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[Mechanisms in pulmonary fibrosis].

Authors
  • Crestani, Bruno1
  • Marchand-Adam, Sylvain
  • Fabre, Aurélie
  • Dehoux, Monique
  • Soler, Paul
  • 1 Unité Inserm 700, faculté Xavier-Bichat, université Paris-7, et service de pneumologie A, hôpital Bichat, 75018 Paris. [email protected]
Type
Published Article
Journal
La Revue du praticien
Publication Date
Dec 31, 2007
Volume
57
Issue
20
Pages
2222–2226
Identifiers
PMID: 18320741
Source
Medline
License
Unknown

Abstract

Despite the continuous and renewed interest in IPF, the precise biological mechanisms underlying the development of fibrosis and leading to the irreversible destruction of the lung are still unknown. Inflammation seems to play a minor role at the initiation of the disease. Identification of excessive apoptosis of alveolar epithelial cells led to the hypothesis that the disorder results from repeated alveolar epithelial cell injury and activation. In turn, alveolar epithelial cells induce the recruitment, proliferation, and activation of mesenchymal cells with the formation of fibroblastic foci and the abnormal accumulation of extracellular matrix. Fibroblastic foci are connected in a tridimensional reticulum. Circulating mesenchymal precursors called fibrocytes, and transdifferenciation of epithelial cells, endothelial cells and/or mesothelial cells, may all contribute to the accumulation of fibroblasts in the lung.

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