forms of systemic vasculitis such as eosinophilic granulomatosis with polyangiitis and Takayasu's arteritis.
authors.affiliation: Hospital for Special Surgery, New York, New York, USA.
authors.forename: Lindsay
authors.initials: L
authors.lastname: Lally
authors.forename: Robert
authors.initials: R
authors.lastname: Spiera
mesh_terms: D056648:Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis / Q000188:drug therapy* / Q000276:immunology* / Q000628:therapy; D001402:B-Lymphocytes / Q000276:immunology*; D015267:Churg-Strauss Syndrome / Q000188:drug therapy / Q000276:immunology; D003449:Cryoglobulinemia / Q000188:drug therapy / Q000276:immunology; D014890:Granulomatosis with Polyangiitis / Q000188:drug therapy / Q000276:immunology; D006801:Humans; D007155:Immunologic Factors / Q000627:therapeutic use; D055953:Microscopic Polyangiitis / Q000188:drug therapy / Q000276:immunology; D058990:Molecular Targeted Therapy; D000069283:Rituximab / Q000627:therapeutic use; D056647:Systemic Vasculitis / Q000188:drug therapy* / Q000276:immunology* / Q000628:therapy; D013625:Takayasu Arteritis / Q000188:drug therapy / Q000276:immunology
chemical_list: D007155:Immunologic Factors; D000069283:Rituximab