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系统性硬化症中的心肌病:最新进展与临床意义

Myocardial Disease in Systemic Sclerosis: Recent Updates and Clinical Implications.

作者信息

Osgueritchian Ryan, Mombeini Hoda, Jani Vivek P, Hsu Steven, Hummers Laura K, Wigley Fredrick M, Mathai Stephen C, Shah Ami A, Mukherjee Monica

机构信息

Johns Hopkins University Division of Cardiology, Baltimore, MD, USA.

Johns Hopkins University Division of Rheumatology, Baltimore, MD, USA.

出版信息

Curr Cardiol Rep. 2025 Jan 4;27(1):3. doi: 10.1007/s11886-024-02164-w.

DOI:10.1007/s11886-024-02164-w
PMID:39754676
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11864186/
Abstract

PURPOSE OF REVIEW

The present review aims to address systemic sclerosis (SSc)-associated myocardial disease, a significant cause of morbidity and mortality, by examining the mechanisms of inflammation, microvascular dysfunction, and fibrosis that drive cardiac involvement. The objective is to elucidate critical risk factors and explore advanced diagnostic tools for early detection, enhancing patient outcomes by identifying those at highest risk.

RECENT FINDINGS

Recent studies underscore the importance of specific autoantibody profiles, disease duration, and cardiovascular comorbidities as key risk factors for severe cardiac manifestations in SSc. Additionally, advanced imaging techniques and biomarker analyses have emerged as pivotal tools for early identification and risk stratification. These innovations enable clinicians to detect subclinical myocardial involvement, potentially averting progression to symptomatic disease. SSc-associated myocardial disease remains challenging to predict, yet novel imaging modalities and biomarker-guided strategies offer a promising pathway for early diagnosis and targeted intervention. Integrating these approaches may enable more effective early detection and screening strategies as well as mitigation of disease progression, ultimately enhancing clinical outcomes for patients with SSc at-risk for adverse clinical outcomes.

摘要

综述目的

本综述旨在通过研究驱动心脏受累的炎症、微血管功能障碍和纤维化机制,探讨系统性硬化症(SSc)相关心肌病,这是发病和死亡的一个重要原因。目的是阐明关键危险因素,并探索用于早期检测的先进诊断工具,通过识别高危患者来改善患者预后。

最新发现

最近的研究强调了特定自身抗体谱、疾病持续时间和心血管合并症作为SSc严重心脏表现关键危险因素的重要性。此外,先进的成像技术和生物标志物分析已成为早期识别和风险分层的关键工具。这些创新使临床医生能够检测亚临床心肌受累,有可能避免进展为有症状的疾病。SSc相关心肌病的预测仍然具有挑战性,但新型成像模式和生物标志物指导的策略为早期诊断和靶向干预提供了一条有前景的途径。整合这些方法可能会实现更有效的早期检测和筛查策略以及减轻疾病进展,最终改善有不良临床结局风险的SSc患者的临床预后。

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Defining Echocardiographic Degrees of Right Heart Size and Function in Pulmonary Vascular Disease from the PVDOMICS Study.从PVDOMICS研究中定义肺血管疾病中右心大小和功能的超声心动图分级
Circ Cardiovasc Imaging. 2024 Oct;17(10). doi: 10.1161/circimaging.124.017074. Epub 2024 Oct 15.
2
Prognostic Value of Echocardiographic Coupling Metrics in Systemic Sclerosis-Associated Pulmonary Vascular Disease.超声心动图耦合指标在系统性硬化症相关肺血管疾病中的预后价值
J Am Soc Echocardiogr. 2025 Feb;38(2):115-126. doi: 10.1016/j.echo.2024.09.010. Epub 2024 Oct 1.
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Predictive accuracy of the ASIG algorithm in a prospective systemic sclerosis cohort undergoing annual screening for pulmonary arterial hypertension.ASIG 算法在接受年度肺动脉高压筛查的系统性硬化症前瞻性队列中的预测准确性。
Intern Med J. 2024 Sep;54(9):1561-1566. doi: 10.1111/imj.16468. Epub 2024 Aug 13.
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Clinical courses and predictors of left ventricular systolic dysfunction in systemic sclerosis: A cohort study.系统性硬化症患者左心室收缩功能障碍的临床病程及预测因素:一项队列研究。
Rheumatol Immunol Res. 2024 Jul 15;5(2):107-116. doi: 10.1515/rir-2024-0014. eCollection 2024 Jun.
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Poor cardiac output reserve in pulmonary arterial hypertension is associated with right ventricular stiffness and impaired interventricular dependence.肺动脉高压时的心输出量储备不足与右心室僵硬度及心室间依赖受损有关。
Eur Respir J. 2024 Jul 18;64(1). doi: 10.1183/13993003.00420-2024. Print 2024 Jul.
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Identifying consistent echocardiographic thresholds for risk stratification in pulmonary arterial hypertension.确定肺动脉高压风险分层的一致超声心动图阈值。
Pulm Circ. 2024 May 24;14(2):e12361. doi: 10.1002/pul2.12361. eCollection 2024 Apr.
7
Spectrum of renal disease in scleroderma other than scleroderma renal crisis: A review of the literature.硬皮病以外的硬皮病肾危象的肾脏疾病谱:文献复习。
Clin Nephrol. 2024 Aug;102(2):97-106. doi: 10.5414/CN111243.
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Semin Arthritis Rheum. 2024 Jun;66:152443. doi: 10.1016/j.semarthrit.2024.152443. Epub 2024 Apr 6.
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