Spectrum of Behçet’s disease in the Indian population

Abstract

The purpose of this investigation is to study the clinical profile of Behçet’s disease in the Indian population as seen in a tertiary referral uveitis clinic. A retrospective non-comparative case series was used. The records of all of the patients seen in our uveitis clinic between January 1990 and December 2006 were screened and patients with a diagnosis of Behçet’s disease were enrolled. The diagnosis was based on the Japanese diagnostic criteria and patients were classified into complete, incomplete, suspected, and possible Behçet’s disease. Information on the patient’s demographic characteristics, ocular and systemic clinical features, complications, investigation results, management protocols, and visual outcome was collected. The records of 4,214 uveitis clinic patients were screened and 53 patients (1.25%) of Behçet’s disease were identified. These included 39 males and 14 females (2.8:1), with a mean age of 27.6 years (range 10–51 years). Fifty (94.3%) of these patients were of either complete or incomplete Behçet’s disease. Ocular involvement was the most common manifestation (49 patients, 92.4%), followed by oral ulcers (88.7%), genital ulcers (61.2%), and skin involvement (28.3%). Panuveitis (57.1%) was the most common ocular presentation, followed by posterior (26.5%) and anterior uveitis (16.3%). More than 95% of the patients required oral steroids, while additional immunosuppressants were used in nearly 53% of the patients. The common complications observed included complicated cataract (57% of eyes), followed by cystoid macular edema (55.1%), occlusive vasculitis (44.8%), and optic atrophy (26.5%). Nearly 36% of the eyes maintained 6/12 or better vision, while 38% of the eyes were less than 6/60 after a mean follow-up period of 4.9 years (range 2–16 years). Behçet’s disease, although rare among Indians, commonly manifests with ocular involvement as non-granulomatous panuveitis with a high incidence of complications and unfavorable visual outcome in nearly one-third of the cases.