Idiopathic pulmonary fibrosis: pathobiology of novel approaches to treatment

Clin Chest Med. 2012 Mar;33(1):69-83. doi: 10.1016/j.ccm.2011.11.002. Epub 2011 Dec 28.


Idiopathic pulmonary fibrosis (IPF) is a progressive disease of unknown cause that conveys a dismal prognosis. In the United States there are currently no licensed therapies for treatment of IPF. The development of effective IPF clinical trials networks across the United States and Europe, however, has led to key developments in the treatment of IPF. Advances in understanding of the pathogenetic processes involved in the development of pulmonary fibrosis have led to novel therapeutic targets. These developments offer hope that there may, in the near future, be therapeutic options available for treatment of this devastating disease.

Publication types

  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Acute Disease
  • Clinical Trials as Topic
  • Connective Tissue Growth Factor / drug effects
  • Connective Tissue Growth Factor / metabolism
  • Cytokines / drug effects
  • Cytokines / metabolism
  • Eicosanoids / pharmacology
  • Humans
  • Idiopathic Pulmonary Fibrosis / drug therapy*
  • Idiopathic Pulmonary Fibrosis / metabolism*
  • Idiopathic Pulmonary Fibrosis / physiopathology
  • Interleukin-13 / metabolism
  • NADPH Oxidases / drug effects
  • NADPH Oxidases / metabolism
  • Oxidative Stress / drug effects
  • Protein Kinases / drug effects
  • Protein Kinases / metabolism
  • Signal Transduction / drug effects
  • Th2 Cells / drug effects
  • Th2 Cells / metabolism
  • Transforming Growth Factor beta / drug effects
  • Transforming Growth Factor beta / metabolism
  • Treatment Failure


  • CCN2 protein, human
  • Cytokines
  • Eicosanoids
  • Interleukin-13
  • Transforming Growth Factor beta
  • Connective Tissue Growth Factor
  • NADPH Oxidases
  • Protein Kinases