Association of Lymphangiogenic Factors With Pulmonary Arterial Hypertension in Systemic Sclerosis

Objective Pulmonary arterial hypertension (PAH) is a major complication in systemic sclerosis (SSc), disease marked by vascular and lymphatic vessel abnormalities. This study was undertaken to assess the role of lymphangiogenic factors endothelial growth factor C (VEGF-C) angiopoietin 2 (Ang-2) soluble forms their respective cognate receptors, VEGF receptor 3 (sVEGFR-3) TIE-2, patients with SSc, evaluate predictive ability as markers for PAH development SSc. Methods In this cohort study, we used multiplex bead assays serum levels well-characterized SSc cohorts: an unselected identification from Oslo University Hospital (n = 371), PAH-enriched validation Zurich 149). As controls cohorts, obtained samples 100 healthy individuals 68 individuals, respectively. Patients whom SSc-related identified right-sided heart catheterization (RHC) both cohorts were studied prediction analyses. defined according European Society Cardiology/European Respiratory 2015 guidelines diagnosis treatment PAH. Associations risk assessed logistic regression Cox analyses expressed hazard ratio (HR) 95% confidence interval (95% CI). Results cohort, had lower mean VEGF-C higher Ang-2 compared (for VEGF-C, ± SD 2.1 0.5 ng/ml versus 2.5 0.4 controls; Ang-2, 6.1 7.6 2.8 1.8 each P < 0.001); these same trends observed those without The association SSc-PAH confirmed RHC cohort. For analyses, assembled all 251 cases cohorts. multivariable adjusted age sex, sVEGFR-3 HR 0.53 [95% CI 0.29–0.97], 0.04; sVEGFR-3, 1.21 1.01–1.45], 0.042). Conclusion These findings support notion that lymphangiogenesis deregulated during indicate could be promising marker early detection