Behçet's syndrome: ocular features in an Australian population

Inflammatory eye disease (IED) is often the most severe manifestation of Behçet's syndrome (BS). This disease is a common cause of uveitis in Mediterranean countries, the Middle East and Japan. In order to ascertain the prevalence of this disease in Australia, we reviewed the aetiology of patients attending our uveitis clinic over a five-year period. Twelve of 426 patients with inflammatory eye disease had definite Behçet's syndrome. Four patients had anterior uveitis, four had posterior uveitis, four had retinal vasculitis and one also had optic neuritis. Although inflammatory eye disease was the initial clinical feature of Behçet's syndrome in only three of our patients, it was the feature that led to a definite diagnosis in all but one patient. The inflammatory eye disease of Behçet's syndrome was characteristically recurrent and severe with significantly decreased vision in 10 eyes, cataracts in six eyes, macula oedema in four eyes and glaucoma in two eyes. We conclude that Behçet's syndrome is a rare cause of inflammatory eye disease in Australia and is unlikely to be recognised as a distinct clinical entity prior to the onset of ocular involvement. The visual prognosis of ocular inflammation in Behçet's syndrome remains guarded despite the use of a variety of immunosuppressive agents.