Tobal Rachid, Potjewijd Judith, van Empel Vanessa P M, Ysermans Renee, Schurgers Leon J, Reutelingsperger Chris P, Damoiseaux Jan G M C, van Paassen Pieter
Division of Nephrology and Clinical and Experimental Immunology, Department of Internal Medicine, Maastricht University Medical Center, Maastricht, Netherlands.
Department of Cardiology, Maastricht University Medical Center, Maastricht, Netherlands.
Front Med (Lausanne). 2021 Dec 22;8:806899. doi: 10.3389/fmed.2021.806899. eCollection 2021.
Pulmonary arterial hypertension (PAH) is a severe disease with high morbidity and mortality. Current therapies are mainly focused on vasodilative agents to improve prognosis. However, recent literature has shown the important interaction between immune cells and stromal vascular cells in the pathogenic modifications of the pulmonary vasculature. The immunological pathogenesis of PAH is known as a complex interplay between immune cells and vascular stromal cells, via direct contacts and/or their production of extra-cellular/diffusible factors such as cytokines, chemokines, and growth factors. These include, the B-cell-mast-cell axis, endothelium mediated fibroblast activation and subsequent M2 macrophage polarization, anti-endothelial cell antibodies and the versatile role of IL-6 on vascular cells. This review aims to outline the major pathophysiological changes in vascular cells caused by immunological mechanisms, leading to vascular remodeling, increased pulmonary vascular resistance and eventually PAH. Considering the underlying immunological mechanisms, these mechanisms may be key to halt progression of disease.
肺动脉高压（PAH）是一种发病率和死亡率都很高的严重疾病。目前的治疗主要集中在血管扩张剂上，以改善预后。然而，最近的文献表明，免疫细胞与肺血管系统致病改变中的基质血管细胞之间存在重要的相互作用。PAH的免疫发病机制被认为是免疫细胞与血管基质细胞之间通过直接接触和/或它们产生细胞外/可扩散因子（如细胞因子、趋化因子和生长因子）而发生的复杂相互作用。这些包括B细胞-肥大细胞轴、内皮介导的成纤维细胞活化及随后的M2巨噬细胞极化、抗内皮细胞抗体以及IL-6在血管细胞上的多种作用。本综述旨在概述由免疫机制引起的血管细胞主要病理生理变化，这些变化导致血管重塑、肺血管阻力增加并最终导致PAH。考虑到潜在的免疫机制，这些机制可能是阻止疾病进展的关键。
