Cytotoxic agents have long been used in Behçet's syndrome, especially for eye involvement, but their effectiveness has been uncertain. We conducted a two-year randomized, placebo-controlled, double-blind trial of azathioprine (2.5 mg per kilogram of body weight per day) in Turkish men with Behçet's syndrome without eye disease (group 1; n = 25) or with eye disease (group 2; n = 48). Corticosteroid treatment remained available to all the patients. All six patients withdrawn from the study because of severe eye disease were receiving placebo (P less than 0.001). Azathioprine was superior to placebo in the prevention of new eye disease in group 1 (1 vs. 8 patients; P less than 0.01) and in group 2 among the 14 patients who at entry had disease in only one eye (P less than 0.001). There were fewer episodes of hypopyon uveitis (1 vs. 15; P less than 0.001) among the group 2 patients who took azathioprine. The patients taking azathioprine also had less frequent oral ulcers, genital ulcers, and arthritis. There were no serious side effects attributable to azathioprine. We conclude that azathioprine is effective in controlling the progression of Behçet's syndrome, especially its most serious manifestation, eye disease.

SUMMARY Eye disease, arthritis, folliculitis, and thrombophlebitis were more common among males, and erythema nodosum among females, in 297 patients with Behqet's syndrome (BS) at their first visit despite an identical disease duration. Younger males and females (age of onset 24 years or less) had a higher prevalence of eye disease and total clinical activity than did the older patients (age of onset 25 years or more). Among the 51 patients followed up for 52 months (SD 7 months) the total clinical activity became significantly less (p

SUMMARY A prospective study of arthritis was performed in 47 patients with Behcet's disease followed up over a 47-month period (mean

A single masked trial of cyclosporin A 5 mg/ kg/day versus monthly 1 g intravenous boluses of cyclophosphamide was conducted among 23 patients with Behset's syndrome and active, potentially reversible uveitis. The trial was unmasked after a mean of 12 (SD 2) months for the cyclosporin A group (n= 12) and a mean of 10 (SD 3) months for the cyclophosphamide group (n=I1). During the initial 6 months the visual acuity significantly improved (p<0 001) in the cyclosporin A group whereas this was not observed in the cyclophosphamide group. The subsequent follow-up of patients up to 24 months suggested that the initial improvement in visual acuity with cyclosporin A was not sustained. More extensive and especially longterm studies of cyclosporin A in the uveitis of Beh4et's syndrome are warranted.

The study's survival rate was similar to developed countries. The success in higher survival rates is based on the authors multidisciplinary team approach done by the same group and the support of the authors' clinic and government in sponsoring the medical insurance of all patients.