Evaluation of Childhood Behçet’s Disease: A Single-Center Experience

Vollständiger Abstract

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Objective: Behçet’s disease (BD) is a rare chronic inflammatory vasculitis in childhood that can involve vessels of all sizes. Clinical findings vary according to age, sex, and geographic regions. This study aimed to describe the demographic, clinical, and immunogenetic features of pediatric patients with BD and to compare clinical characteristics between human leukocyte antigen (HLA)-B51–positive and –negative patients. Methods: Medical records of patients diagnosed with BD between January 2020 and June 2025 were retrospectively reviewed. Patients younger than 18 years at diagnosis who met international criteria were included. Results: A total of 38 patients (20 females) were included. The median age at onset was 12.5 years and at diagnosis was 14 years. A family history of BD was present in 19 patients. HLA-B51 positivity was detected in 20 patients, and the pathergy test was positive in 13 patients. Subgroup analysis comparing HLA-B51–positive and –negative patients revealed no significant differences in demographic and clinical variables (P > .05). Recurrent oral ulcers were observed in all patients, skin lesions in 28 patients, genital ulcers in 25 patients, uveitis in 11 patients, and joint involvement in 10 patients. All patients received colchicine. Corticosteroids and azathioprine were used in 12 and 11 patients, respectively, while other immunosuppressive or biologic agents were reserved for severe organ involvement. Conclusion: In childhood BD, mucocutaneous findings are the most common manifestations. The high frequency of family history and HLA-B51 positivity highlights genetic predisposition. Colchicine is effective as first-line therapy, with additional immunosuppressive agents used according to organ involvement.