title: Long-term safety and efficacy of rituximab in 7 Japanese patients with ANCA-associated vasculitis.
abstract: OBJECTIVE
  The safety and efficacy of rituximab were examined in a multicenter open-label pilot study in patients with anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) in Japan.
  
  
  METHODS
  Patients with refractory AAV were administered a rituximab infusion at a weekly dose of 375 mg/m(2) for 4 weeks. All patients also received oral daily prednisolone. The primary outcome was complete remission, which was defined as a Birmingham Vasculitis Activity Score (BVAS) of 0 or 1.
  
  
  RESULTS
  The mean age of the 7 patients was 57 (range, 34-71) years. The mean follow-up period after rituximab treatment was 62.9 (range, 4.8-81) months. The mean BVAS at entry was 16.7 (range, 2-34). Complete remission occurred in all cases, except in 1 case in which the patient died, with a significant decline in BVAS from baseline at 12 months after initiation of rituximab. Rituximab reduced granulomatous orbital involvement in a patient with granulomatosis with polyangiitis. Relapse occurred in five patients. Adverse events included de novo hepatitis B in one patient, cancer (hepatocellular carcinoma and prostate cancer) in two patients, and transient visual disturbance, atypical mycobacterial infection, urinary tract infection, sepsis, and cytomegalovirus infection. Two patients died due to recurrent infections and airway obstruction, caused by an AAV lesion.
  
  
  CONCLUSIONS
  Rituximab had a beneficial effect on refractory AAV in Japanese patients, but several adverse effects occurred during rituximab treatment.
authors.affiliation: Division of Rheumatology, Department of Internal Medicine, St. Marianna University School of Medicine , Miyamae-ku, Kawasaki , Japan.
authors.forename: Hiroko
authors.initials: H
authors.lastname: Nagafuchi
authors.forename: Tatsuya
authors.initials: T
authors.lastname: Atsumi
authors.forename: Kazuhiro
authors.initials: K
authors.lastname: Hatta
authors.forename: Eri
authors.initials: E
authors.lastname: Muso
authors.forename: Mitsuhiro
authors.initials: M
authors.lastname: Takeno
authors.forename: Hidehiro
authors.initials: H
authors.lastname: Yamada
authors.forename: Shoichi
authors.initials: S
authors.lastname: Ozaki
mesh_terms: D000328:Adult; D000368:Aged; D056648:Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis / Q000188:drug therapy* / Q000453:epidemiology / Q000276:immunology; D019268:Antibodies, Antineutrophil Cytoplasmic / Q000276:immunology*; D004305:Dose-Response Relationship, Drug; D005260:Female; D006801:Humans; D007155:Immunologic Factors / Q000008:administration & dosage; D015994:Incidence; D007564:Japan / Q000453:epidemiology; D008297:Male; D008875:Middle Aged; D010865:Pilot Projects; D012008:Recurrence; D012074:Remission Induction; D000069283:Rituximab / Q000008:administration & dosage*; D016896:Treatment Outcome
chemical_list: D019268:Antibodies, Antineutrophil Cytoplasmic; D007155:Immunologic Factors; D000069283:Rituximab
keywords: ANCA-associated vasculitis; Efficacy; Rituximab; Safety