Integrating mechanisms of pulmonary fibrosis

J Exp Med. 2011 Jul 4;208(7):1339-50. doi: 10.1084/jem.20110551.


Pulmonary fibrosis is a highly heterogeneous and lethal pathological process with limited therapeutic options. Although research on the pathogenesis of pulmonary fibrosis has frequently focused on the mechanisms that regulate the proliferation, activation, and differentiation of collagen-secreting myofibroblasts, recent studies have identified new pathogenic mechanisms that are critically involved in the initiation and progression of fibrosis in a variety of settings. A more detailed and integrated understanding of the cellular and molecular mechanisms of pulmonary fibrosis could help pave the way for effective therapeutics for this devastating and complex disease.

Publication types

  • Research Support, N.I.H., Intramural
  • Review

MeSH terms

  • Animals
  • Cell Differentiation
  • Cell Proliferation
  • Chemokines / physiology
  • Humans
  • Inflammation Mediators / physiology
  • Interleukin-13 / physiology
  • Leukocytes / pathology
  • Leukocytes / physiology
  • Macrophages / pathology
  • Macrophages / physiology
  • Models, Biological
  • Myofibroblasts / pathology
  • Pulmonary Fibrosis / etiology*
  • Pulmonary Fibrosis / pathology
  • Pulmonary Fibrosis / physiopathology
  • Th2 Cells / physiology
  • Transforming Growth Factor beta / physiology
  • Wound Healing


  • Chemokines
  • Inflammation Mediators
  • Interleukin-13
  • Transforming Growth Factor beta