ArticleWorld journal of pediatrics : WJP2024
Clinical heterogeneity and five phenotypes identified in pediatric Behçet's syndrome: a cohort study from Shanghai Behçet's syndrome database.
Article in World journal of pediatrics : WJP, 2024. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 12 papers.
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Who cites it
12 citing papers in PubMed.
- Predictive role of inflammatory indexes in systemic manifestations of pediatric Behçet's disease.Annals of medicine · 2026Article
- Mucocutaneous-Predominant Pediatric Behçet's Disease With Recurrent Oral and Genital Ulceration: A Case Report.Clinical case reports · 2026Article
- Clinical characteristics, treatment, and prognosis of pediatric Vasculo-Behçet's syndrome: a retrospective analysis of 12 cases.Frontiers in pediatrics · 2026Article
- Case Report: From idiopathic recurrent pericarditis to systemic Behçet's Disease: unmasking a unified IL-1-driven autoinflammatory phenotype.Frontiers in immunology · 2026Article
- [Clinical characteristics of Behçet syndrome in 45 children].Zhongguo dang dai er ke za zhi = Chinese journal of contemporary pediatrics · 2025Article
- Cluster and correspondence analyses for oral ulcer activity related factors in Behçet's syndrome.Medicina oral, patologia oral y cirugia bucal · 2025Article
- Unraveling the complexities of pediatric Behçet's disease clusters.World journal of pediatrics : WJP · 2025Article
- Article
- Clinical features and novel pathogenic variants of patients with Behçet's disease like trisomy 8.Orphanet journal of rare diseases · 2025Article
- [Update on Behçet syndrome].Zeitschrift fur Rheumatologie · 2024Review
- Challenges and opportunities in transitional care process in Behçet's syndrome.Frontiers in medicine · 2024Article
- Uveitis in paediatric Behçet disease: a large multicentric Italian cohort.Therapeutic advances in musculoskeletal disease · 2024Article
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10 authors.
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Abstract
objectivesBehçet's syndrome (BS) is a rare disease of unknown etiology, with limited reports especially in pediatric BS. The clinical characteristics and phenotypes of pediatric BS as a highly heterogeneous variable vessel vasculitis were investigated in this study.
methodsA cross-sectional study was conducted to compare clinical variables and descriptive characteristics of BS by age of onset and gender. Cluster analysis was then performed to identify the phenotypes of pediatric BS.
resultsA total of 2082 BS patients were included in this study, 1834 adults and 248 children. Compared with adult-onset BS, pediatric BS had a higher incidence of folliculitis [relative risks (RR) and 95% confidence interval (CI) 1.3 (1.0-1.5)], uveitis of the left eye [RR and 95% CI 2.3 (1.0-5.0)], intestinal ulcer complications [RR and 95% CI 2.1 (1.1-4.2)], pericarditis [RR and 95% CI 2.5 (1.0-6.2)], and psychiatric disorders [RR and 95% CI 2.8(1.0-7.9)], while the incidence of thrombocytopenia was lower [RR 0.2 (0.1-1.0)]. Among pediatric BS, females had more genital ulcers, while males were more likely to have skin lesions, panuveitis, vascular involvement, venous lesions, cardiac involvement, and aortic aneurysms. Cluster analysis classified pediatric BS into five clusters (C1-C5): C1 (n = 61, 24.6%) showed gastrointestinal (GI) involvement; C2 (n = 44, 17.7%) was the central nervous system (CNS) type where 23 cases overlapped joint involvement; in C3 (n = 35, 14.1%), all patients presented with arthritis or arthralgia; all patients in C4 (n = 29, 11.7%) manifested ocular involvement, with a few patients overlapping with GI involvement or joint damage; C5 (n = 79, 31.9%) was the mucocutaneous type, presenting both oral ulcers, genital ulcers, and skin lesions.
conclusionsThe clinical features of pediatric and adult BS differ significantly. Male and female pediatric BS also have a distinct demography. Five phenotypes including GI, CNS, joint, ocular, and mucocutaneous types were identified for pediatric BS.
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