Rituximab Is Effective in the Treatment of Refractory Ophthalmic Wegener's Granulomatosis

Objective. To investigate the efficacy of rituximab in patients with refractory ophthalmic Wegener's granulomatosis (WG). Methods. Data from 10 consecutive patients with refractory ophthalmic WG treated with rituximab were retrospectively reviewed. In all patients, the ophthalmic disease was driving treatment decisions, and disease activity had persisted despite standard immunosuppressive treatment. Patients had refractory scleritis (n = 3), orbital granulomas causing optic nerve compromise (n = 4), or a combination of both conditions (n = 3). All patients had been treated with at least 3 different immunosuppressive agents, and 5 patients had previously been treated with tumor necrosis factor a blockade. Rituximab was administered intravenously in 2 doses, 2 weeks apart, in combination with standard treatment. Disease activity was monitored clinically by an interdisciplinary approach, including disease activity scoring, immunodiagnostics, and magnetic resonance imaging, as well as by corresponding reductions in the required dose of conventional medication. Results. A beneficial response to treatment with rituximab was seen in all 10 patients, including induction of clinical remission. In all patients, the peripheral blood B cell count fell to zero during treatment with rituximab. Titers of classic antineutrophil cytoplasmic antibodies fell in association with B cell counts, and this reduction was correlated with improved clinical findings. Conclusion. In contrast to previous observations, this study showed that treatment with rituximab was associated with clinical improvement in patients with refractory ophthalmic WG.