Eye disease in Behçet Syndrome

Abstract

Ocular involvement is the main cause of morbidity in Behçet syndrome (BS). While the overall frequency is -50%, the young males have -70%, while the older females have -20% chance of acquiring eye disease. It starts within the first few years of the disease, involves both eyes in the majority of the patients, and runs a more severe course in males. It is a nongranulomatous panuveitis and retinal vasculitis that has a periodic course characterized by activations and remissions. Impairment or blurring of vision, redness, and ocular discomfort are the main symptoms during an attack which usually subsides within a few weeks. Cellular infiltration within the anterior chamber is a common presenting sign. It can be accompanied by exudation of protein from the inflamed iris vessels. When the cellular infiltrate and fibrinous deposits are extensive, a hypopyon is formed due to gravity. Recurrent inflammatory activity in the anterior chamber may lead to anterior and posterior synechiae, secondary glaucoma, and cataract. Vitreal inflammatory signs in the form of haze and cells are the main inflammatory findings of posterior segment involvement. Occlusive retinal vasculitis, which is the hallmark of ocular disease of BS, almost always accompanies posterior segment involvement. During activations, funduscopic examination may show vitreal deposits, retinal infiltrates, retinal edema, hemorrhages, and macular edema. In advanced stages, the visual loss becomes permanent and sheathing of blood vessels, ghost vessels, atrophic and pigmentary macular changes, and optic atrophy are seen. Visual prognosis of BS has dramatically improved in the last two to three decades by better use of conventional immunosuppressives such as cyclosporine and azathioprine and more recently biologics like anti-TNF alpha agents and interferon alpha. Ophthalmic surgery for secondary cataract and glaucoma as well as for vitreoretinal complications has also become much more successful.