Background: Behçet's disease (BD) is a systemic vasculitis of unknown origin. Up to now ocular involvement has a poor visual prognosis irrespective of immunosuppressive treatment. Now recent open studies have shown that interferon alfa-2a (IFN alfa-2a) is very effective in the treatment of ocular BD. The purpose of this study was to evaluate the long-term development of visual acuity (VA) in patients with severe ocular BD who were treated with IFN alfa-2a.
Methods: We included 15 eyes of 9 patients with an active panuveitis and/or retinal vasculitis due to BD refractory to immunosuppressive treatment. VA before initiation of IFN-therapy was compared to VA at the end of the follow-up time.
Results: Mean follow-up time was 68.8+/-10.6 months. Mean duration of IFN-treatment was 40.6+/-17.0 months. With the exception of one patient IFN alfa-2a could be discontinued in complete remission of ocular symptoms. During the follow-up time 10 eyes showed an increase of VA of two lines or more. In 5 eyes VA remained stable. There was no decrease of VA in any eye. If a macular edema was present, a quick response to IFN alfa-2a was seen. No eye developed a pale optic disk during follow-up.
Conclusion: Compared to conventional immunosuppressants, interferon alfa-2a seems to be much more effective to prevent a loss or decrease of VA over a long period of time in patients with severe ocular BD.