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The pathogenesis of idiopathic pulmonary fibrosis.
Ther Adv Respir Dis 2010; 4(6):367-88TA

Abstract

Idiopathic pulmonary fibrosis (IPF) is a progressive fibrotic lung disease with an appalling prognosis. The failure of anti-inflammatory therapies coupled with the observation that deranged epithelium overlies proliferative myofibroblasts to form the fibroblastic focus has lead to the emerging concept that IPF is a disease of deregulated epithelial-mesenchymal crosstalk. IPF is triggered by an as yet unidentified alveolar injury that leads to activation of transforming growth factor-β (TGF-β) and alveolar basement membrane disruption. In the presence of persisting injurious pathways, or disrupted repair pathways, activated TGF-β can lead to enhanced epithelial apoptosis and epithelial-to-mesenchymal transition (EMT) as well as fibroblast, and fibrocyte, transformation into myofibroblasts which are resistant to apoptosis. The resulting deposition of excess disrupted matrix by these myofibroblasts leads to the development of IPF.

Authors+Show Affiliations

Nottingham Respiratory Biomedical Research Unit, Clinical Sciences Building, Nottingham City Campus, Nottingham, UK.No affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article
Research Support, Non-U.S. Gov't
Review

Language

eng

PubMed ID

20952439

Citation

Coward, William R., et al. "The Pathogenesis of Idiopathic Pulmonary Fibrosis." Therapeutic Advances in Respiratory Disease, vol. 4, no. 6, 2010, pp. 367-88.
Coward WR, Saini G, Jenkins G. The pathogenesis of idiopathic pulmonary fibrosis. Ther Adv Respir Dis. 2010;4(6):367-88.
Coward, W. R., Saini, G., & Jenkins, G. (2010). The pathogenesis of idiopathic pulmonary fibrosis. Therapeutic Advances in Respiratory Disease, 4(6), pp. 367-88. doi:10.1177/1753465810379801.
Coward WR, Saini G, Jenkins G. The Pathogenesis of Idiopathic Pulmonary Fibrosis. Ther Adv Respir Dis. 2010;4(6):367-88. PubMed PMID: 20952439.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - The pathogenesis of idiopathic pulmonary fibrosis. AU - Coward,William R, AU - Saini,Gauri, AU - Jenkins,Gisli, Y1 - 2010/10/15/ PY - 2010/10/19/entrez PY - 2010/10/19/pubmed PY - 2011/2/23/medline SP - 367 EP - 88 JF - Therapeutic advances in respiratory disease JO - Ther Adv Respir Dis VL - 4 IS - 6 N2 - Idiopathic pulmonary fibrosis (IPF) is a progressive fibrotic lung disease with an appalling prognosis. The failure of anti-inflammatory therapies coupled with the observation that deranged epithelium overlies proliferative myofibroblasts to form the fibroblastic focus has lead to the emerging concept that IPF is a disease of deregulated epithelial-mesenchymal crosstalk. IPF is triggered by an as yet unidentified alveolar injury that leads to activation of transforming growth factor-β (TGF-β) and alveolar basement membrane disruption. In the presence of persisting injurious pathways, or disrupted repair pathways, activated TGF-β can lead to enhanced epithelial apoptosis and epithelial-to-mesenchymal transition (EMT) as well as fibroblast, and fibrocyte, transformation into myofibroblasts which are resistant to apoptosis. The resulting deposition of excess disrupted matrix by these myofibroblasts leads to the development of IPF. SN - 1753-4666 UR - https://www.unboundmedicine.com/medline/citation/20952439/The_pathogenesis_of_idiopathic_pulmonary_fibrosis_ L2 - http://journals.sagepub.com/doi/full/10.1177/1753465810379801?url_ver=Z39.88-2003&rfr_id=ori:rid:crossref.org&rfr_dat=cr_pub=pubmed DB - PRIME DP - Unbound Medicine ER -