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Smouldering fire or conflagration? An illustrated update on the concept of inflammation in pulmonary arterial hypertension

Pulmonary arterial hypertension (PAH) is a rare condition that is characterised by a progressive increase of pulmonary vascular resistances that leads to right ventricular failure and death, if untreated. The underlying narrowing of the pulmonary vasculature relies on several independent and interde...

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Detalles Bibliográficos
Autores principales: Perros, Frédéric, Humbert, Marc, Dorfmüller, Peter
Formato: Online Artículo Texto
Lenguaje:English
Publicado: European Respiratory Society 2021
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9488866/
https://www.ncbi.nlm.nih.gov/pubmed/34937704
http://dx.doi.org/10.1183/16000617.0161-2021
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author Perros, Frédéric
Humbert, Marc
Dorfmüller, Peter
author_facet Perros, Frédéric
Humbert, Marc
Dorfmüller, Peter
author_sort Perros, Frédéric
collection PubMed
description Pulmonary arterial hypertension (PAH) is a rare condition that is characterised by a progressive increase of pulmonary vascular resistances that leads to right ventricular failure and death, if untreated. The underlying narrowing of the pulmonary vasculature relies on several independent and interdependent biological pathways, such as genetic predisposition and epigenetic changes, imbalance of vasodilating and vasoconstrictive mediators, as well as dysimmunity and inflammation that will trigger endothelial dysfunction, smooth muscle cell proliferation, fibroblast activation and collagen deposition. Progressive constriction of the pulmonary vasculature, in turn, initiates and sustains hypertrophic and maladaptive myocardial remodelling of the right ventricle. In this review, we focus on the role of inflammation and dysimmunity in PAH which is generally accepted today, although existing PAH-specific medical therapies still lack targeted immune-modulating approaches.
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spelling pubmed-94888662022-11-14 Smouldering fire or conflagration? An illustrated update on the concept of inflammation in pulmonary arterial hypertension Perros, Frédéric Humbert, Marc Dorfmüller, Peter Eur Respir Rev Reviews Pulmonary arterial hypertension (PAH) is a rare condition that is characterised by a progressive increase of pulmonary vascular resistances that leads to right ventricular failure and death, if untreated. The underlying narrowing of the pulmonary vasculature relies on several independent and interdependent biological pathways, such as genetic predisposition and epigenetic changes, imbalance of vasodilating and vasoconstrictive mediators, as well as dysimmunity and inflammation that will trigger endothelial dysfunction, smooth muscle cell proliferation, fibroblast activation and collagen deposition. Progressive constriction of the pulmonary vasculature, in turn, initiates and sustains hypertrophic and maladaptive myocardial remodelling of the right ventricle. In this review, we focus on the role of inflammation and dysimmunity in PAH which is generally accepted today, although existing PAH-specific medical therapies still lack targeted immune-modulating approaches. European Respiratory Society 2021-12-22 /pmc/articles/PMC9488866/ /pubmed/34937704 http://dx.doi.org/10.1183/16000617.0161-2021 Text en Copyright ©The authors 2021 https://creativecommons.org/licenses/by-nc/4.0/This version is distributed under the terms of the Creative Commons Attribution Non-Commercial Licence 4.0. For commercial reproduction rights and permissions contact permissions@ersnet.org (mailto:permissions@ersnet.org)
spellingShingle Reviews
Perros, Frédéric
Humbert, Marc
Dorfmüller, Peter
Smouldering fire or conflagration? An illustrated update on the concept of inflammation in pulmonary arterial hypertension
title Smouldering fire or conflagration? An illustrated update on the concept of inflammation in pulmonary arterial hypertension
title_full Smouldering fire or conflagration? An illustrated update on the concept of inflammation in pulmonary arterial hypertension
title_fullStr Smouldering fire or conflagration? An illustrated update on the concept of inflammation in pulmonary arterial hypertension
title_full_unstemmed Smouldering fire or conflagration? An illustrated update on the concept of inflammation in pulmonary arterial hypertension
title_short Smouldering fire or conflagration? An illustrated update on the concept of inflammation in pulmonary arterial hypertension
title_sort smouldering fire or conflagration? an illustrated update on the concept of inflammation in pulmonary arterial hypertension
topic Reviews
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9488866/
https://www.ncbi.nlm.nih.gov/pubmed/34937704
http://dx.doi.org/10.1183/16000617.0161-2021
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