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Br J Ophthalmol 87:423-431 doi:10.1136/bjo.87.4.423
  • Original Article
    • Clinical science

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

  1. I Kötter1,
  2. M Zierhut2,
  3. A K Eckstein3,
  4. R Vonthein4,
  5. T Ness5,
  6. I Günaydin1,
  7. B Grimbacher6,
  8. S Blaschke7,
  9. W Meyer-Riemann8,
  10. H H Peter9,
  11. N Stübiger2
  1. 1University Hospital, Departments of Internal Medicine II (Hematology, Oncology, Immunology and Rheumatology) and Ophthalmology, Tübingen, Germany
  2. 2University Hospital, Department of Ophthalmology I, Tübingen, Germany
  3. 3University Hospital, Department of Ophthalmology, Essen, Germany
  4. 4Department of Medical Biometry, University of Tübingen, Germany
  5. 5University Hospital, Department of Ophthalmology, Freiburg, Germany
  6. 6University Hospital, Division of Rheumatology and Clinical Immunology, Freiburg, Germany
  7. 7University Hospital, Department of Nephrology and Rheumatology, Göttingen, Germany
  8. 8University Hospital, Department of Ophthalmology, Göttingen, Germany
  9. 9University Hospital, Division of Rheumatology and Clinical Immunology, Freiburg, Germany
  1. Correspondence to: Ina Kötter, MD, Department of Internal Medicine II, (Hematology/Oncology/Immunology/Rheumatology) University Hospital, Otfried-Müller Strasse 10, D-72076 Tübingen, Germany; ina.koetter{at}med.uni-tuebingen.de
  • Accepted 14 October 2002

Abstract

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.

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