Do all patients with idiopathic pulmonary fibrosis warrant a trial of therapeutic intervention? A pro-con perspective

Respirology. 2015 Apr;20(3):389-94. doi: 10.1111/resp.12484. Epub 2015 Feb 26.

Abstract

Idiopathic pulmonary fibrosis (IPF) is an incurable condition that is characterized by progressive pulmonary fibrosis, architectural distortion of the lung and loss of gas exchange units. Until recently, there was no effective treatment for this condition. However, there were two landmark trials published earlier this year, which have changed the management of this condition. Pirfenidone (Assessment of Pirfenidone to Confirm Efficacy and Safety in Idiopathic Pulmonary Fibrosis trial) and nintedanib (Efficacy and Safety of Nintedanib in Idiopathic Pulmonary Fibrosis-1 and -2 trials) have both demonstrated positive outcomes in patients with IPF. In this perspective, we critically discuss the role of these agents in IPF and in the broader pulmonary fibrosis population.

Keywords: idiopathic pulmonary fibrosis; nintedanib; pirfenidone; usual interstitial pneumonia.

Publication types

  • Review

MeSH terms

  • Antineoplastic Agents / pharmacology
  • Clinical Trials as Topic
  • Disease Management
  • Disease Progression
  • Humans
  • Idiopathic Pulmonary Fibrosis / diagnosis
  • Idiopathic Pulmonary Fibrosis / drug therapy*
  • Idiopathic Pulmonary Fibrosis / metabolism
  • Idiopathic Pulmonary Fibrosis / physiopathology
  • Indoles / pharmacology*
  • Lung* / pathology
  • Lung* / physiopathology
  • Pyridones / pharmacology*
  • Treatment Outcome
  • Tumor Necrosis Factor-alpha / antagonists & inhibitors

Substances

  • Antineoplastic Agents
  • Indoles
  • Pyridones
  • Tumor Necrosis Factor-alpha
  • pirfenidone
  • nintedanib