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Abstract

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.

Clinical Outcomes of Cyclosporine Treatment for Noninfectious Uveitis

“…Previous studies reported similar or inferior effectiveness of cyclosporine for ocular inflammatory disease compared to our study [2,6-9,12-14,17,20,21,26]. This disparity comes from the differences in outcome measures and the definition of success in uveitis control, thus making direct comparison between studies impossible.…”

“…Only a small group of patients in our study discontinued cyclosporine due to toxicity, which is superior to other studies that reported adverse effects with low-dose cyclosporine for ocular inflammation [17,20,21,27]. We used a protocol of relatively rapid cyclosporine dose reduction according to inflammation status, which might have also contributed the lower rates of toxicity.…”

Outcome Measures Used in Clinical Trials for Behçet Syndrome: A Systematic Review

“…Visual acuity 9,15,16,120,144-196,252-254 and inflammatory attacks9,15,120,144,147,155-158,161,162,164,165,166,169,170,171,173,176,181,183,184,186,188-196 are the most widely used outcome measures. However, the definition of an inflammatory flare varied widely among studies, and visual acuity was reported in several different ways, including change on Snellen chart or Japanese standard Landolt visual acuity chart, calculating the LogMar (logarithm of the minimum angle of resolution), or the percentage of patients who had a certain level of improvement.…”

Management of pediatric uveitis

“…Cyclosporine has been observed to be particularly effective in patients with Behçet's disease-associated uveitis [25]; in children with juvenile idiopathic arthritis-associated uveitis, cyclosporine is more effective when used in combination with methotrexate [26]. Initial dosing is recommended at 3-5 mg/kg/day divided into two doses, followed by a maintenance dose of 2-3 mg/kg/day [6,27].…”