Eye Disease in Behçet’s Syndrome

Abstract

Ocular involvement is the main cause of morbidity in Behcet’s 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 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 synechia, secondary glaucoma and cataract. Vitreal inflammatory signs in the form of haze or 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. In severe disease, fundoscopic examination may show vitreal deposits, choroidal or retinal exudates, sheathing of blood vessels, retinal edema, hemorrhages, and macular degeneration. In advanced stages, the visual loss becomes permanent and each acute flare adds more to the degree of visual loss. Visual prognosis of BS has dramatically improved in the last two to three decades by better use of conventional immunosuppressors such as cyclosporine and azathioprine, and more recently biologics like anti-TNF alpha agents and interferon alpha. Ophtalmic surgery has also become much more successful.