Posterior segment involvement is the most serious ocular complication of BD, leading to blindness with recurrent attacks. Following patients closely, performing fluorescein angiography in all patients diagnosed as BD even they have no clinical ocular involvement, and early treatment are very important in the prognosis of the disease.
Ocular complications of Behçet's disease can be severe and lead to blindness in 90% of untreated patients. We aimed to evaluate the long-term use of low dose cyclosporin-A (CsA) which is a potent immunomodulatory agent in the treatment of ocular Behçet's disease. Fifty-two patients (104 eyes) with ocular Behçet's disease using CsA for at least 1 year were included in this study. All the patients underwent complete ophthalmological and systemic examination. Five mg/kg/per day CsA was started to the patients with severe posterior uveitis and/or frequent anterior inflammatory attacks unresponsive to the conventional therapeutic agents. According to ocular response and adverse effects, the dose was tapered gradually over 2 months to a maintenance dose of 3 mg/kg/per day. Prednisone (0.2-0.8 mg/kg/per day) was added when necessary. Forty-six of the patients (88.5%) were males and six (11.5%) were females. The mean age was 33.65 +/- 7.75 (range, 19-53) years. The mean ocular involvement period was 64.1 (range, 12-180) months. Posterior uveitis was present in 49 (94.21%) and severe, recurrent anterior uveitis in three (5.8%) patients. The mean CsA administration period was 38 +/- 18.1 months. Visual acuity improved in 31(29.8%), deteriorated in 32 (30.8%) and unchanged in 41(39.4%) of the 104 eyes. No ocular attacks occurred in 50% of the eyes during therapy. Nine (17.3%) of the patients had to stop the therapy because of the adverse effects of the CsA and the others tolerated well for a long-term period. CsA is not the ideal therapeutic agent in ocular Behçet's disease because it can not completely eliminate the disease, but it is currently one of the most effective and efficient drug to control the uveitis and its complications until better treatment modalities are developed.
Although the arteries are rarely affected in retinal vasculitis due to BD, it has to be considered in the differential diagnosis of retinal arterial occlusions especially in countries where the disease is prevalent. To our knowledge, this is the first reported case of ocular BD complicated with central retinal artery occlusion.
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