Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 2020 Sep;26(5):429-435.
doi: 10.1097/MCP.0000000000000706.

Pathogenesis of fibrosis in interstitial lung disease

Olivier Burgy  1   2 , Pierre-Simon Bellaye  1   3 , Guillaume Beltramo  1   2   4 , Françoise Goirand  1   2 , Philippe Bonniaud  1   2   4
Affiliations

Pathogenesis of fibrosis in interstitial lung disease

Olivier Burgy et al. Curr Opin Pulm Med. 2020 Sep.

Abstract

Purpose of review: Pulmonary fibrosis is a chronic and progressive lung disease involving unclear pathological mechanisms. The present review presents and discusses the major and recent advances in our knowledge of the pathogenesis of lung fibrosis.

Recent findings: The past months have deepened our understanding on the cellular actors of fibrosis with a better characterization of the abnormal lung epithelial cells observed during lung fibrosis. Better insight has been gained into fibroblast biology and the role of immune cells during fibrosis. Mechanistically, senescence appears as a key driver of the fibrotic process. Extracellular vesicles have been discovered as participating in the impaired cellular cross-talk during fibrosis and deeper understanding has been made on developmental signaling in lung fibrosis.

Summary: This review emphasizes the contribution of different cell types and mechanisms during pulmonary fibrosis, highlights new insights for identification of potential therapeutic strategies, and underlines where future research is needed to answer remaining open questions.

PubMed Disclaimer

Similar articles

See all similar articles

Cited by

References

    1. Lederer DJ, Martinez FJ. Idiopathic pulmonary fibrosis. N Engl J Med 2018; 378:1811–1823.
    1. Bonniaud P, Fabre A, Frossard N, et al. Optimising experimental research in respiratory diseases: an ERS statement. Eur Respir J 2018; 51:1702133doi: 10.1183/13993003.02133-2017. - DOI
    1. Kolb P, Upagupta C, Vierhout M, et al. The importance of interventional timing in the bleomycin model of pulmonary fibrosis. Eur Respir J 2020; 55:1901105doi: 10.1183/13993003.01105-2019. - DOI
    1. Jiang P, Gil de Rubio R, Hrycaj SM, et al. Ineffectual AEC2-to-AEC1 differentiation in IPF: persistence of KRT8(hi) transitional state. Am J Respir Crit Care Med 2020.
    1. Duerr J, Leitz DH, Szczygiel M, et al. Conditional deletion of Nedd4–2 in lung epithelial cells causes progressive pulmonary fibrosis in adult mice. Nat Commun 2020; 11:2012.

Publication types

  NODES
twitter 2