Experimental evidence revealed that EndMT may also contribute to the establishment of SSc-associated interstitial lung disease and PAH [17,18], and that a variety of pathways implicated in the pathogenesis of SSc, including transforming growth factor-β (TGFβ), endothelin-1, Notch, Sonic Hedgehog, and Wnt signaling pathways, may trigger the EndMT process [15]. This evidence concerns the gene EDN1 and systemic sclerosis.