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Endothelial to mesenchymal transition (EndMT) and vascular remodeling in pulmonary hypertension and idiopathic pulmonary fibrosis

Gaikwad, AV, Eapen, MS ORCID: 0000-0003-0570-7059, McAlinden, KD, Chia, C, Larby, J, Myers, S ORCID: 0000-0003-4793-3820, Dey, S, Haug, G, Markos, J, Glanville, AR and Sohal, SS ORCID: 0000-0001-9627-6498 2020 , 'Endothelial to mesenchymal transition (EndMT) and vascular remodeling in pulmonary hypertension and idiopathic pulmonary fibrosis' , Expert Review of Respiratory Medicine, vol. 14, no. 10 , pp. 1027-1043 , doi: 10.1080/17476348.2020.1795832.

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Introduction: Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive, and irreversible fibroticdisease associated with respiratory failure. The disease remains idiopathic, but repeated alveolarepithelium injury, disruption of alveolar-capillary integrity, abnormal vascular repair, and pulmonaryvascular remodeling are considered possible pathogenic mechanisms. Also, the development ofcomorbidities such as pulmonary hypertension (PH) could further impact disease outcome, quality oflife and survival rates in IPF.Areas covered: The current review provides a comprehensive literature survey of the mechanismsinvolved in the development and manifestations of IPF and their links to PH pathology. This review alsoprovides the current understanding of molecular mechanisms that link the two pathologies and willspecifically decipher the role of endothelial to mesenchymal transition (EndMT) along with the possibletriggers 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 raredisease. Thus, it is of utmost importance and urgency that the underlying profibrotic pathways andmechanisms are fully understood, to enable the development of novel therapeutic strategies.

Item Type: Article
Authors/Creators: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
Keywords: idiopathic pulmonary fibrosis, endothelial-to-mesenchymal transition and extracellular matrix, pulmonary arterial hypertension, pulmonary hypertension, pulmonary vascular remodeling
Journal or Publication Title: Expert Review of Respiratory Medicine
Publisher: Taylor & Francis
ISSN: 1747-6348
DOI / ID Number: 10.1080/17476348.2020.1795832
Copyright Information:

Copyright 2020 Informa UK Limited, trading as Taylor & Francis Group

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