Pirfenidone is an anti-fibrotic agent with approval for the treatment of idiopathic pulmonary fibrosis (IPF) that also counteracts TGF-β signaling by downregulating pro-fibrotic cytokines, attenuating lung fibroblast proliferation, and decreasing extracellular matrix deposition [178–181]. Here, TGFB1 is linked to idiopathic pulmonary fibrosis.