Dilara ÜNAL, Erdal SAĞ, Selcan DEMİR, Seza ÖZEN
Trends in Pediatrics - 2026;7(2):145-150
Objective: Behçet's disease (BD) is a variable-vessel vasculitis with multisystemic involvement. Although cytokine dysregulation has been extensively investigated, data on angiogenic and vascular marker profiles remain limited in pediatric BD. This exploratory study aimed to characterize circulating vascular and inflammatory mediator profiles in pediatric BD, compare them with deficiency of adenosine deaminase 2 (DADA2) and polyarteritis nodosa (PAN). It further aimed to assess whether these markers were associated with vascular or central nervous system (CNS) involvement in BD. Methods: Serum samples from BD (n=34), DADA2 (n=20), PAN (n=10), and healthy controls (n=8) were analyzed for thirteen vascular and inflammatory markers (TIE1, TIE2, FLT1, sT2, RAGE, CD40L, LIGHT, PlGF, TNF-alpha, IL-6, IL-10, IL-18, MCP-1) using a multiplex bead-based immunoassay. Statistical analyses included Kruskal-Wallis and Mann-Whitney U tests for group comparisons, logistic and linear regression for associations with clinical features, and Spearman correlation to explore interrelations among markers. Results: TIE2 levels were significantly reduced in BD compared with healthy controls (p=0.010). DADA2 patients exhibited a distinct angiogenic-inflammatory profile, with significantly elevated levels of TIE1, TIE2, FLT1, TNF-alpha, IL-10, IL-18, and MCP-1 (all p<0.010) compared with both BD and PAN. None of the circulating markers independently predicted vascular or CNS involvement in BD. Strong positive correlations among angiogenic mediators (FLT1, PlGF, LIGHT) suggested a coordinated vascular signaling pattern, particularly in DADA2. Conclusion: Although individual circulating mediators showed limited discriminatory performance across vasculitis subtypes, DADA2 displayed a more distinct vascular-inflammatory profile characterized by TNF-alpha- and TIE2-related pathways. In pediatric BD, reduced TIE2 may reflect altered endothelial homeostasis, although this finding should be interpreted cautiously. These results support the need for integrated multi-marker approaches and longitudinal sampling to better define vascular-immune phenotypes in pediatric vasculitis.
