The Spectrum of Uveitis and Retinal Vasculitis in Systemic Sclerosis: A Case Series

PURPOSE: To describe the spectrum of ocular inflammation in a series of patients with systemic sclerosis. METHODS: Retrospective case series of six patients diagnosed with systemic sclerosis presenting with either uveitis or retinal vasculitis. RESULTS: Presenting symptoms included blurry vision, flashes, floaters, photophobia, and eye pain. Visual acuity at presentation ranged from 20/20 to 20/125. Retinal vascular involvement was present in all patients. One patient was symptomatic in only one eye but was found to have bilateral retinal vasculitis. Two patients had findings consistent with retinal artery occlusion. One patient presented with posterior uveitis, one with panuveitis, and two initially presented with anterior uveitis and later developed retinal vasculitis. All six patients required systemic corticosteroid therapy. CONCLUSION: Systemic sclerosis in adults rarely causes ocular inflammation; when present, it is most often associated with retinal vasculitis. This is the first case series to report active retinal vasculitis in association with systemic sclerosis. Patients may be asymptomatic despite significant vascular leakage. Retinal vasculitis should be considered in patients with systemic sclerosis who develop ocular complaints.