Many molecular and biological evidence highlight a key-role of the platelet-derived growth factor (PDGF) receptor, a tyrosine kinase-associated receptor, in the development of systemic sclerosis: in particular, imatinib is an anti-fibrotic drug belonging to the tyrosine kinase inhibitor family, that interferes with both transforming growth factor beta (TGF-β) and PDGF signaling pathways by blocking the activity of c-Abl, c-Kit, and PDGF receptors, respectively [30]. This evidence concerns the gene TGFB1 and systemic sclerosis.