Update in diffuse parenchymal lung disease, 2013

Am J Respir Crit Care Med. 2015 Feb 1;191(3):270-4. doi: 10.1164/rccm.201405-0856UP.

Abstract

The period covered by this update can be considered as the most exciting period in idiopathic pulmonary fibrosis (IPF) research. It started with the identification of genetic variants that are associated with IPF in the majority of patients and continued with discovery of molecular and genetic biomarkers that predict distinct clinical presentations of patients with IPF and potential new biological mechanisms. More importantly, the period ends with the publication of two groundbreaking studies that confirmed that two drugs, pirfenidone and nintedanib, slowed disease progression, leading to a historic approval by the FDA. In this update, we describe these key advances, their scientific and significant clinical implications, and future directions.

Keywords: ILD; IPF; genomics; immunity; personalized medicine.

Publication types

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

MeSH terms

  • Anti-Inflammatory Agents, Non-Steroidal / therapeutic use*
  • Clinical Trials, Phase III as Topic
  • Disease Progression
  • Drug Approval
  • Enzyme Inhibitors / therapeutic use*
  • Genomics
  • Humans
  • Idiopathic Pulmonary Fibrosis / drug therapy*
  • Idiopathic Pulmonary Fibrosis / genetics
  • Indoles / therapeutic use*
  • Lung Diseases, Interstitial / drug therapy*
  • Lung Diseases, Interstitial / genetics
  • Polymorphism, Genetic
  • Pyridones / therapeutic use*
  • Treatment Outcome
  • United States
  • United States Food and Drug Administration

Substances

  • Anti-Inflammatory Agents, Non-Steroidal
  • Enzyme Inhibitors
  • Indoles
  • Pyridones
  • pirfenidone
  • nintedanib