全身性強皮症における心臓の関与：メカニズム、徴候、および管理

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Updated: Feb 6, 2026

Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease

Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease

Published on: June 16, 2020

Chin Yit Soo¹, Nicholas Jex¹, Lesley-Anne Bissell²

¹Multidisciplinary Cardiovascular Research Centre and Biomedical Imaging Science Department, Leeds Institute of Cardiovascular and Metabolic Medicine, University of Leeds, LS2 9JT, United Kingdom.

Rheumatology (Oxford, England)

|February 4, 2026

Abstract:

Cardiovascular disease remains a leading cause of mortality in Systemic Sclerosis (SSc). This review summarises key mechanisms in the pathophysiology of SSc primary heart involvement (SSc-PHI), presents the main clinical manifestations and their management, and discusses unmet needs in SSc-PHI with potential solutions. The pathophysiology of SSc-PHI involves both vascular and immune-mediated pathways, exacerbated by the NLRP3 inflammasome and pro-fibrotic cytokines, leading to myocardial fibrosis as the main cardiac manifestation. Regular screening is recommended to detect SSc-PHI, incorporating clinical assessments, cardiac biomarkers, and transthoracic echocardiography. Cardiac magnetic resonance imaging (CMR) enables detection of subclinical cardiac involvement, with certain CMR phenotypes holding prognostic significance. Early identification of subclinical disease is a critical unmet need to enhance risk stratification and improve outcomes. Emerging programs and cardiology-rheumatology collaboration will enhance interdisciplinary care and diagnostic strategies.

キーワード:

全身性強皮症心臓のエネルギー代謝心筋線維症心機能心臓MRI 冠状微小血管循環原発性心臓の関与予後

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Comprehensive Autopsy Program for Individuals with Multiple Sclerosis

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Mechanical Control of Relaxation Using Intact Cardiac Trabeculae

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Published on: February 17, 2023

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