Epithelial injury and dysfunction in the pathogenesis of idiopathic PulmonaryFibrosis

NI Winters, A Burman, JA Kropski… - The American journal of the …, 2019 - Elsevier
Idiopathic pulmonary fibrosis is a disease of older adults leading to progressive dyspnea
and reduced exercise capacity, typically resulting in death within 3-5years of diagnosis.
Underlying genetic susceptibility combined with environmental insults is proposed to trigger
a chronic wound repair response, leading to activation of the fibrotic cascade. Perturbations
in several molecular pathways mediate vulnerability of the alveolar epithelium to injurious
agents, including the unfolded protein response, autophagy, mitophagy, and cellular …