Cyclophosphamide or Rituximab Treatment of Scleritis and Uveitis for Patients with Granulomatosis with Polyangiitis

Abstract

Purpose: Vision-threatening ocular inflammation can be a devastating complication of granulomatosis with polyangiitis (GPA). Here we performed a retrospective observational study to describe the safety and efficacy of treating scleritis and uveitis with either cyclophosphamide or rituximab in GPA. Methods: A chart review of patients diagnosed with GPA-associated scleritis or uveitis, treated with either cyclophosphamide or rituximab as the final therapy at our clinic, was conducted. A total of 1 year of follow-up visits was required for inclusion in the study. Results: Thirteen patients (19 eyes) suffering from GPA-associated scleritis and/or uveitis were identified. As the final therapy, rituximab was administered to 9 patients and cyclophosphamide to 4. Mean duration of follow-up was 55 months (range 16–23 months). Remission was observed in all patients. Three patients had a flare of scleritis after the completion of therapy, and they were restarted on their respective agents. One patient had a flare of retinal vasculitis during rituximab therapy. One patient on cyclophosphamide experienced transient leukopenia. No adverse side effects of rituximab were noted throughout the course of treatment. Conclusions: Cyclophosphamide and rituximab are safe and effective agents for controlling scleritis and uveitis associated with GPA, with eventual progression towards steroid-sparing remission.

Therapy of ocular complications in ANCA+ associated vasculitis

“…The EULAR guideline can be applied in ophthalmological context, as studies and case reports showed efficacy in treating ocular inflammation. For instance, a small study [24] of 13 patients (19 eyes) suffering from scleritis, uveitis and/ or peripheral ulcerative keratitis proved that both cyclophosphamide and rituximab induced remission of the inflammation, with most of the treated eyes (79%) preserving or improving vision at follow-up.…”

Opthalmic Involvement in AAV

“…A recent study by Ahmed et al confirmed that both CYC and RTX were effective in inducing the remission of GPA-associated scleritis and uveitis. Moreover, RTX appeared to be superior in terms of safety and efficacy, with less need for treatment interruptions and dose adjustments [59]. Along with the systemic treatment, local therapeutic agents such as antibiotic, lubricant and/or steroid containing eye drops, and surgical approaches including dacryocystorhinostomy, ocular surface reconstruction by mucosal or amniotic membrane graft, keratoplasty are also used.…”

Biotherapies in Uveitis

“…Rituximab is approved in the treatment of rheumatoid arthritis and granulomatosis with polyangiitis. Ahmed et al [114] observed an efficacy of rituximab alone, without corticosteroid, in 60% of patients (3 of 5 patients), with a mean duration of treatment of 32 months. In Lasave's study [115], 11 patients with posterior refractory uveitis were treated with rituximab alone, without corticosteroid, during a minimal follow-up of 24 months.…”