Neurovascular dysregulation in systemic sclerosis: novel insights into pathophysiology, diagnosis, and treatment utilizing invasive cardiopulmonary exercise testing.

IF 5.2 2区 医学 Q1 RHEUMATOLOGY Current opinion in rheumatology Pub Date : 2024-11-11 DOI:10.1097/BOR.0000000000001070
Elizabeth Tarras, Phillip Joseph
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Abstract

Purpose of review: Pathologic abnormalities in skeletal muscle and the systemic vasculature are common in patients with systemic sclerosis (SSc). These abnormalities may lead to impaired systemic peripheral oxygen extraction (EO 2 ), known as neurovascular dysregulation, which may be because of abnormal blood flow distribution in the vasculature, microvascular shunting, and/or skeletal muscle mitochondrial dysfunction. Findings from invasive cardiopulmonary exercising testing (iCPET) provide important insights and enable diagnosis and treatment of this SSc disease manifestation.

Recent findings: Recent findings from noninvasive cardiopulmonary exercise testing (niCPET) support the existence of neurovascular dysregulation in patients with SSc. Invasive cardiopulmonary exercise testing (iCPET) has pointed to reduced systemic vascular distensibility as a possible mechanism for neurovascular dysregulation in patients with connective tissue diseases, including SSc.

Summary: Neurovascular dysregulation is likely an underappreciated cause of exercise impairment and dyspnea in patients with SSc in the presence or absence of underlying cardiopulmonary disease. It is posited to be related to microcirculatory and muscle dysfunction. Further studies are needed to clarify the pathophysiology of neurovascular dysregulation in SSc and to identify novel treatment targets and additional therapies.

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系统性硬化症的神经血管失调:利用侵入性心肺运动测试对病理生理学、诊断和治疗的新见解。
综述的目的:系统性硬化症(SSc)患者的骨骼肌和全身血管普遍存在病理异常。这些异常可能导致全身外周氧萃取(EO2)受损,即神经血管失调,其原因可能是血管中的血流分布异常、微血管分流和/或骨骼肌线粒体功能障碍。有创心肺运动试验(iCPET)的研究结果为诊断和治疗这种 SSc 疾病表现提供了重要依据:无创心肺运动测试(niCPET)的最新研究结果表明,SSc 患者存在神经血管失调。有创心肺运动测试(iCPET)显示,结缔组织疾病(包括 SSc)患者神经血管失调的可能机制之一是全身血管舒张性降低。据推测,这与微循环和肌肉功能障碍有关。需要进一步研究以明确 SSc 神经血管失调的病理生理学,并确定新的治疗目标和其他疗法。
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来源期刊
Current opinion in rheumatology
Current opinion in rheumatology 医学-风湿病学
CiteScore
9.70
自引率
2.00%
发文量
89
审稿时长
6-12 weeks
期刊介绍: A high impact review journal which boasts an international readership, Current Opinion in Rheumatology offers a broad-based perspective on the most recent and exciting developments within the field of rheumatology. Published bimonthly, each issue features insightful editorials and high quality invited reviews covering two or three key disciplines which include vasculitis syndromes, medical physiology and rheumatic diseases, crystal deposition diseases and rheumatoid arthritis. Each discipline introduces world renowned guest editors to ensure the journal is at the forefront of knowledge development and delivers balanced, expert assessments of advances from the previous year.
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