肺动脉高压的肺外表现。

Expert review of respiratory medicine Pub Date : 2024-03-01 Epub Date: 2024-05-30 DOI:10.1080/17476348.2024.2361037
Navneet Singh, Nadine Al-Naamani, Mary Beth Brown, Gary Marshall Long, Thenappan Thenappan, Soban Umar, Corey E Ventetuolo, Tim Lahm
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引用次数: 0

摘要

导言:肺动脉高压(PAH)的肺外表现可能起着关键的病理生物学作用,深入了解肺外表现将有助于深入了解其机制和新的治疗靶点。本手稿回顾了我们对 PAH 肺外表现的理解:我们组建了一个专家小组,并在 PubMed 上进行了免费检索(2023 年 10 月至 2024 年 3 月)。在整个中枢神经系统中都能观察到炎症,尝试操纵炎症是向新型疗法迈出的令人鼓舞的一步。视网膜血管成像有望成为检测早期疾病和监测治疗反应的无创方法。PAH 患者的肠道菌群发生改变,菌群失调可能在全身炎症中发挥作用。尽管观察结果不一致,但肥胖、胰岛素抵抗和代谢失调的作用可能会通过对身体成分进行深度表型而得到阐明。代谢功能障碍、炎症和低灌注使骨骼肌功能障碍长期存在,但运动训练却能使骨骼肌功能受益。PAH 可观察到肾脏、肝脏和骨髓异常,这些异常可能代表终末器官损伤和疾病调节因素:对 PAH 全身表现的了解将阐明疾病机制和新的治疗靶点。还需要进行更多的研究,以了解肺外表现是 PAH 的原因还是影响,以及肺外表现如何影响预后。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Extrapulmonary manifestations of pulmonary arterial hypertension.

Introduction: Extrapulmonary manifestations of pulmonary arterial hypertension (PAH) may play a critical pathobiological role and a deeper understanding will advance insight into mechanisms and novel therapeutic targets. This manuscript reviews our understanding of extrapulmonary manifestations of PAH.

Areas covered: A group of experts was assembled and a complimentary PubMed search performed (October 2023 - March 2024). Inflammation is observed throughout the central nervous system and attempts at manipulation are an encouraging step toward novel therapeutics. Retinal vascular imaging holds promise as a noninvasive method of detecting early disease and monitoring treatment responses. PAH patients have gut flora alterations and dysbiosis likely plays a role in systemic inflammation. Despite inconsistent observations, the roles of obesity, insulin resistance and dysregulated metabolism may be illuminated by deep phenotyping of body composition. Skeletal muscle dysfunction is perpetuated by metabolic dysfunction, inflammation, and hypoperfusion, but exercise training shows benefit. Renal, hepatic, and bone marrow abnormalities are observed in PAH and may represent both end-organ damage and disease modifiers.

Expert opinion: Insights into systemic manifestations of PAH will illuminate disease mechanisms and novel therapeutic targets. Additional study is needed to understand whether extrapulmonary manifestations are a cause or effect of PAH and how manipulation may affect outcomes.

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