Idiopathic pulmonary fibrosis: an epithelial/fibroblastic cross-talk disorder

Respir Res. 2002;3(1):3. doi: 10.1186/rr175. Epub 2001 Oct 11.


Idiopathic pulmonary fibrosis is a chronic and usually progressive lung disorder of unknown etiology. A growing body of evidence suggests that, in contrast to other interstitial lung diseases, IPF is a distinct entity in which inflammation is a secondary and non-relevant pathogenic partner. Evidence includes the presence of similar mild/moderate inflammation either in early or late disease, and the lack of response to potent anti-inflammatory therapy. Additionally, it is clear from experimental models and some human diseases that it is possible to have fibrosis without inflammation. An evolving hypothesis proposes that IPF may result from epithelial micro-injuries and abnormal wound healing.

Publication types

  • Review

MeSH terms

  • Animals
  • Cell Communication / physiology*
  • Epithelial Cells / pathology*
  • Epithelial Cells / physiology
  • Fibroblasts / pathology*
  • Fibroblasts / physiology
  • Humans
  • Pulmonary Fibrosis / classification*
  • Pulmonary Fibrosis / pathology*
  • Pulmonary Fibrosis / physiopathology