Role of epithelial cells in idiopathic pulmonary fibrosis: from innocent targets to serial killers

Proc Am Thorac Soc. 2006 Jun;3(4):364-72. doi: 10.1513/pats.200601-003TK.


Idiopathic pulmonary fibrosis (IPF), a progressive and relentless lung scarring of unknown etiology, has been recognized as the most lethal interstitial lung disease. Despite the growing interest in IPF, the precise molecular mechanisms underlying the development of fibrosis and leading to the irreversible destruction of the lung are still unknown. Recently, it has been proposed that IPF, instead of being a chronic inflammatory disorder, results from multiple cycles of epithelial cell injury and activation. In turn, active alveolar epithelial cells provoke the migration, proliferation, and activation of mesenchymal cells with the formation of fibroblastic/myofibroblastic foci and the exaggerated accumulation of extracellular matrix, mirroring abnormal wound repair. In this article, some characteristics of the alveolar epithelium are briefly outlined, and the fibrogenic mechanisms specifically operated by active abnormal epithelial cells are examined.

Publication types

  • Review

MeSH terms

  • Apoptosis / physiology
  • Cell Differentiation
  • Cell Movement
  • Cell Proliferation
  • Epithelial Cells / pathology*
  • Fibroblasts / pathology
  • Humans
  • Mesoderm / cytology
  • Pulmonary Alveoli / cytology*
  • Pulmonary Fibrosis / physiopathology*