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Clusters in paediatric Behçet's disease: a multicentre international study
Ummusen Kaya Akca1, Farhad Shahram2, Erdem Karabulut3
1Department of Pediatric Rheumatology, Hacettepe University Faculty of Medicine, Ankara, Turkey.
RMD Open
|July 5, 2025
Summary
This study analyzed pediatric Behçet's disease (BD) in Turkey, Iran, and Europe, identifying distinct clinical clusters and regional variations. Findings show BD presents in specific patterns in children, varying by geography.
Area of Science:
- Rheumatology
- Pediatric Rheumatology
- Clinical Immunology
Background:
- Behçet's disease (BD) exhibits significant clinical variability influenced by gender and geography.
- Understanding these variations is crucial for accurate diagnosis and management in pediatric populations.
Purpose of the Study:
- To describe clinical characteristics of pediatric BD patients.
- To identify distinct clinical clusters within a large cohort of pediatric BD.
- To compare clinical manifestations across Turkey, Iran, and Europe.
Main Methods:
- Retrospective evaluation of pediatric-onset BD patients (<18 years) from Turkey, Iran, France, and Italy.
- Inclusion required a minimum follow-up period of 6 months.
- Analysis of clinical features, geographical distribution, and cluster identification.
Main Results:
- 600 pediatric BD patients were included; mucocutaneous involvement was most common (97.5%).
- Significant regional variations observed: ocular in Iran, GI in Europe, musculoskeletal/vascular in Turkey.
- Seven distinct clusters were identified, including vascular, mucocutaneous-only, ocular, GI, mixed, neurologic/ocular, and mucocutaneous-musculoskeletal patterns.
Conclusions:
- Pediatric BD tends to present in identifiable clinical clusters.
- Classification of these clusters is challenging due to disease complexity and multifactorial etiology.
- Geographical region significantly impacts the prevalence of certain clinical features in pediatric BD.
