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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.
Insights
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.
Objectives:
Clinical features of Behçet's disease (BD) exhibit significant variability, not only from patient to patient but also according to gender and geographical region. This study aims to describe the clinical characteristics, identify distinct clusters in a large cohort of paediatric BD patients and compare the clinical manifestations of patients across three geographical regions: Turkey, Europe and Iran.
Method:
Patients with paediatric-onset BD (<18 years of age) from Turkey, Iran and European countries (France and Italy) were retrospectively evaluated. A follow-up period of at least 6 months was required for inclusion.
Results:
The study included 600 patients (297 females, 49.5%), with cases from Turkey (n=231), Iran (n=306), France (n=44) and Italy (n=19). The most common presentations were mucocutaneous involvement (97.5%), followed by ocular (48.0%), musculoskeletal (43.2%), neurological (11.8%), vascular (11.5%), gastrointestinal (9.0%) and cardiac (2.0%) involvement. Ocular involvement was more prevalent in Iran, gastrointestinal involvement in Europe, and musculoskeletal and vascular involvement in Turkey compared with the other geographical regions. Seven distinct clusters of paediatric BD as vascular (cluster 1 (C1)), mucocutaneous only (C2), ocular (C3), gastrointestinal (C4), mixed (C5), neurologic and ocular (C6), and mucocutaneous-musculoskeletal cluster (C7) were identified, although there was some overlap in system involvements.
Conclusions:
Our study supports the notion that BD may tend to present in certain clusters in children as well. Since BD is a complex disease with a multifactorial aetiology, involving the interaction of pathogenic pathways, classification of clusters presents a significant challenge. We have also shown that certain clinical features vary among geographical regions.
