Pathogenesis of idiopathic pulmonary fibrosis and its clinical implications

Expert Rev Clin Immunol. 2014 Aug;10(8):1005-17. doi: 10.1586/1744666X.2014.917050. Epub 2014 Jun 23.


Idiopathic pulmonary fibrosis (IPF) is the most common and lethal form of idiopathic interstitial pneumonia. The disease is thought to arise following an aberrant reparative response to recurrent alveolar epithelial cell injury leading to progressive loss of function. The median survival time is 3-5 years from diagnosis. Cigarette smoking, exposure to organic and inorganic dust and genetic factors have been shown to increase the risk of disease, although the cause of IPF remains elusive and its pathogenesis incompletely understood. In the last decade, several clinical trials evaluating novel therapies for IPF have been conducted but the results have been mostly disappointing. Conversely, compounds that target anti-fibrotic and growth factor pathways have been proven effective in slowing functional decline and disease progression. These promising results notwithstanding, truly effective therapeutic strategies will likely require combinations of drugs in order to target the multitude of pathways involved in disease pathogenesis.

Keywords: idiopathic pulmonary fibrosis; pathogenesis; pulmonary fibrosis; therapy; treatment.

Publication types

  • Review

MeSH terms

  • Animals
  • Clinical Trials as Topic
  • Environmental Exposure / adverse effects
  • Fibrinolytic Agents / therapeutic use*
  • Gene-Environment Interaction
  • Humans
  • Idiopathic Pulmonary Fibrosis / drug therapy*
  • Idiopathic Pulmonary Fibrosis / etiology
  • Intercellular Signaling Peptides and Proteins / metabolism
  • Respiratory Mucosa / pathology*
  • Smoking / adverse effects


  • Fibrinolytic Agents
  • Intercellular Signaling Peptides and Proteins