title: Human recombinant interferon alfa-2a for the treatment of Behçet's disease with sight threatening posterior or panuveitis.
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 (rhIFNalpha-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
  rhIFNalpha-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.
authors.affiliation: University Hospital, Departments of Internal Medicine II (Hematology, Oncology, Immunology and Rheumatology) and Ophthalmology, Tübingen, Germany. ina.koetter@med.uni-tuebingen.de
authors.forename: I
authors.initials: I
authors.lastname: Kötter
authors.forename: M
authors.initials: M
authors.lastname: Zierhut
authors.forename: A K
authors.initials: AK
authors.lastname: Eckstein
authors.forename: R
authors.initials: R
authors.lastname: Vonthein
authors.forename: T
authors.initials: T
authors.lastname: Ness
authors.forename: I
authors.initials: I
authors.lastname: Günaydin
authors.forename: B
authors.initials: B
authors.lastname: Grimbacher
authors.forename: S
authors.initials: S
authors.lastname: Blaschke
authors.forename: W
authors.initials: W
authors.lastname: Meyer-Riemann
authors.forename: H H
authors.initials: HH
authors.lastname: Peter
authors.forename: N
authors.initials: N
authors.lastname: Stübiger
mesh_terms: D000328:Adult; D020533:Angiogenesis Inhibitors / Q000009:adverse effects / Q000627:therapeutic use*; D001528:Behcet Syndrome / Q000150:complications / Q000188:drug therapy*; D005260:Female; D006801:Humans; D000077190:Interferon alpha-2; D016898:Interferon-alpha / Q000009:adverse effects / Q000627:therapeutic use*; D008297:Male; D008875:Middle Aged; D015864:Panuveitis / Q000188:drug therapy* / Q000209:etiology; D010349:Patient Compliance; D011446:Prospective Studies; D011994:Recombinant Proteins; D012008:Recurrence; D012074:Remission Induction; D031300:Retinal Vasculitis / Q000188:drug therapy / Q000209:etiology; D012985:Software Design; D016896:Treatment Outcome; D014606:Uveitis, Anterior / Q000188:drug therapy / Q000209:etiology; D015866:Uveitis, Posterior / Q000188:drug therapy / Q000209:etiology; D014792:Visual Acuity
chemical_list: D020533:Angiogenesis Inhibitors; D000077190:Interferon alpha-2; D016898:Interferon-alpha; D011994:Recombinant Proteins