Upregulation of TGF-β and its receptors [30–32] and of the TGF-β-regulated gene CCN2 or connective tissue growth factor (CTGF) also known as CCN2 has been reported in SSc fibroblasts and tissue samples [33], although the increased expression of CCN2/CTGF is Smad3-independent but Smad1-dependent [34,35]. Here, TGFB1 is linked to systemic sclerosis.