Progress in Understanding and Treating Idiopathic Pulmonary Fibrosis

Annu Rev Med. 2019 Jan 27;70:211-224. doi: 10.1146/annurev-med-041317-102715.


This is a time of substantial progress in the evaluation and care of patients with idiopathic pulmonary fibrosis (IPF). In addition to the approval and widespread availability of the first IPF-specific therapies, there have been improvements in imaging interpretation and lung biopsy methods to enable more expeditious and more accurate diagnosis. Recent advances in identifying genetic factors that underlie susceptibility to IPF and affect prognosis have raised the possibility of personalized therapeutic approaches in the future. Further, evolving work is elucidating novel mechanisms influencing epithelial, mesenchymal, and inflammatory cell responses during the injury-repair process, thus advancing understanding of disease pathogenesis. As analytic approaches mature, the field is now poised to harness the power of rapidly advancing "omics" technologies to further accelerate progress.

Keywords: alveolar epithelial cell; cryobiopsy; familial interstitial pneumonia; fibroblast; genetics; interstitial lung disease.

Publication types

  • Research Support, N.I.H., Extramural
  • Research Support, Non-U.S. Gov't
  • Research Support, U.S. Gov't, Non-P.H.S.
  • Review

MeSH terms

  • Aged
  • Biopsy, Needle
  • Chronic Disease
  • Comprehension
  • Disease Management*
  • Disease Progression
  • Female
  • Humans
  • Idiopathic Pulmonary Fibrosis / diagnosis*
  • Idiopathic Pulmonary Fibrosis / epidemiology
  • Idiopathic Pulmonary Fibrosis / therapy*
  • Immunohistochemistry
  • Indoles / administration & dosage*
  • Lung Transplantation / methods
  • Male
  • Middle Aged
  • Precision Medicine / trends
  • Prognosis
  • Pyridones / administration & dosage*
  • Respiratory Function Tests
  • Risk Assessment
  • Severity of Illness Index
  • Treatment Outcome


  • Indoles
  • Pyridones
  • pirfenidone
  • nintedanib