Human recombinant interferon alfa-2a for the treatment of Behçet's disease with sight threatening posterior or panuveitis

• Published in: British journal of ophthalmology Vol. 87; no. 4; pp. 423 - 431
• Main Authors: Kötter, I, Zierhut, M, Eckstein, A K, Vonthein, R, Ness, T, Günaydin, I, Grimbacher, B, Blaschke, S, Meyer-Riemann, W, Peter, H H, Stübiger, N
• Format: Journal Article
• Language: English
• Published: BMA House, Tavistock Square, London, WC1H 9JR BMJ Publishing Group Ltd 01.04.2003; BMJ; BMJ Publishing Group LTD; Copyright 2003 British Journal of Ophthalmology
• Subjects: Adult; Angiogenesis Inhibitors - adverse effects; Angiogenesis Inhibitors - therapeutic use; Behcet Syndrome - complications; Behcet Syndrome - drug therapy; Behçet's disease; Biological and medical sciences; Clinical Science; Drug dosages; Eye and associated structures. Visual pathways and centers. Vision; Eye diseases; Female; Fibromyalgia; Fundamental and applied biological sciences. Psychology; Hepatitis; human recombinant interferon alfa-2α; Humans; Immunoglobulins; Interferon; Interferon alpha-2; Interferon-alpha - adverse effects; Interferon-alpha - therapeutic use; Male; Medical sciences; Middle Aged; Ophthalmology; Panuveitis - drug therapy; Panuveitis - etiology; Patient Compliance; Prospective Studies; Psoriasis; Recombinant Proteins; Recurrence; Remission Induction; Retinal Vasculitis - drug therapy; Retinal Vasculitis - etiology; retinitis; Sarcoidosis. Granulomatous diseases of unproved etiology. Connective tissue diseases. Elastic tissue diseases. Vasculitis; Software Design; Steroids; Studies; Thyroid gland; Treatment Outcome; Uvea diseases; uveitis; Uveitis, Anterior - drug therapy; Uveitis, Anterior - etiology; Uveitis, Posterior - drug therapy; Uveitis, Posterior - etiology; Vertebrates: nervous system and sense organs; Visual Acuity; Human; Eye disease; Vasculitis; Treatment; Alpha interferon; Immunotherapy; Posterior; Uveitis; Uvea disease; Behçet syndrome
• ISSN: 0007-1161; 1468-2079
• DOI: 10.1136/bjo.87.4.423

Background: Behçet's disease is a multisystem vasculitis of unknown origin. Standard treatment mainly comprises systemic immunosuppressive agents. Ocular involvement, mostly posterior uveitis with retinal vasculitis, leads to blindness in 20–50% of the involved eyes within 5 years. The efficacy of interferon alfa-2a was studied in patients with sight threatening posterior uveitis or retinal vasculitis. Methods: 50 patients were included in this open, non-randomised, uncontrolled prospective study. Recombinant human interferon alfa-2a (rhIFNα-2a) was applied at a dose of 6 million units subcutaneously daily. Dose reduction was performed according to a decision tree until discontinuation. Disease activity was evaluated every 2 weeks by the Behçet's disease activity scoring system and the uveitis scoring system. Results: Response rate of the ocular manifestations was 92% (three non-responder, one incomplete response). Mean visual acuity rose significantly from 0.56 to 0.84 at week 24 (p<0.0001). Posterior uveitis score of the affected eyes fell by 46% every week (p<0.001). Remission of retinal inflammation was achieved by week 24. Mean Behçet's disease activity score fell from 5.8 to 3.3 at week 24 and further to 2.8 at week 52. After a mean observation period of 36.4 months (range 12–72), 20 patients (40%) are off treatment and disease free for 7–58 months (mean 29.5). In the other patients maintenance IFN dosage is three million units three times weekly. Conclusions: rhIFNα-2a is effective in ocular Behçet's disease, leading to significant improvement of vision and complete remission of ocular vasculitis in the majority of the patients.