Do risk scores in chronic thromboembolic pulmonary hypertension need to account for inflammation?

Extract Pulmonary hypertension (PH) encompasses a group of diseases characterised by pulmonary vascular remodelling and increased pulmonary artery pressure, leading to progressive right ventricular dysfunction and early mortality. Although the exact pathogenesis of many of the causes of PH are incompletely understood, evidence from histology, experimental animal models, and studies of biomarker profiles establishes a link between inflammation and PH, for example with the development of idiopathic pulmonary arterial hypertension (IPAH) and chronic thromboembolic pulmonary hypertension (CTEPH) [1, 2]. One of the most established proposed pathways involves the proinflammatory cytokine interleukin-6 (IL-6), which is consistently elevated in patients with IPAH and correlates with right ventricular dysfunction, disease severity and adverse outcomes [3–5]. In CTEPH, endothelial dysfunction is characterised by increased production of inflammatory cytokines, including IL-6, which facilitate smooth muscle cell proliferation and monocyte migration, supporting local inflammation and vascular remodelling [6].