Gaikwad, AV and Eapen, MS and McAlinden, KD and Chia, C and Larby, J and Myers, S and Dey, S and Haug, G and Markos, J and Glanville, AR and Sohal, SS, Endothelial to mesenchymal transition (EndMT) and vascular remodeling in pulmonary hypertension and idiopathic pulmonary fibrosis, Expert Review of Respiratory Medicine, 14, (10) pp. 1027-1043. ISSN 1747-6348 (2020) [Refereed Article]
Copyright 2020 Informa UK Limited, trading as Taylor & Francis Group
Introduction: Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive, and irreversible fibrotic disease associated with respiratory failure. The disease remains idiopathic, but repeated alveolar epithelium injury, disruption of alveolar-capillary integrity, abnormal vascular repair, and pulmonary vascular remodeling are considered possible pathogenic mechanisms. Also, the development of comorbidities such as pulmonary hypertension (PH) could further impact disease outcome, quality of life and survival rates in IPF.
Areas covered: The current review provides a comprehensive literature survey of the mechanisms involved in the development and manifestations of IPF and their links to PH pathology. This review also provides the current understanding of molecular mechanisms that link the two pathologies and will specifically decipher the role of endothelial to mesenchymal transition (EndMT) along with the possible triggers of EndMT. The possibility of targeting EndMT as a therapeutic option in IPF is discussed.
Expert opinion: With a steady increase in prevalence and mortality, IPF is no longer considered a rare disease. Thus, it is of utmost importance and urgency that the underlying profibrotic pathways and mechanisms are fully understood, to enable the development of novel therapeutic strategies.
|Item Type:||Refereed Article|
|Keywords:||idiopathic pulmonary fibrosis, endothelial-to-mesenchymal transition and extracellular matrix, pulmonary arterial hypertension, pulmonary hypertension, pulmonary vascular remodeling|
|Research Division:||Biomedical and Clinical Sciences|
|Research Group:||Cardiovascular medicine and haematology|
|Research Field:||Respiratory diseases|
|Objective Group:||Clinical health|
|Objective Field:||Clinical health not elsewhere classified|
|UTAS Author:||Gaikwad, AV (Ms Archana Gaikwad)|
|UTAS Author:||Eapen, MS (Dr Mathew Eapen)|
|UTAS Author:||McAlinden, KD (Mr Kielan McAlinden)|
|UTAS Author:||Larby, J (Dr Josie Larby)|
|UTAS Author:||Myers, S (Dr Stephen Myers)|
|UTAS Author:||Dey, S (Mr Surajit Dey)|
|UTAS Author:||Haug, G (Dr Greg Haug)|
|UTAS Author:||Markos, J (Dr Jim Markos)|
|UTAS Author:||Sohal, SS (Dr Sukhwinder Sohal)|
|Web of Science® Times Cited:||21|
|Deposited By:||Health Sciences|
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