title: Differential efficacy of human recombinant interferon-alpha2a on ocular and extraocular manifestations of Behçet disease: results of an open 4-center trial.
abstract: OBJECTIVE
  Behçet disease (BD) is a multisystem vasculitis of unknown origin. Standard treatment mainly comprises systemic immunosuppressive agents. In a study primarily designed for refractory ocular disease, we additionally evaluated the efficacy of recombinant human interferon-alpha2a (rhIFN-alpha2a) on the extraocular manifestations of BD.
  
  
  METHODS
  Fifty patients were included in the study. RhIFN-alpha2a was applied at a dose of 6 x 10(6) units subcutaneously daily. Dose reduction was performed according to a decision tree until discontinuation. Disease activity was evaluated by the Behçet's Disease Activity Scoring System and the Uveitis Scoring System.
  
  
  RESULTS
  Response rate of the ocular manifestations was 92%. Visual acuity rose significantly from 0.56 to 0.84 at week 24 (P <.0001). Mean Behçet's Disease Activity Score fell in a dose-dependent fashion by 1.2 points in the first week (P <.0001) and 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, 17 patients have been off treatment and disease-free for 29.5 months (mean). In the other patients, maintenance IFN dosage is 3 million units 3 times weekly. Whereas extraocular manifestations such as genital ulcerations, arthritis, and skin lesions remitted with IFN, only 36% of oral aphthous ulcers responded.
  
  
  CONCLUSIONS
  RhIFN-alpha2a is effective in ocular BD, resulting in significant improvement of vision and complete remission of ocular vasculitis in the majority of patients. It is also beneficial for the extraocular manifestations of the disease, although less so for oral aphthous ulcers.
authors.affiliation: University Hospital, Department of Internal Medicine II, Tübingen, Germany. ina.koetter@med.uni-tuenbingen.de
authors.forename: Ina
authors.initials: I
authors.lastname: Kötter
authors.forename: Reinhard
authors.initials: R
authors.lastname: Vonthein
authors.forename: Manfred
authors.initials: M
authors.lastname: Zierhut
authors.forename: Anja Katrin
authors.initials: AK
authors.lastname: Eckstein
authors.forename: Thomas
authors.initials: T
authors.lastname: Ness
authors.forename: Ilhan
authors.initials: I
authors.lastname: Günaydin
authors.forename: Bodo
authors.initials: B
authors.lastname: Grimbacher
authors.forename: Sabine
authors.initials: S
authors.lastname: Blaschke
authors.forename: Hans Hartmut
authors.initials: HH
authors.lastname: Peter
authors.forename: Nicole
authors.initials: N
authors.lastname: Stübiger
mesh_terms: D000328:Adult; D001528:Behcet Syndrome / Q000188:drug therapy*; D005260:Female; D006801:Humans; D007166:Immunosuppressive Agents / Q000008:administration & dosage / Q000627:therapeutic use*; D000077190:Interferon alpha-2; D016898:Interferon-alpha / Q000008:administration & dosage / Q000627:therapeutic use*; D008297:Male; D010349:Patient Compliance; D011994:Recombinant Proteins; D014792:Visual Acuity / Q000187:drug effects
chemical_list: D007166:Immunosuppressive Agents; D000077190:Interferon alpha-2; D016898:Interferon-alpha; D011994:Recombinant Proteins