Table 1.

Biomarkers of endothelial dysfunction in CTD-PAH.

Molecular Mechanisms of Endothelial Dysfunction	Biomarkers
Imbalance between vasoactive mediators	ET-1 [32,34,35,36,37,39] NO [37] ADMA [41,42]
Endothelial-to-mesenchymal transition	GDF-15 [53,54] Eng [56] FSTL-3, MDK [59] Chemerin [60] RAGE, MMP2 [61] proMMP-10 [62] TIMP-4 [63,64] OPN [70] UA [23,75,76]
Impaired neoangiogenesis	VEGF [78] VEGF-A165B [79] PlGF [79] ES [79,81] FLT-1 [83] NP-1 [60]
Platelet activation	vWF [85,86,88] TM [88,90]

ADMA, asymmetric dimethylarginine; Eng, endoglin; ES, endostatin; ET-1, endothelin-1; FLT-1, PlGF and VEGF receptor-1; FSTL-3, follistatin-like 3; GDF-15, growth differentiation factor-15; MDK, midkine; MMP, matrix metalloproteinase; NO, nitric oxide; NP-1, neuropilin-1; OPN, osteopontin; PlGF, placental growth factor; RAGE, receptor for advanced glycation end products; TIMP, tissue inhibitor of metalloproteinase; TM, thrombomodulin; UA, uric acid; VEGF, vascular endothelial growth factor; vWF, von Willebrand factor.