Insights into interstitial lung disease pathogenesis
(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
- Vasarmidi, Eirini ; Worrell, Julie C. ; Persson, Irma Mahmutovic LU ; Yaqub, Naheem ; Miądlikowska, Ewa ; Barnig, Cindy ; Boots, Agnes ; Reynaert, Niki L. and Ocaña, Sara Cuevas
- organization
- publishing date
- 2025-04
- 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}}, }