Rituximab in refractory ophthalmic Wegener's granulomatosis: PR3 titers may predict relapse, but repeat treatment can be effective

Ophthalmology. 2011 Dec;118(12):2498-503. doi: 10.1016/j.ophtha.2011.06.009. Epub 2011 Sep 9.

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.

Design: Retrospective case series.

Participants: We included 20 consecutive patients with refractory ophthalmic WG treated with RIT.

Intervention: Intravenous RIT infusion, 2 doses of 1 g given 2 weeks apart.

Main outcome measures: 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.

Financial disclosure(s): Proprietary or commercial disclosure may be found after the references.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adult
  • Aged
  • Antibodies, Antineutrophil Cytoplasmic / blood*
  • Antibodies, Monoclonal, Murine-Derived / administration & dosage
  • Antibodies, Monoclonal, Murine-Derived / therapeutic use*
  • Autoantibodies / blood*
  • B-Lymphocytes / immunology
  • Eye Diseases / diagnosis
  • Eye Diseases / drug therapy*
  • Eye Diseases / immunology
  • Female
  • Flow Cytometry
  • Granulomatosis with Polyangiitis / diagnosis
  • Granulomatosis with Polyangiitis / drug therapy*
  • Granulomatosis with Polyangiitis / immunology
  • Humans
  • Immunologic Factors / administration & dosage
  • Immunologic Factors / therapeutic use*
  • Immunosuppressive Agents / therapeutic use
  • Infusions, Intravenous
  • Male
  • Middle Aged
  • Myeloblastin / immunology*
  • Recurrence
  • Retreatment
  • Retrospective Studies
  • Risk Factors
  • Rituximab
  • Treatment Outcome
  • Young Adult

Substances

  • Antibodies, Antineutrophil Cytoplasmic
  • Antibodies, Monoclonal, Murine-Derived
  • Autoantibodies
  • Immunologic Factors
  • Immunosuppressive Agents
  • Rituximab
  • Myeloblastin