Isolated Ocular Behçet´s Disease: Therapy with Interferon Alpha
Stübiger N.1, Deuter C. M. E.1, Guenaydin I.2, Zierhut M.1, Koetter I.2
1Department of Ophthalmology I, 2Department of Internal Medicine II, University of Tuebingen
Purpose: Behçet´s disease (BD), a systemic vasculitis, is diagnosed according the criteria of the International Study Group of Behçet´s Disease. Patients, suffering from a retinal occlusive vasculitis (arteries and veins involved), which is typical of BD but show no other signs of BD are most commonly diagnosed as a retinal vasculitis of unknown origin, irrespective of the fact, that in up to 20% the ocular involvement is the primary manifestation of this disease.
Method: Seven men and one woman received human recombinant Interferon a2a (IFNa2a), that is very effective in ocular BD. This patients suffered from an occlusive retinal vasculitis, which was refractory to other immunosuppressive therapies. Mean age of our patients was 34 years, five patients were of German origin and 3 patients Mediterraneans. Five patients were HLA B51 positive. Former therapies (Cyclosporine A, Azathioprine, Mycophenolatmofetile, Aciclo- and Ganciclovir, and Corticosteroids) had been ineffective. Initially IFNa2a was applied at a dose of 6 MioIU/day and dose reduction was performed according to a decision tree. Activity of ocular changes was evaluated by the Uveitis Scoring System of BenEzra.
Results: During IFN-treatment seven of the eight patients (88%) achieved
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