Inflammation and immunity in the pathogenesis of pulmonary arterial hypertension.

Inflammation and immunity in the pathogenesis of pulmonary arterial hypertension.
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DOI:
10.1161/circresaha.113.301141
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发表时间:
2014-06-20
影响因子:
20.1
通讯作者:
Nicolls MR
Nicolls MR
中科院分区:
医学1区
文献类型:
--
作者:
Rabinovitch M;Guignabert C;Humbert M;Nicolls MR

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这篇综述总结了一个不断扩大的知识体系,表明未能解决炎症和免疫过程的改变是肺动脉高压发展的基础。趋化因子和细胞因子参与肺动脉高压,可以形成一个生物标志物平台进行了讨论。临床前研究提供了疾病的动物模型免疫失调的基础进行审查。此外,我们提出了针对目前正在招募患者的炎症/免疫机制的疗法,并讨论了其他正在开发的疗法。我们展示了遗传和代谢异常是如何与免疫失调和肺动脉不良重塑密不可分的。
This review summarizes an expanding body of knowledge indicating that failure to resolve inflammation and altered immune processes underlie the development of pulmonary arterial hypertension. The chemokines and cytokines implicated in pulmonary arterial hypertension that could form a biomarker platform are discussed. Pre-clinical studies that provide the basis for dysregulated immunity in animal models of the disease are reviewed. In addition we present therapies that target inflammatory/immune mechanisms that are currently enrolling patients and discuss others in development. We show how genetic and metabolic abnormalities are inextricably linked to dysregulated immunity and adverse remodeling in the pulmonary arteries.