The transformation of endothelial cells in profibrotic myofibroblasts also seems to be integral for SSc-related fibrosis [6,8,10,50], since cells undergoing EndoMT were detected in the skin vessels of SSc patients, as well as in animal models of SSc (bleomycin-induced fibrosis/urokinase-type plasminogen activator receptor (uPAR)-deficient mice) [6]. This evidence concerns the gene PLAUR and systemic sclerosis.