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Updated: Sep 28, 2025

Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
Published on: June 16, 2020
Update on assessment and management of primary cardiac involvement in systemic sclerosis
Vasiliki-Kalliopi Bournia1, Christos Tountas1, Athanase D Protogerou2
1First Department of Propaedeutic and Internal Medicine and Joined Rheumatology Program, Medical School, National and Kapodistrian University of Athens, Laikon Hospital, Athens - Greece.
Insights
Systemic sclerosis frequently causes severe heart problems, often silently. Advanced imaging like cardiovascular magnetic resonance can detect these issues, potentially reducing related deaths.
Area of Science:
- Cardiology
- Rheumatology
- Medical Imaging
Background:
- Primary cardiac involvement is a frequent and severe complication of systemic sclerosis (SSc).
- SSc can affect all cardiac structures: pericardium, myocardium, endocardium, valves, and conduction system.
- Cardiac disease in SSc is often clinically silent, leading to delayed diagnosis.
Purpose of the Study:
- To review current understanding of cardiac complications in systemic sclerosis.
- To highlight the role of advanced imaging in diagnosing SSc-related heart disease.
- To emphasize the need for systematic assessment and therapeutic strategies.
Main Methods:
- Review of current literature on cardiac involvement in systemic sclerosis.
- Discussion of advanced imaging techniques, including speckle-tracking echocardiography and cardiovascular magnetic resonance (CMR).
- Detailed description of CMR protocols for assessing SSc cardiac disease (function, inflammation, fibrosis).
Main Results:
- Cardiac disease accounts for approximately 25% of SSc-related deaths.
- Advanced imaging modalities reveal abnormalities in the majority of SSc patients.
- CMR can effectively evaluate ventricular function, myocardial edema (T2-W), and fibrosis (late-gadolinium enhancement).
Conclusions:
- Cardiac complications are a major cause of mortality in systemic sclerosis.
- Early detection and characterization of cardiac involvement using advanced imaging are crucial.
- Further systematic studies and guidelines are needed for optimal management of SSc-related cardiac disease.
Abstract:
Primary cardiac involvement is a common and severe complication of systemic sclerosis, which may affect all of the hearts' structural components, including pericardium, myocardium, endocardium, cardiac valves, and conduction system. While cardiac disease can be clinically silent and only diagnosed in autopsy, new imaging modalities such as speckle-tracking echocardiography and cardiovascular magnetic resonance may reveal various abnormal findings in the majority of patients. Cardiovascular magnetic resonance evaluation should include assessment of left and right ventricular function, inflammation (STIR T2-weighted sequences (T2-W) for edema detection), and fibrosis (T1-weighted sequences 15 min after Gd-DTPA contrast medium injection (late-gadolinium enhancement). Notably, cardiac disease is responsible for about one-fourth of systemic sclerosis-related deaths. Systematic studies for the assessment and therapy of systemic sclerosis-related cardiac complications, as well as relevant guidelines from the European League Against Rheumatism and the American College of Rheumatology, are currently lacking. However, research advances reviewed herein allow for a better understanding of the mechanisms that alter cardiac function. Implementation of such knowledge should reduce cardiac morbidity and mortality in systemic sclerosis patients.
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