Posterior Scleritis in Familial Mediterranean Fever

Purpose: Familial Mediterranean fever (FMF) is a monogenic autoinflammatory disease presenting as sporadic paroxysmal attacks of fever and abdominal pain. The inflammation of serosal spaces, joints, and skin is caused by the production of an abnormal protein called pyrin. Ocular pathology is scarce in FMF. Case Report: Herein we describe a case of FMF presenting with painful loss of vision in the left eye. Serous macular detachment assessed by OCT, leaky pinpoint subretinal foci temporal to the fovea examined by fluorescein angiography, scleral and choroidal thickening seen on ultrasonography, and a negative systemic workup for vasculitis established the diagnosis of FMF-related posterior scleritis. The posterior scleritis responded promptly to moderate-dose oral corticosteroids with return of vision to baseline and resolution of the subretinal fluid. Conclusions: FMF rarely involves the posterior pole. Visual loss in FMF results from either posterior scleritis or posterior uveitis. A high degree of suspicion of posterior scleritis is warranted in female patients with known FMF presenting with cloudy serous macular detachment. (C) 2019 The Author(s) Published by S. Karger AG, Basel