- recently reported the effectiveness and safety of cyclosporin A (CsA) treatment in patients with Behqet's disease. We have also used CsA in Behqet's disease patients and have noted similar degrees of effectiveness, but we have rather different findings with respect to renal tolerance.
Of 150 patients with Behqet's disease, 5 were treated with CsA because of severe, refractory bilateral uveitis. All patients were men; 3 were from northern Africa, and 2 were from France. Their ages ranged from 24-36 (mean 31). Behqet's disease was complete in 3 of them, and incomplete in 2 of them. The main symptoms were: oral ulcers (5 patients), genital ulcers (4 patients), skin involvement (4 patients), arthritis (3 patients), phlebitis (3 patients), and central nervous system involvement (1 patient). These patients had failed to respond to, or had had relapses despite administration of, high doses of steroids (5 patients), plasma exchange (3 patients), and/or immunosuppressive agents (chlorambucil in 2 patients, cyclophosphamide in 2 patients), which were given simultaneously or successively.
Patients entering the study received 10 mg/kg of CsA orally, in 2 or 3 divided doses, daily. Cytotoxic agents had been stopped at least 2 weeks before institution of CsA therapy. Steroids were initially maintained and then were slowly tapered, depending upon ocular improvement. CsA was monitored to achieve serum trough levels of <200 ng/ml (radioimmunoassay ; Sandoz, Paris, France). Duration of CsA treatment ranged from 5-17 months (mean 10 months). CsA was effective in treatment of the uveitis, resulting in stabilization of the disease in 1 patient, and improvement of both visual acuity and retinal vasculitis score, despite a 75% reduction in steroid dosage (3 patients) or complete withdrawal of steroid therapy (1 patient).
All patients developed side effects: hirsutism (4 patients), gynecomastia (2 patients), hypertension (3 patients, l