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Insights into interstitial lung disease pathogenesis

Vasarmidi, Eirini ; Worrell, Julie C. ; Persson, Irma Mahmutovic LU ; Yaqub, Naheem ; Miądlikowska, Ewa ; Barnig, Cindy ; Boots, Agnes ; Reynaert, Niki L. and Ocaña, Sara Cuevas (2025) In Breathe 21(2).
Abstract

This review summarises some of the key features of interstitial lung diseases (ILDs) from a translational science point of view and brings insights into potential therapeutic options. Genetic predisposition and environmental factors like smoking, pollution and infections significantly impact the onset, progression and treatment response in ILDs, highlighting the need for personalised management. Fibroblasts are central to ILD pathology, influencing the tissue microenvironment, immune cell interactions and extracellular matrix (ECM) production, making them critical therapeutic targets. Monocyte-derived M2 macrophages drive fibrosis in idiopathic pulmonary fibrosis by secreting cytokines and remodelling the ECM. Understanding macrophage... (More)

This review summarises some of the key features of interstitial lung diseases (ILDs) from a translational science point of view and brings insights into potential therapeutic options. Genetic predisposition and environmental factors like smoking, pollution and infections significantly impact the onset, progression and treatment response in ILDs, highlighting the need for personalised management. Fibroblasts are central to ILD pathology, influencing the tissue microenvironment, immune cell interactions and extracellular matrix (ECM) production, making them critical therapeutic targets. Monocyte-derived M2 macrophages drive fibrosis in idiopathic pulmonary fibrosis by secreting cytokines and remodelling the ECM. Understanding macrophage subtypes and their dynamics offers new therapeutic possibilities. Chronic type 2 immunity contributes to fibrosis, emphasising the need to enhance protective markers in order to even out the balance shift of pathological immune responses in ILD treatments. Serum biomarkers like Krebs von den Lungen-6 (KL-6), surfactant protein (SFTP) D, matrix metalloproteinase-7 (MMP-7), and C-C motif chemokine ligand (CCL)-18 are valuable for diagnosing and predicting ILD progression, although more research is needed for clinical application. Animal models, especially bleomycin-based models, offer insights into ILD pathology, but challenges like lung hyperinflation highlight the need for careful model selection and translational research to bridge preclinical and clinical findings.

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author
; ; ; ; ; ; ; and
organization
publishing date
type
Contribution to journal
publication status
published
subject
in
Breathe
volume
21
issue
2
article number
240261
publisher
European Respiratory Society
external identifiers
  • pmid:40365095
  • scopus:105006834938
ISSN
1810-6838
DOI
10.1183/20734735.0261-2024
language
English
LU publication?
yes
id
58924251-5136-4348-9caf-548f7015c709
date added to LUP
2025-08-12 12:06:41
date last changed
2025-08-13 03:00:02
@article{58924251-5136-4348-9caf-548f7015c709,
  abstract     = {{<p>This review summarises some of the key features of interstitial lung diseases (ILDs) from a translational science point of view and brings insights into potential therapeutic options. Genetic predisposition and environmental factors like smoking, pollution and infections significantly impact the onset, progression and treatment response in ILDs, highlighting the need for personalised management. Fibroblasts are central to ILD pathology, influencing the tissue microenvironment, immune cell interactions and extracellular matrix (ECM) production, making them critical therapeutic targets. Monocyte-derived M2 macrophages drive fibrosis in idiopathic pulmonary fibrosis by secreting cytokines and remodelling the ECM. Understanding macrophage subtypes and their dynamics offers new therapeutic possibilities. Chronic type 2 immunity contributes to fibrosis, emphasising the need to enhance protective markers in order to even out the balance shift of pathological immune responses in ILD treatments. Serum biomarkers like Krebs von den Lungen-6 (KL-6), surfactant protein (SFTP) D, matrix metalloproteinase-7 (MMP-7), and C-C motif chemokine ligand (CCL)-18 are valuable for diagnosing and predicting ILD progression, although more research is needed for clinical application. Animal models, especially bleomycin-based models, offer insights into ILD pathology, but challenges like lung hyperinflation highlight the need for careful model selection and translational research to bridge preclinical and clinical findings.</p>}},
  author       = {{Vasarmidi, Eirini and Worrell, Julie C. and Persson, Irma Mahmutovic and Yaqub, Naheem and Miądlikowska, Ewa and Barnig, Cindy and Boots, Agnes and Reynaert, Niki L. and Ocaña, Sara Cuevas}},
  issn         = {{1810-6838}},
  language     = {{eng}},
  number       = {{2}},
  publisher    = {{European Respiratory Society}},
  series       = {{Breathe}},
  title        = {{Insights into interstitial lung disease pathogenesis}},
  url          = {{http://dx.doi.org/10.1183/20734735.0261-2024}},
  doi          = {{10.1183/20734735.0261-2024}},
  volume       = {{21}},
  year         = {{2025}},
}