Idiopathic pulmonary fibrosis: a disorder of epithelial cell dysfunction

DF Zoz, WE Lawson, TS Blackwell - The American journal of the medical …, 2011 - Elsevier
Idiopathic pulmonary fibrosis (IPF) is characterized by progressive dyspnea, interstitial
infiltrates in lung parenchyma and restriction on pulmonary function testing. IPF is the most
common and severe of the idiopathic interstitial pneumonias, with most individuals
progressing to respiratory failure. Multiple lines of evidence reveal prominent roles for
alveolar epithelial cells (AECs) in disease. The current disease paradigm is that ongoing or
repetitive injurious stimuli in the presence of a genetic or acquired dysfunctional type II AEC …