title: Rituximab in refractory ophthalmic Wegener's granulomatosis: PR3 titers may predict relapse, but repeat treatment can be effective.
abstract: OBJECTIVE
  To report the long-term outcome of the treatment of refractory ophthalmic Wegener's granulomatosis (WG) with rituximab (RIT), including rates of relapse, predictors of relapse, and results of repeat treatment.
  
  
  METHODS
  Retrospective case series.
  
  
  METHODS
  We included 20 consecutive patients with refractory ophthalmic WG treated with RIT.
  
  
  METHODS
  Intravenous RIT infusion, 2 doses of 1 g given 2 weeks apart.
  
  
  METHODS
  Regular clinical, serologic, and immunologic examinations for disease activity and extent, and for treatment-related side effects.
  
  
  RESULTS
  All 20 patients entered remission, the median time to remission being 2 months (range, 1-6). Seven patients (35%) relapsed at a median of 13 months (range, 9-18). Five of these patients took a second course of RIT, and all achieved remission without further relapse. In the 16 patients with positive anti-proteinase-3 (PR3) titers at baseline, rising anti-PR3 titer was a statistically significant predictor of relapse. There were 4 severe adverse events during the study, of which one was directly attributed to treatment with RIT.
  
  
  CONCLUSIONS
  In this series of 20 patients with refractory ophthalmic WG, RIT was effective in inducing remission. Relapse occurred in one third of patients within 18 months and seemed to be predictable by rising anti-PR3 titers, but retreatment with RIT was effective in this group. In patients with ophthalmic WG, RIT may be capable of inducing extended remission, in contrast with other biologic and conventional treatments in common use.
  
  
  BACKGROUND
  Proprietary or commercial disclosure may be found after the references.
authors: UCL Institute of Ophthalmology, London, United Kingdom.
authors: Lavnish
authors: L
authors: Joshi
authors: Sue L
authors: SL
authors: Lightman
authors: Alan D
authors: AD
authors: Salama
authors: Amy Lee
authors: AL
authors: Shirodkar
authors: Charles D
authors: CD
authors: Pusey
authors: Simon R J
authors: SR
authors: Taylor
mesh_terms: D000328:Adult; D000368:Aged; D019268:Antibodies, Antineutrophil Cytoplasmic / Q000097:blood*; D058846:Antibodies, Monoclonal, Murine-Derived / Q000008:administration & dosage / Q000627:therapeutic use*; D001323:Autoantibodies / Q000097:blood*; D001402:B-Lymphocytes / Q000276:immunology; D005128:Eye Diseases / Q000175:diagnosis / Q000188:drug therapy* / Q000276:immunology; D005260:Female; D005434:Flow Cytometry; D014890:Granulomatosis with Polyangiitis / Q000175:diagnosis / Q000188:drug therapy* / Q000276:immunology; D006801:Humans; D007155:Immunologic Factors / Q000008:administration & dosage / Q000627:therapeutic use*; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D007262:Infusions, Intravenous; D008297:Male; D008875:Middle Aged; D053146:Myeloblastin / Q000276:immunology*; D012008:Recurrence; D019233:Retreatment; D012189:Retrospective Studies; D012307:Risk Factors; D000069283:Rituximab; D016896:Treatment Outcome; D055815:Young Adult
chemical_list: D019268:Antibodies, Antineutrophil Cytoplasmic; D058846:Antibodies, Monoclonal, Murine-Derived; D001323:Autoantibodies; D007155:Immunologic Factors; D007166:Immunosuppressive Agents; D000069283:Rituximab; D053146:Myeloblastin