RT Journal Article
SR Electronic
T1 What have we learned from basic science studies on idiopathic pulmonary fibrosis?
JF European Respiratory Review
JO EUROPEAN RESPIRATORY REVIEW
FD European Respiratory Society
SP 190029
DO 10.1183/16000617.0029-2019
VO 28
IS 153
A1 Toyoshi Yanagihara
A1 Seidai Sato
A1 Chandak Upagupta
A1 Martin Kolb
YR 2019
UL http://err.ersjournals.com/content/28/153/190029.abstract
AB Idiopathic pulmonary fibrosis is a fatal age-related lung disease characterised by progressive and irreversible scarring of the lung. Although the details are not fully understood, there has been tremendous progress in understanding the pathogenesis of idiopathic pulmonary fibrosis, which has led to the identification of many new potential therapeutic targets. In this review we discuss several of these advances with a focus on genetic susceptibility and cellular senescence primarily affecting epithelial cells, activation of profibrotic pathways, disease-enhancing fibrogenic cell types and the role of the remodelled extracellular matrix.This review provides a summary of the most important findings in basic science investigations in pulmonary fibrosis and how they affect drug development and future patient management. http://bit.ly/2RjGMFZ