In idiopathic pulmonary fibrosis (IPF), lung ECs exposed to chronic injury and pro-fibrotic stimuli (TGF-β, hypoxia) have been reported to undergo EndMT, contributing to the emergence of fibroblast-like or myofibroblast populations, excessive ECM deposition and interstitial scarring that disrupts the alveolar-capillary interface and impairs gas exchange [106,107,108]. Here, TGFB1 is linked to idiopathic pulmonary fibrosis.