Abstract
Purpose :
Vision-threatening ocular inflammation can be a complication of granulomatosis with polyangiitis (GPA) that is difficult to control. Here we performed a retrospective observational study to describe the safety and efficacy of treating scleritis and uveitis seen in patients with GPA with either cyclophosphamide or rituximab.
Methods :
A chart review of patients with GPA-associated scleritis or uveitis, refractory to steroids and treated with either cyclophosphamide or rituximab as final therapy, was conducted. Patients were searched from the Massachusetts Eye Research and Surgery Institution database between 2006 and 2016. A total of six months of follow up visits was required for inclusion in the study. Demographic dates, serology, previous therapy, and treatment outcomes were individually assessed. Remission was defined as showing no signs of active ocular inflammation on exam, in the presence or absence of therapy.
Results :
Thirteen patients (19 eyes) suffering from GPA-associated scleritis and/or uveitis were identified. The average age was 61 years, with a total of 1 male and 12 females. Of note, 12 patients were of Causasian background, and 1 patient of Asian background. Rituximab was administered as final therapy to 10 patients while cyclophosphamide was administered as final therapy to 3 patients. Seven patients (54%) on rituximab as final therapy were previously on cyclophosphamide. Only one patient on cyclophosphamide was previously on rituximab. Mean duration of follow-up was 56 months (range: 16-123 mo). Remission was observed in all patients. Four of these patients were taken off of therapy due to completion of a predetermined protocol (31%), two from each treatment group. Average time on therapy until resolution of inflammation was 12 weeks (SEM = 2.8) on rituximab, and 29 weeks (SEM = 4.8) on cyclophosphamide. One patient had a recurrence of scleritis four years after achieving remission on cyclophosphamide and was restarted on therapy. The most common inflammatory ocular co-morbidity was peripheral ulcerative keratitis (PUK), found in six patients. No adverse side effects to therapy were noted throughout course of treatment.
Conclusions :
Cyclophosphamide and rituximab are safe and effective agents for controlling scleritis and uveitis associated with steroid refractory GPA, with eventual progression towards remission.
This is an abstract that was submitted for the 2017 ARVO Annual Meeting, held in Baltimore, MD, May 7-11, 2017.