Idiopathic pulmonary fibrosis: unmasking cryptogenic environmental factors

Eur Respir J. 2019 Feb 21;53(2):1801699. doi: 10.1183/13993003.01699-2018. Print 2019 Feb.

Abstract

Idiopathic pulmonary fibrosis (IPF) is a progressive fibrotic lung disease of unknown origin that is associated with high morbidity and mortality. In this perspective, we briefly review the current understanding of the pathophysiology of IPF and the importance of environmental triggers as a precipitant of disease. We discuss occult intrinsic and extrinsic environmental factors that affect the lung microenvironment and may contribute to the development and progression of disease. The clinical implications of this framework need to be further elucidated, because prompt identification and elimination of occult exposures may represent a novel treatment modality.

Publication types

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

MeSH terms

  • Air Pollutants
  • Air Pollution
  • Biomarkers / blood
  • Disease Progression
  • Environmental Exposure*
  • Epigenesis, Genetic
  • Fibroblasts / metabolism
  • Gastroesophageal Reflux / physiopathology
  • Genetic Predisposition to Disease
  • Humans
  • Idiopathic Pulmonary Fibrosis / etiology
  • Idiopathic Pulmonary Fibrosis / genetics
  • Idiopathic Pulmonary Fibrosis / physiopathology*
  • Inflammation
  • Lung / microbiology
  • Lung / pathology*
  • Lung / physiopathology*
  • Occupational Exposure
  • Oxidative Stress
  • Risk Factors
  • Smoking / adverse effects
  • Surveys and Questionnaires

Substances

  • Air Pollutants
  • Biomarkers