Phenotype-associated microvascular differences in pediatric Behçet's disease revealed by nailfold videocapillaroscopy.
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.
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.
• 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.
• 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.
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.
• 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.
• 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.
Authors
Ozdemir Ozdemir, Kucuk Kucuk, Koru Koru, Kaya Kaya, Aydin Aydin, Meric Toprak Meric Toprak, Ayyildiz Ayyildiz, Dursun Dursun, Dizman Dizman, Balci Balci, Ozturk Ozturk, Haslak Haslak
View on Pubmed