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Phenotype-associated microvascular differences in pediatric Behçet’s disease revealed by nailfold videocapillaroscopy

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Abstract

Behçet’s disease (BD) is a chronic multisystem vasculitis that may begin in childhood. Microvascular dysfunction is central to its pathogenesis, yet pediatric data are scarce. This study evaluated nailfold videocapillaroscopy (NVC) findings in children with BD using standardized methodology and compared microvascular parameters between complete and incomplete disease phenotypes, as well as with age-matched healthy reference data. In this cross-sectional study, pediatric BD patients fulfilling the pediatric Behçet’s disease (PEDBD) criteria underwent NVC following the European Alliance of Associations for Rheumatology (EULAR) microcirculation protocol. Capillary density, morphology, and microhemorrhages were compared between complete and incomplete BD and with age-matched healthy reference data. Associations between NVC parameters and clinical or laboratory findings were analyzed. Complete BD patients (n = 15, 40.5%) had significantly lower capillary density and higher apical loop width, tortuosity, dilatation, abnormal capillaries, and microhemorrhage scores than incomplete BD (n = 22, 59.5%). Compared with healthy peers, BD patients showed significantly lower capillary density, arterial and venous diameters, and capillary length but higher intercapillary distance and width.
Conclusion: Pediatric BD is associated with distinct NVC abnormalities compared with healthy reference data. Differences in capillary density and morphology were observed between complete and incomplete phenotypes. These findings provide a descriptive overview of phenotype-associated microvascular features in pediatric BD.
What Is Known:
• Nailfold videocapillaroscopy (NVC) can detect microvascular abnormalities in adult Behçet’s disease, but pediatric data are extremely limited.
• Childhood-onset Behçet’s disease often evolves over time from incomplete to complete phenotype, and reliable objective markers reflecting vascular involvement are lacking.
What Is New:
• Pediatric Behçet’s disease shows measurable NVC abnormalities compared with healthy children, even in the absence of clinically overt vascular involvement.
• Microvascular alterations are significantly more pronounced in complete phenotype than incomplete phenotype, suggesting cumulative vascular burden independent of disease activity scores.
Title
Phenotype-associated microvascular differences in pediatric Behçet’s disease revealed by nailfold videocapillaroscopy
Authors
Ufuk Furkan Ozdemir
Elif Kucuk
Lutfiye Koru
Feray Kaya
Zelal Aydin
Serpil Meric Toprak
Betul Aysegul Ayyildiz
Hatice Kubra Dursun
Eda Nur Dizman
Merve Ozen Balci
Kubra Ozturk
Fatih Haslak
Publication date
01-03-2026
Publisher
Springer Berlin Heidelberg
Published in
European Journal of Pediatrics / Issue 3/2026
Print ISSN: 0340-6199
Electronic ISSN: 1432-1076
DOI
https://doi.org/10.1007/s00431-026-06804-x
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