Current and novel drug therapies for idiopathic pulmonary fibrosis

Drug Des Devel Ther. 2012;6:261-72. doi: 10.2147/DDDT.S29928. Epub 2012 Sep 26.


Over the past decade, there has been a cohesive effort from patients, physicians, clinical and basic scientists, and the pharmaceutical industry to find definitive treatments for idiopathic pulmonary fibrosis (IPF). As understanding of disease behavior and pathogenesis has improved, the aims of those treating IPF have shifted from reversing the disease to slowing or preventing progression of this chronic fibrotic illness. It is to be hoped that by slowing disease progression, survival will be improved from the current dismal median of 3.5 years following diagnosis. In Europe and Asia, a milestone has recently been reached with the licensing of the first IPF-specific drug, pirfenidone. This review assesses the current treatment modalities available for IPF, including pirfenidone. It also turns an eye to the future and discusses the growing number of promising compounds currently in development that it is hoped, in time, will make their way into the clinic as treatments for IPF.

Keywords: acute exacerbations; clinical trials; interstitial lung disease; pirfenidone; usual interstitial pneumonia.

Publication types

  • Review

MeSH terms

  • Acetylcysteine / therapeutic use
  • Amino Acid Oxidoreductases / physiology
  • Animals
  • Antiviral Agents / therapeutic use
  • Gastroesophageal Reflux / drug therapy
  • Humans
  • Idiopathic Pulmonary Fibrosis / drug therapy*
  • Interleukin-13 / antagonists & inhibitors
  • Lung Transplantation
  • MicroRNAs / physiology
  • Protein Kinase Inhibitors / therapeutic use
  • Pyridones / adverse effects
  • Pyridones / therapeutic use
  • Transforming Growth Factor beta / antagonists & inhibitors


  • Antiviral Agents
  • Interleukin-13
  • MicroRNAs
  • Protein Kinase Inhibitors
  • Pyridones
  • Transforming Growth Factor beta
  • pirfenidone
  • Amino Acid Oxidoreductases
  • LOXL2 protein, human
  • Acetylcysteine