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M. R. Kesen, D. A. Goldstein, H. H. Tessler; Uveitis in Children With Behcet's Disease. Invest. Ophthalmol. Vis. Sci. 2007;48(13):3897.
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© ARVO (1962-2015); The Authors (2016-present)
To describe the demographics, clinical presentation, visual outcome and prognosis of uveitis in children with Behcet’s disease and to compare childhood-onset with adult-onset Behcet’s uveitis.
Retrospective review of uveitis patient database at the University of Illinois at Chicago (UIC) from 1975 to 2006.
5,275 new patients were seen by the uveitis service at UIC between 1975 and 2006. 101 cases met diagnostic criteria for Behcet’s Disease. 28 charts were available for review. 6 were female (21%), 22 were male (79%). 4 of 28 patients were children, all were male. The mean follow-up period for children was 85 months, among adults it was 43.6 months. The mean age at presentation to the uveitis clinic among children was 16.5 years, it was 35.1 years among adults. Mean age at onset of uveitis for children was 12.8 years and for adults 33 years. Mean age at BD diagnosis was 14 years for children and 34.3 years for adults. Oral ulcers were the most common initial symptom in both children and adults. 75% of both children and adults presented with panuveitis with retinal vasculitis. 1 of 4 children and 7 of 24 adults developed a hypopyon at some point during their course. 3 out of 4 children and 23 out of 24 adults developed retinal vasculitis. 1 out of 2 tested children and 11 out of 22 tested adults were HLA B5 positive. Among children, 6 of 8 eyes had visual acuity of 20/50 or worse at presentation and at 3 years 3 eyes had improved, 4 remained stable, 1 worsened. 28 out of 48 eyes among adults had 20/50 or worse vision, which remained stable throughout. All children were treated with immunosuppressive agents. 2 were treated with alkylators and went into drug free remission; one for 2 years and one for 15 years. 20 out of 24 adults received immunosuppressive therapy. Complications due to treatment were similar for children and adults, and included decreased cell counts and renal dysfunction. The most common ocular complications among children were cataract (75%), vascular occlusions (75%) and maculopathy (75%). Among adults most common were cataract (50%) and posterior synechiae (50%).
Behcet’s disease is an uncommon cause of pediatric uveitis in the United States, even in a tertiary referral center. Behcet’s disease accounted for less than 2% of all uveitis seen in this series, and children made up only 14% of Behcet’s patients. For both children and adults, there was, on average, a one year delay in diagnosis from onset of uveitis to diagnosis of Behcet’s disease. Despite aggressive systemic immunosuppressive therapy, complications were common. Behcet’s disease has a fairly poor prognosis in children, as in adults.
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