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

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.Retrospective case series.We included 20 consecutive patients with refractory ophthalmic WG treated with RIT.Intravenous RIT infusion, 2 doses of 1 g given 2 weeks apart.Regular clinical, serologic, and immunologic examinations for disease activity and extent, and for treatment-related side effects.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.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.Proprietary or commercial disclosure may be found after the references.