Molecular alterations of components of the SSc vasculature. Pericytes exhibit markers of immaturity or proliferation, including upregulation of RGS5 and PDGFRβ and downregulation of α-SMA. Endothelial cells are characterized by an increased level of the proapoptotic transcription factor Fra2, and decreased levels of the Fli1 transcription factor, VE-cadherin and PECAM1, which are indicative of impaired cell–cell contact, indicating a potential contribution to vessel leakiness. The levels of collagen type IV are reduced, suggesting alterations in the composition of the basement membrane. Abbreviations: α-SMA, α-smooth muscle actin; PDGFRβ, platelet-derived growth factor β receptor; PECAM1, platelet endothelial cell adhesion molecule 1; RGS5, regulator of G protein signaling 5; SSc, systemic sclerosis; VE-cadherin, vascular endothelial cadherin.