VWF and systemic sclerosis: SSc-induced pulmonary vascular injury is characterized by the gradual loss of capillaries caused by the necrosis of vascular endothelial cells, which ultimately leads to tissue hypoxia and activation of dermal fibroblasts.Necrosis of endothelial cells can be evaluated using specific protein markers in serum, such as von Willebrand factor, vascular cell adhesion molecule-1 (VCAM-1), intercellular adhesion molecule-1 (ICAM-1), and E-selectin (SELE) [17–19].