Symptomatic Occlusive Retinal Vasculitis as an Unusual Presentation of Biopsy-Proven Sarcoidosis: A Case Series

The presentation of ocular sarcoidosis may involve different parts of the eye and/or adnexal tissues. Uveitis is the most common manifestation, and when it involves the posterior segment, it often presents as peripheral multifocal choroiditis. The occurrence of symptomatic occlusive retinal vasculitis (ORV) associated with ocular sarcoidosis is not well described in the literature. The objective of this article is to describe well characterized cases of biopsy-proven ocular sarcoidosis presenting with symptomatic ORV.

We conducted a retrospective study using the ophthalmological database, to include patients with symptomatic ORV and biopsy-proven sarcoidosis, who were followed at a tertiary ophthalmology and internal medicine center. We described analysis of ocular and systemic manifestations at baseline and response to local and systemic treatments.

We identified five cases, all male, including four from sub-Saharan Africa and one from France. Uveitis affected both eyes in all cases, and none showed intermediate uveitis, panuveitis, or peripheral multifocal choroiditis. Ocular manifestations were consistently associated with constitutional symptoms and pulmonary involvement, with a marked increase in acute phase reactants in all cases and lymphocytic meningitis in 2/5. All patients responded well to local and systemic glucocorticoids (GCs), with two patients showing GC dependence requiring the addition of an immunosuppressant.

We provide a comprehensive analysis of the clinical findings and management strategies of newly-diagnosed sarcoidosis presenting with symptomatic ORV. Although such cases are unusual, the diagnosis of sarcoidosis should be considered when ORV is encountered.