Pathology of idiopathic pulmonary fibrosis with particular focus on vascular endothelium and epithelial injury and their therapeutic potential
Lu, Wenying ; Teoh, Alan ; Waters, Maddison ; Haug, Greg ; Shakeel, Ilma ; Hassan, Imtaiyaz ; Shahzad, Affan Mahmood ; Callerfelt, Anna-Karin Larsson LU
; Piccari, Lucilla
and Sohal, Sukhwinder Singh
(2025)
In Pharmacology and Therapeutics
265.
- Abstract
Idiopathic pulmonary fibrosis (IPF) remains a challenging disease with no drugs available to change the trajectory. It is a condition associated with excessive and highly progressive scarring of the lungs with remodelling and extracellular matrix deposition. It is a highly "destructive" disease of the lungs. The diagnosis of IPF is challenging due to continuous evolution of the disease, which also makes early interventions very difficult. The role of vascular endothelial cells has not been explored in IPF in great detail. We do not know much about their contribution to arterial or vascular remodelling, extracellular matrix changes and contribution to pulmonary hypertension and lung fibrosis in general. Endothelial to mesenchymal... (More)
Idiopathic pulmonary fibrosis (IPF) remains a challenging disease with no drugs available to change the trajectory. It is a condition associated with excessive and highly progressive scarring of the lungs with remodelling and extracellular matrix deposition. It is a highly "destructive" disease of the lungs. The diagnosis of IPF is challenging due to continuous evolution of the disease, which also makes early interventions very difficult. The role of vascular endothelial cells has not been explored in IPF in great detail. We do not know much about their contribution to arterial or vascular remodelling, extracellular matrix changes and contribution to pulmonary hypertension and lung fibrosis in general. Endothelial to mesenchymal transition appears to be central to such changes in IPF. Similarly, for epithelial changes, the process of epithelial to mesenchymal transition seem to be the key both for airway epithelial cells and type-2 pneumocytes. We focus here on endothelial and epithelial cell changes and its contributions to IPF. In this review we revisit the pathology of IPF, mechanistic signalling pathways, clinical definition, update on diagnosis and new advances made in treatment of this disease. We discuss ongoing clinical trials with mode of action. A multidisciplinary collaborative approach is needed to understand this treacherous disease for new therapeutic targets.
(Less)
- author
- Lu, Wenying
; Teoh, Alan
; Waters, Maddison
; Haug, Greg
; Shakeel, Ilma
; Hassan, Imtaiyaz
; Shahzad, Affan Mahmood
; Callerfelt, Anna-Karin Larsson
LU
; Piccari, Lucilla
and Sohal, Sukhwinder Singh
- organization
- publishing date
- 2025-01
- type
- Contribution to journal
- publication status
- published
- subject
- keywords
- Humans, Idiopathic Pulmonary Fibrosis/pathology, Animals, Endothelium, Vascular/pathology, Epithelial-Mesenchymal Transition, Epithelial Cells/pathology
- in
- Pharmacology and Therapeutics
- volume
- 265
- article number
- 108757
- publisher
- Elsevier
- external identifiers
-
- scopus:85210059153
- pmid:39586361
- ISSN
- 0163-7258
- DOI
- 10.1016/j.pharmthera.2024.108757
- language
- English
- LU publication?
- yes
- additional info
- Copyright © 2024 The Authors. Published by Elsevier Inc. All rights reserved.
- id
- 54f236ad-1667-4284-b1af-9059612a4641
- date added to LUP
- 2025-01-27 13:28:34
- date last changed
- 2025-07-15 17:15:59
@article{54f236ad-1667-4284-b1af-9059612a4641,
abstract = {{<p>Idiopathic pulmonary fibrosis (IPF) remains a challenging disease with no drugs available to change the trajectory. It is a condition associated with excessive and highly progressive scarring of the lungs with remodelling and extracellular matrix deposition. It is a highly "destructive" disease of the lungs. The diagnosis of IPF is challenging due to continuous evolution of the disease, which also makes early interventions very difficult. The role of vascular endothelial cells has not been explored in IPF in great detail. We do not know much about their contribution to arterial or vascular remodelling, extracellular matrix changes and contribution to pulmonary hypertension and lung fibrosis in general. Endothelial to mesenchymal transition appears to be central to such changes in IPF. Similarly, for epithelial changes, the process of epithelial to mesenchymal transition seem to be the key both for airway epithelial cells and type-2 pneumocytes. We focus here on endothelial and epithelial cell changes and its contributions to IPF. In this review we revisit the pathology of IPF, mechanistic signalling pathways, clinical definition, update on diagnosis and new advances made in treatment of this disease. We discuss ongoing clinical trials with mode of action. A multidisciplinary collaborative approach is needed to understand this treacherous disease for new therapeutic targets.</p>}},
author = {{Lu, Wenying and Teoh, Alan and Waters, Maddison and Haug, Greg and Shakeel, Ilma and Hassan, Imtaiyaz and Shahzad, Affan Mahmood and Callerfelt, Anna-Karin Larsson and Piccari, Lucilla and Sohal, Sukhwinder Singh}},
issn = {{0163-7258}},
keywords = {{Humans; Idiopathic Pulmonary Fibrosis/pathology; Animals; Endothelium, Vascular/pathology; Epithelial-Mesenchymal Transition; Epithelial Cells/pathology}},
language = {{eng}},
publisher = {{Elsevier}},
series = {{Pharmacology and Therapeutics}},
title = {{Pathology of idiopathic pulmonary fibrosis with particular focus on vascular endothelium and epithelial injury and their therapeutic potential}},
url = {{http://dx.doi.org/10.1016/j.pharmthera.2024.108757}},
doi = {{10.1016/j.pharmthera.2024.108757}},
volume = {{265}},
year = {{2025}},
}