PT  - JOURNAL ARTICLE
AU  - Toyoshi Yanagihara
AU  - Seidai Sato
AU  - Chandak Upagupta
AU  - Martin Kolb
TI  - What have we learned from basic science studies on idiopathic pulmonary fibrosis?
AID  - 10.1183/16000617.0029-2019
DP  - 2019 Sep 30
TA  - European Respiratory Review
PG  - 190029
VI  - 28
IP  - 153
4099  - http://err.ersjournals.com/content/28/153/190029.short
4100  - http://err.ersjournals.com/content/28/153/190029.full
SO  - EUROPEAN RESPIRATORY REVIEW2019 Sep 30; 28
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