Developmental pathways in the pathogenesis of lung fibrosis

D Chanda, E Otoupalova, SR Smith, T Volckaert… - Molecular aspects of …, 2019 - Elsevier
Idiopathic pulmonary fibrosis (IPF) is a progressive and terminal lung disease with no known
cure. IPF is a disease of aging, with median age of diagnosis over 65 years. Median survival
is between 3 and 5 years after diagnosis. IPF is characterized primarily by excessive
deposition of extracellular matrix (ECM) proteins by activated lung fibroblasts and
myofibroblasts, resulting in reduced gas exchange and impaired pulmonary function.
Growing evidence supports the concept of a pro-fibrotic environment orchestrated by …