Why do patients get idiopathic pulmonary fibrosis Current concepts in the pathogenesis of pulmonary fibrosisReport as inadecuate

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BMC Medicine

, 13:176

First Online: 24 September 2015Received: 24 June 2015Accepted: 25 June 2015DOI: 10.1186-s12916-015-0412-6

Cite this article as: Bellaye, PS. & Kolb, M. BMC Med 2015 13: 176. doi:10.1186-s12916-015-0412-6


Idiopathic pulmonary fibrosis IPF is a devastating lung disease of unknown origin. Recent findings suggest that IPF results from multiple factors that eventually lead to interstitial lung injury. In the pathogenesis it is likely that complex relationships between genetic predispositions, environmental exposures, and lung infections promote the fibrotic processes causing IPF; it is this complexity and the multiplicity of causes that make the population and clinical course of IPF so heterogeneous. Thus, it is clear that one common factor driving IPF pathogenesis in all patients would be far too simplified of an understanding. In recent years, efforts have been made in finding therapeutic strategies that target disease progression rather than disease onset. The biochemical composition and abnormal stiffness of the matrix might be crucial in controlling the cellular phenotype in fibrotic lungs that promotes disease progression and persistence. Though there has been substantial progress in the IPF field in recent years, much more work is required in order to improve the prognosis associated with this disease.

KeywordsExtracellular matrix Fibrogenesis Idiopathic pulmonary fibrosis Lung injury Myofibroblast Wound healing AbbreviationsECMExtracellular matrix

IPFIdiopathic pulmonary fibrosis

TGF-β1Transforming growth factor-β1

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Author: Pierre-Simon Bellaye - Martin Kolb

Source: https://link.springer.com/

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