Advances in therapies have led to the development and approval of two drugs that slow the rate of fibrosis and associated functional decline in IPF: nintedanib, a tyrosine kinase inhibitor that targets growth factor signaling downstream of VEGFR, FGFR, and PDGFR; pirfenidone, which exerts anti-inflammatory as well as anti-fibrotic effects via inhibition of collagen synthesis, downregulation of TGF-β and TNF alpha, and decreased fibroblast proliferation [177]. This evidence concerns the gene TGFB1 and idiopathic pulmonary fibrosis.