TGF-β, the leading cytokine in IPF, is responsible for the epithelial-to-mesenchymal transformation (EMT) of epithelial cells into a myofibroblastic phenotype that is related to the transformation of lung structure and the production of extracellular matrix (ECM) [74], thus, interference with its complex signaling cascade represents a reasonable therapeutic approach for IPF, supported by a strong mechanistic rationale. This evidence concerns the gene TGFB1 and idiopathic pulmonary fibrosis.