Uveitis in juvenile Behçet's disease: clinical course and visual outcome compared with adult patients

• Published in: Eye (London) Vol. 23; no. 11; pp. 2034 - 2041
• Main Authors: Kramer, M, Amer, R, Mukamel, M, Snir, M, Jaouni, T, Friling, R
• Format: Journal Article
• Language: English
• Published: London Nature Publishing Group UK 01.11.2009; Nature Publishing Group
• Subjects: Adolescent; Adrenal Cortex Hormones - therapeutic use; Adult; Behcet Syndrome - complications; Behcet Syndrome - drug therapy; Behcet Syndrome - physiopathology; Biological and medical sciences; Child; Chronic Disease; clinical-study; Female; Humans; Immunosuppressive Agents - therapeutic use; Laboratory Medicine; Male; Medical sciences; Medicine; Medicine & Public Health; Miscellaneous; Ophthalmology; Pharmaceutical Sciences/Technology; Recurrence; Sarcoidosis. Granulomatous diseases of unproved etiology. Connective tissue diseases. Elastic tissue diseases. Vasculitis; Surgery; Surgical Oncology; Uvea diseases; Uveitis - drug therapy; Uveitis - etiology; Uveitis - physiopathology; Visual Acuity; Young Adult; uveitis; Behçet's disease; juvenile; Human; Skin disease; Prognosis; Stomatology; Cardiovascular disease; Uvea disease; Behçet syndrome; Genital diseases; Vascular disease; Eye disease; Vasculitis; Systemic disease; Uveitis; Adult; Evolution; Ophthalmology; Comparative study
• ISSN: 0950-222X; 1476-5454
• DOI: 10.1038/eye.2008.397

Purpose To characterize the disease course and visual outcome of uveitis in juvenile Behçet's disease (BD) compared with adults. Methods The study population included 13 children (mean age 14±2.4 years; 22 eyes) and 16 adults (mean age 30±8.8 years; 27 eyes) with uveitis in BD diagnosed between 1997 and 2007. Results The male/female ratio was 1.6:1 in the paediatric group and 3:1 in the adult group. Five children (38%) and four adults (25%) had complete BD. Mean duration of follow-up for both groups was 4.7 years. The children had more acute exacerbations (4.1±2.7 vs 2.3±1.5, P =0.054). Treatment in both groups included systemic steroids and immunosuppressive agents. In children, mean initial visual acuity in the affected eyes ( n =22) was 0.6±0.7 logMAR (range, 0–2.2). It decreased during exacerbations in 15 eyes (68%; mean, 1.6±0.8 logMAR), severely reduced (worse than 1 logMAR) in 11 eyes (50%; mean, 2.0±0.45 logMAR), and improved significantly in 12 of 13 promptly treated eyes (92%; 6/12 or better in 11; mean 0.2±0.4 logMAR, P <0.001). The visual outcome pattern was similar in the adults. Conclusions Uveitis in juvenile BD is characterized by frequent exacerbations of explosive nature with profoundly reduced visual acuity. Similar disease pattern was observed in children and adults, as well as in patients with complete or incomplete disease. Early diagnosis, even before all systemic criteria are fulfilled, is important because early aggressive therapy can achieve long-term useful visual acuity.