Distinct clinical characteristics of pediatric Behçet’s syndrome: A study from a referral center in China

Abstract

Objectives To describe the clinical features and patterns of phenotype aggregation in pediatric Behçet’s syndrome (BS) in a tertiary center in China. Methods This was a cross-sectional study of consecutive BS patients in Huadong Hospital, Fudan University between September 2012 and January 2020. Pediatric BS was defined as diagnosed before 16 years old. We compared clinical variables between pediatric and adult patients. We calculate relative risks (RRs) of clinical variables according to sex. Moreover, a hierarchical cluster analysis was undertaken according to 29 clinical variables to determine homogeneous subgroups. Results From 1596 consecutive BS cases, we identified 69 pediatric BS. Compared with adult-diagnosed BS, pediatric-diagnosed patients had a higher frequency of folliculitis [RR 1.57 (95% CI 1.12, 2.20)], a lower frequency of arthralgia [RR 0.15 (95% CI 0.02, 1.07)] and panuveitis [RR 0.43 (95% CI 0.18, 1.03)], no cardiac lesion. There was an association between male and arterial thrombosis or aneurysms (p = .006). A cluster analysis stratified three sub-clusters (C1–C3): C1 (n = 30) showed a disease type merely affecting skin and mucosa. C2 (n = 20) represented the gastrointestinal type; most patients presented with intestinal involvement, and two cases with esophageal ulcers. In C3 (n = 19), showing a mixture of uveitis, vascular and central nervous system (CNS) involvement, six patients presented with uveitis and nine had vascular lesions, and three cases had CNS lesions. Conclusion We conducted a comprehensive statistical analysis in a cohort of pediatric patients with BS in China. Less ocular involvement and no cardiac lesions were observed in childhood-diagnosed patients. For the first time, three distinct phenotype subgroups in pediatric-diagnosed patients were identified by cluster analysis. Key messages This study demonstrated the phenotype discrepancy between childhood and adult-diagnosed BS. Three distinct clusters were identified, with skin-mucosa, gastrointestinal, panuveitis, vascular and CNS subgroups.