Uveitis Associated With Pediatric Behçet Disease in the American Midwest

Purpose: To describe demographics, clinical course, treatment, and visual prognosis of uveitis in pediatric patients with Behçet disease, and to compare childhood-onset with adult-onset Behçet uveitis. Design: Retrospective, observational case series. Methods: setting: Uveitis service, University of Illinois at Chicago and private office of one of the authors (H.H.T.). study population: Patients diagnosed with Behçet uveitis using International Study Group criteria between January 1, 1973 to December 31, 2007. main outcome measures: Demographics, symptoms, clinical course, management, laboratory tests, complications, and visual prognoses were recorded. Results: Thirty-three patients had available records with at least one month follow-up; four were children and 29 were adults. All children and 19 adults were male. The most common initial symptom was oral ulcers and the most common uveitis type was panuveitis with retinal vasculitis in both groups. There was an average delay of one year among children and 1.8 years among adults between onset of uveitis and disease diagnosis. Immunosuppressive therapy was employed in all children and 23 of 29 adults. Treatment response was variable in both groups. Maculopathy was the most common cause of permanent visual impairment, both in children and in adults. Six of eight pediatric eyes (75%) and 13 of 25 adult eyes (52%) retained a visual acuity better than 20/200 at three years. Conclusions: Behçet disease is an uncommon cause of uveitis in the United States, with a male predominance. Although the clinical picture of Behçet uveitis was similar among both groups, the visual prognosis appeared worse in adults. Awareness of this disease should be increased in nonendemic areas to prevent blindness resulting from delays in diagnosis.