Background/Purpose: Primary retinal vasculitis (PRV) refers to inflammation in the retinal vasculature without any infectious etiology, systemic disease association, or concomitant ocular disease. PRV is a diagnosis of exclusion. Retinal vasculitis patients usually undergo a thorough diagnostic evaluation to search for an underlying systemic association and often require visits from multiple specialists, including ophthalmologists and rheumatologists. Prompt identification and treatment of patients with PRV can often be challenging, yet it is necessary to prevent any significant vision loss. The primary goal of this study is to identify the clinical characteristics of patients with PRV and treatment strategies used at a major tertiary center.

Methods: The Yale University Human Investigation Committee approved this retrospective study of patients with primary retinal vasculitis that were seen at the Yale Eye Center. Patients with any infectious causes, autoimmune diseases, or concurrent inflammatory ocular pathology were excluded from this study. Patient demographic information, including age at diagnosis, medical history, smoking history, and gender, were included for each subject. For each eye with retinal vasculitis, visual chief complaint, exam findings on dilated fundoscopic exam (DFE) and intravenous fluorescein angiography (IVFA), topical and systemic agents for treatment, duration of treatment, and dates of recurrence and remission were recorded.

Results: Thirty eyes from fifteen patients were included in this study. The mean age of PRV patients was 57±19 years (y). Sixty percent of the patients were female. The most common presenting symptoms for these patients were blurry vision and floaters. Each patient underwent comprehensive lab testing, including RPR, QuantiFERON-TB Gold, ACE, ANA, and ANCA, with three patients having an incidentally positive ANA. Two patients had type II diabetes, while three patients had hypertension. One patient was an active smoker, while three were former smokers. Twenty-two eyes (73%) had visual acuity of 20/40 or better on presentation. Eight eyes were treated with topical prednisolone drops for concurrent macular leakage, and intravitreal dexamethasone was used to treat inflammation in twelve eyes. Fifty-three percent (53%) of patients required systemic steroids, and for immunomodulatory therapies (IMT), 33% of patients were on methotrexate, 27% on mycophenolate mofetil, 20% on adalimumab, and 33% on rituximab. Three patients were able to discontinue systemic therapy after an average of 24 months, while six eyes experienced recurrent vasculitis flare while on IMT.

Conclusion: PRV often requires a multimodal treatment strategy for controlling inflammation with the use of systemic therapies and topical therapies for macular leakage. There can be wide variability in duration of therapy and treatment outcomes for these patients with PRV. Hence, more refined studies and algorithms are needed to identify and treat patients with PRV.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/characteristics-of-patients-with-primary-retinal-vasculitis-at-a-tertiary-referral-center/