Idiopathic pulmonary fibrosis
- PMID: 21719092
- DOI: 10.1016/S0140-6736(11)60052-4
Idiopathic pulmonary fibrosis
Abstract
Idiopathic pulmonary fibrosis is a devastating, age-related lung disease of unknown cause that has few treatment options. This disease was once thought to be a chronic inflammatory process, but current evidence indicates that the fibrotic response is driven by abnormally activated alveolar epithelial cells (AECs). These cells produce mediators that induce the formation of fibroblast and myofibroblast foci through the proliferation of resident mesenchymal cells, attraction of circulating fibrocytes, and stimulation of the epithelial to mesenchymal transition. The fibroblast and myofibroblast foci secrete excessive amounts of extracellular matrix, mainly collagens, resulting in scarring and destruction of the lung architecture. The mechanisms that link idiopathic pulmonary fibrosis with ageing and aberrant epithelial activation are unknown; evidence suggests that the abnormal recapitulation of developmental pathways and epigenetic changes have a role. In this Seminar, we review recent data on the clinical course, therapeutic options, and underlying mechanisms thought to be involved in the pathogenesis of idiopathic pulmonary fibrosis.
Copyright © 2011 Elsevier Ltd. All rights reserved.
Similar articles
-
Inflammation and dysregulated fibroblast proliferation--new mechanisms?Sarcoidosis Vasc Diffuse Lung Dis. 2013 Sep 1;30 Suppl 1:21-6. Sarcoidosis Vasc Diffuse Lung Dis. 2013. PMID: 24081240 Review.
-
Idiopathic pulmonary fibrosis.Thorax. 2012 Aug;67(8):742-6. doi: 10.1136/thoraxjnl-2011-200515. Epub 2011 Jun 22. Thorax. 2012. PMID: 21697287 Review.
-
Treatments in idiopathic pulmonary fibrosis: time for a more targeted approach?QJM. 2012 Oct;105(10):929-34. doi: 10.1093/qjmed/hcs076. Epub 2012 May 29. QJM. 2012. PMID: 22647761 Review.
-
Age-driven developmental drift in the pathogenesis of idiopathic pulmonary fibrosis.Eur Respir J. 2016 Aug;48(2):538-52. doi: 10.1183/13993003.00398-2016. Epub 2016 Jul 7. Eur Respir J. 2016. PMID: 27390284 Review.
-
Revealing the pathogenic and aging-related mechanisms of the enigmatic idiopathic pulmonary fibrosis. an integral model.Am J Respir Crit Care Med. 2014 May 15;189(10):1161-72. doi: 10.1164/rccm.201312-2221PP. Am J Respir Crit Care Med. 2014. PMID: 24641682
Cited by
-
Notch signaling regulates pulmonary fibrosis.Front Cell Dev Biol. 2024 Oct 10;12:1450038. doi: 10.3389/fcell.2024.1450038. eCollection 2024. Front Cell Dev Biol. 2024. PMID: 39450276 Free PMC article. Review.
-
First evidence of microplastic inhalation among free-ranging small cetaceans.PLoS One. 2024 Oct 16;19(10):e0309377. doi: 10.1371/journal.pone.0309377. eCollection 2024. PLoS One. 2024. PMID: 39413051 Free PMC article.
-
Preventive Effect of 3,3'-dimethoxy-4,4'-dihydroxy-stilbene Triazole on Pulmonary Fibrosis through Inhibition of Inflammation and Down-regulation of TGF-b Signaling Pathway.Dokl Biochem Biophys. 2024 Oct 11. doi: 10.1134/S1607672924600350. Online ahead of print. Dokl Biochem Biophys. 2024. PMID: 39400768
-
The involvement of HDAC3 in the pathogenesis of lung injury and pulmonary fibrosis.Front Immunol. 2024 Sep 26;15:1392145. doi: 10.3389/fimmu.2024.1392145. eCollection 2024. Front Immunol. 2024. PMID: 39391308 Free PMC article. Review.
-
Transformation of macrophages into myofibroblasts in fibrosis-related diseases: emerging biological concepts and potential mechanism.Front Immunol. 2024 Sep 25;15:1474688. doi: 10.3389/fimmu.2024.1474688. eCollection 2024. Front Immunol. 2024. PMID: 39386212 Free PMC article. Review.
Publication types
MeSH terms
LinkOut - more resources
Full Text Sources
Other Literature Sources