CCN2 and idiopathic pulmonary fibrosis: Among significantly upregulated genes in both SSc-ILD and IPF/UIP fibroblasts, we identify a recognised fibrosis signature, including smooth muscle actin (ACTA2), CTGF, and PAI1 (SERPINE1), along with genes more recently associated with lung fibrogenesis, including ID1, ID3, IL11, and NOX4. Inhibitor of DNA binding 1 and 3 (ID1 and ID3), are target genes of bone morphogenetic proteins, and control cell differentiation by dominant negative inhibition of helix-loop-helix transcription factors [39].