Ophthalmic manifestations in familial Mediterranean fever: a case series of 6 patients.

PURPOSE: To describe the ocular involvement of patients with familial Mediterranean fever (FMF) followed in a tertiary referral center.

METHODS: The data of 6 patients with FMF were retrospectively reviewed. Detailed ophthalmologic examinations, type of inflammation, course of the disease, number of recurrences, treatment regimens, complications, and comorbid ocular or systemic diseases were noted.

RESULTS: The mean age ± SD at diagnosis was 29.3 ± 19.3 (4-53) years. A total of 66.7% of the patients were male and 66.7% of the patients had bilateral disease. The anatomical distribution of the ophthalmic involvement was as follows: posterior uveitis in 2 (33.3%), anterior uveitis in 2 (33.3%), posterior scleritis in 1 (16.7%), and intermediate uveitis in 1 (16.7%) patient. The course was recurrent in 50% of the patients. Final visual acuities were favorable except in the patients with chronic course uveitis. Cystoid macula edema, epiretinal membrane, retinal ischemia, cataract, glaucoma, and band keratopathy were complications noted in the follow-up period. Both cataract and glaucoma patients (50%) needed a surgical intervention. In 33.3% of patients, Behçet disease was present as a concurrent disease. In patients with posterior uveitis and the patient with intermediate uveitis (50%), systemic immunosuppression was required.

CONCLUSIONS: There was a male and bilateral involvement predominance. The course of the inflammation was recurrent in half of the patients. Since ocular involvement in FMF is very rare, it should be considered as diagnosis of exclusion.