Animal models of fibrotic lung disease

Am J Respir Cell Mol Biol. 2013 Aug;49(2):167-79. doi: 10.1165/rcmb.2013-0094TR.

Abstract

Interstitial lung fibrosis can develop as a consequence of occupational or medical exposure, as a result of genetic defects, and after trauma or acute lung injury leading to fibroproliferative acute respiratory distress syndrome, or it can develop in an idiopathic manner. The pathogenesis of each form of lung fibrosis remains poorly understood. They each result in a progressive loss of lung function with increasing dyspnea, and most forms ultimately result in mortality. To better understand the pathogenesis of lung fibrotic disorders, multiple animal models have been developed. This review summarizes the common and emerging models of lung fibrosis to highlight their usefulness in understanding the cell-cell and soluble mediator interactions that drive fibrotic responses. Recent advances have allowed for the development of models to study targeted injuries of Type II alveolar epithelial cells, fibroblastic autonomous effects, and targeted genetic defects. Repetitive dosing in some models has more closely mimicked the pathology of human fibrotic lung disease. We also have a much better understanding of the fact that the aged lung has increased susceptibility to fibrosis. Each of the models reviewed in this report offers a powerful tool for studying some aspect of fibrotic lung disease.

Publication types

  • Research Support, N.I.H., Extramural
  • Research Support, U.S. Gov't, Non-P.H.S.
  • Review

MeSH terms

  • Animals
  • Cell Communication*
  • Disease Models, Animal*
  • Epithelial Cells / metabolism
  • Epithelial Cells / pathology
  • Genetic Diseases, Inborn* / genetics
  • Genetic Diseases, Inborn* / metabolism
  • Genetic Diseases, Inborn* / pathology
  • Genetic Diseases, Inborn* / physiopathology
  • Humans
  • Lung Diseases, Interstitial / genetics
  • Lung Diseases, Interstitial / metabolism
  • Lung Diseases, Interstitial / pathology
  • Lung Diseases, Interstitial / physiopathology
  • Pulmonary Alveoli / metabolism
  • Pulmonary Alveoli / pathology
  • Pulmonary Alveoli / physiopathology
  • Pulmonary Fibrosis* / genetics
  • Pulmonary Fibrosis* / metabolism
  • Pulmonary Fibrosis* / pathology
  • Pulmonary Fibrosis* / physiopathology
  • Respiratory Distress Syndrome, Adult / genetics
  • Respiratory Distress Syndrome, Adult / metabolism
  • Respiratory Distress Syndrome, Adult / pathology
  • Respiratory Distress Syndrome, Adult / physiopathology
  • Respiratory Mucosa / metabolism
  • Respiratory Mucosa / pathology
  • Respiratory Mucosa / physiopathology