RT Journal Article
SR Electronic
T1 Smouldering fire or conflagration? An illustrated update on the concept of inflammation in pulmonary arterial hypertension
JF European Respiratory Review
JO EUROPEAN RESPIRATORY REVIEW
FD European Respiratory Society
SP 210161
DO 10.1183/16000617.0161-2021
VO 30
IS 162
A1 Perros, Frédéric
A1 Humbert, Marc
A1 Dorfmüller, Peter
YR 2021
UL http://err.ersjournals.com/content/30/162/210161.abstract
AB 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.Several pro-inflammatory triggers and pathways ignite and fuel persistent pulmonary vascular inflammation and obstructive vascular remodelling in PAH. Individual inflammatory susceptibility might reveal responder populations for immunomodulatory therapies. https://bit.ly/3GC1XMC