Pathogenesis, current treatments and future directions for idiopathic pulmonary fibrosis

Curr Opin Pharmacol. 2013 Jun;13(3):377-85. doi: 10.1016/j.coph.2013.03.015. Epub 2013 Apr 18.


Idiopathic pulmonary fibrosis (IPF) is a progressive interstitial lung disease (ILD) of unknown origin characterized by epithelial cell dysfunctions, accumulation of fibroblasts and myofibroblasts and relentless deposition of extracellular matrix (ECM). Improved diagnostic accuracy and better trial design have provided important insights from recent clinical trials. Perhaps the most important insight was the realization that 'standard therapy' was actually harmful! This review summarizes the current understanding of the cell types that are altered in IPF and the pathogenic mechanisms that have been identified. It also reviews recent clinical trial results and interpretations. Finally, we highlight attractive biologic targets and therapies in development with recommendations for future therapeutic avenues.

Publication types

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

MeSH terms

  • Animals
  • Comorbidity
  • Familial Primary Pulmonary Hypertension
  • Gastroesophageal Reflux / drug therapy
  • Gastroesophageal Reflux / epidemiology
  • Humans
  • Hypertension, Pulmonary / drug therapy
  • Hypertension, Pulmonary / epidemiology
  • Idiopathic Pulmonary Fibrosis* / epidemiology
  • Idiopathic Pulmonary Fibrosis* / pathology
  • Idiopathic Pulmonary Fibrosis* / therapy