Myocardial Disease in Systemic Sclerosis: Recent Updates and Clinical Implications
- PMID: 39754676
- PMCID: PMC11864186
- DOI: 10.1007/s11886-024-02164-w
Myocardial Disease in Systemic Sclerosis: Recent Updates and Clinical Implications
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.
Keywords: Biomarkers; Cardiac disease; Echocardiography; Systemic sclerosis.
© 2024. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.
Conflict of interest statement
Declarations. Competing Interests: The authors declare no competing interests. Human and Animal Rights and Informed Consent: This article does not contain any studies with human or animal subjects performed by any of the authors.
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Bruni C, Buch MH, Djokovic A, De Luca G, Dumitru RB, Giollo A, Galetti I, Steelandt A, Bratis K, Suliman YA, et al. Consensus on the assessment of systemic sclerosis-associated primary heart involvement: World Scleroderma Foundation/Heart Failure Association guidance on screening, diagnosis, and follow-up assessment. J Scleroderma Relat Disord. 2023;8:169–182. doi: 10.1177/23971983231163413.
This consensus document provides essential, evidence-based guidance on the screening, diagnosis, and follow-up of SSc-associated cardiac involvement, incorporating the latest advancements in imaging and biomarkers to improve early detection and management.
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- Hsu S, Kokkonen-Simon KM, Kirk JA, Kolb TM, Damico RL, Mathai SC, Mukherjee M, Shah AA, Wigley FM, Margulies KB, et al. Right Ventricular Myofilament Functional Differences in Humans With Systemic Sclerosis-Associated Versus Idiopathic Pulmonary Arterial Hypertension. Circulation. 2018;137:2360–2370. doi: 10.1161/circulationaha.117.033147 - DOI - PMC - PubMed
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