TY - JOUR 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 DO - 10.1183/16000617.0161-2021 VL - 30 IS - 162 SP - 210161 AU - Frédéric Perros AU - Marc Humbert AU - Peter Dorfmüller Y1 - 2021/12/31 UR - http://err.ersjournals.com/content/30/162/210161.abstract N2 - 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 ER -