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Purpose: To investigate long-term responsiveness of patients with severe uveitis associated with Behçet disease to interferon alpha2a (IFN-alpha2a).Design: Retrospective study.Methods: Thirty-two patients with sight-threatening uveitis associated with Behçet disease who relapsed despite corticosteroids and immunosuppressive agents were included. IFN-alpha2a was administered subcutaneously (three million units thrice a week). Ophthalmologic examination and fluorescein angiography associated with laboratory tests were performed at regular intervals. Main outcome measures were visual acuity and recurrence of uveitis attacks before, during, and after initiation of IFN-alpha2a therapy.Results: Control of inflammation was achieved in 28 of 32 patients (88%). Mean observation period of the 28 responder patients was 70.6 months (range, 30.3 to 129.2 months). Median visual acuity improved from 0.52 to 0.33 (logarithm of the minimum angle of resolution units; P = .005) two years after initiation of IFN-alpha2a therapy. The relapse rate decreased significantly during IFN-alpha2a treatment from 1.68 +/- 1.22 relapses/patient/year to 0.11 +/- 0.20 relapses/patient/year (P < .0001). IFN-alpha2a was discontinued in 19 of 28 patients (68%) after 32 months of treatment (range, 16 to 50 months). Mean follow-up after IFN-alpha2a discontinuation was 43 months (range, 11 to 84 months). After IFN-alpha2a discontinuation, the relapse rate increased from 0.08 +/- 0.21 relapse/person/year to 0.74 +/- 1.40 relapse/person/year (P = 0.04).Conclusions: IFN-alpha2a is efficient and safe for the long-term management of severe uveitis associated with Behçet disease. Meanwhile it seems to be a suspensive therapeutic strategy, even though long-term remission is possible in some patients. [ABSTRACT FROM AUTHOR]