title: Rituximab with or without a conventional maintenance agent in the treatment of relapsing granulomatosis with polyangiitis (Wegener's): a retrospective single-center study.
abstract: OBJECTIVE
  To evaluate the efficacy and safety of rituximab (RTX) induction therapy and the duration of remission, when RTX is used with or without a conventional maintenance agent, in a cohort of patients with granulomatosis with polyangiitis (Wegener's) (GPA).
  
  
  METHODS
  This was a retrospective, single-center study of patients with relapsing GPA treated with at least 1 course of RTX (4 weekly doses of 375 mg/m(2) intravenously [IV] or 2 fixed doses of 1,000 mg IV 2 weeks apart). Complete remission was defined as the absence of disease activity measured by a Birmingham Vasculitis Activity Score for Wegener's granulomatosis of 0 and not qualified by the prednisone dosage at the time.
  
  
  RESULTS
  Eighty-nine patients achieved remission after their first course of RTX and were not re-treated preemptively with RTX to maintain remission of their disease during followup. Among these patients, relapse-free survival was significantly higher in those who received a conventional maintenance agent (azathioprine, methotrexate, or mycophenolate mofetil) in conjunction with RTX and glucocorticoids (n = 47) than in those who received no additional immunosuppressive agent (n = 42) (P = 0.04). The hazard ratio of relapse in those receiving a maintenance agent was 0.53 (95% confidence interval 0.29-0.97). Serious adverse events did not differ between the 2 groups. Within a subset of 15 patients in the cohort who were relapse free 2 years after 1 course of RTX, remissions endured for 2-6 years in 8 patients.
  
  
  CONCLUSIONS
  RTX is an effective remission-inducing agent in GPA. The addition of a conventional maintenance agent to RTX and glucocorticoids decreased the incidence of relapse and did not result in a higher incidence of adverse events.
authors.affiliation: Cleveland Clinic, Cleveland, Ohio.
authors.forename: Lama
authors.initials: L
authors.lastname: Azar
authors.forename: Jason
authors.initials: J
authors.lastname: Springer
authors.forename: Carol A
authors.initials: CA
authors.lastname: Langford
authors.forename: Gary S
authors.initials: GS
authors.lastname: Hoffman
mesh_terms: D000328:Adult; D000368:Aged; D058846:Antibodies, Monoclonal, Murine-Derived / Q000627:therapeutic use*; D001379:Azathioprine / Q000627:therapeutic use*; D004359:Drug Therapy, Combination; D005260:Female; D014890:Granulomatosis with Polyangiitis / Q000188:drug therapy*; D006801:Humans; D007155:Immunologic Factors / Q000627:therapeutic use*; D008297:Male; D008727:Methotrexate / Q000627:therapeutic use*; D008875:Middle Aged; D009173:Mycophenolic Acid / Q000031:analogs & derivatives* / Q000627:therapeutic use; D012074:Remission Induction; D012189:Retrospective Studies; D000069283:Rituximab; D016896:Treatment Outcome
chemical_list: D058846:Antibodies, Monoclonal, Murine-Derived; D007155:Immunologic Factors; D000069283:Rituximab; D009173:Mycophenolic Acid; D001379:Azathioprine; D008727:Methotrexate