Introduction: Behçet's disease (BD) is a multisystem vasculitis of unknown origin. Visual outcome in BD is bad irrespective of therapy. We investigated in a prospective, open clinical trial wether interferon alpha2a (IFN alpha2a) is effective as single agent in severe ocular BD.
Patients and methods: 33 patients (11 female, 22 male) with severe ocular BD (posterior uveitis/panuveitis), fulfilling the Criteria of the "International Study Group of Behçet's Disease", were treated with IFN alpha2a. Other immunosuppressive drugs were stopped at the beginning of interferon-therapy. Efficacy was evaluated by the uveitis scoring system (Ben Ezra et al.), visual acuity and BD activity score (Rigby et al.).
Results: In 32 (97 %) patients complete remission of the inflammatory changes occured. Mean posterior uveitis score fell from 3.7 to 0.4, visual acuity rose from 0.5 to 0.9, and mean BD activity score fell from 5.1 to 3.0 during a mean observation period of 28.6 months. There was only one non-responder. Side effects were hair loss (69 %), depression (36 %), itching (33 %), fibromyalgia (10 %), and the development of autoantibodies (21 %). Fever, arthralgia and headache occured in all patients, but only during the first weeks of therapy.
Conclusion: IFN alpha2a seems to be very effective in ocular BD. Controlled randomized studies are warranted in order to prove the efficacy of IFN alpha2a in ocular BD and to compare it with other, established treatments, such as azathioprine or cyclosporin A.