Markers of vascular injury such as vascular endothelial growth factor (VEGF), endothelin-1 (ET-1), and von Willebrand factor (vWF), as well as the soluble adhesion molecules ICAM-1 and VCAM-1 have been showed to be elevated in SSc sera and associated with PAH [129, 130]. Here, VWF is linked to pulmonary arterial hypertension.