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Updated: Jul 1, 2026

Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
Published on: June 16, 2020
Outcome measures for heart involvement in systemic sclerosis
1Department of Rheumatology A, Paris Descartes University, Cochin Hospital, AP-HP, Paris, France. yannick.allanore@cch.aphp.fr
Insights
Systemic sclerosis (SSc) often involves the heart, leading to fibrosis from ischemic injury. Early detection of cardiac dysfunction in SSc patients is crucial for improving prognosis and guiding treatment strategies.
Area of Science:
- Cardiology
- Rheumatology
- Medical Imaging
Background:
- Primary myocardial involvement is frequent in Systemic Sclerosis (SSc).
- Cardiac involvement in SSc is linked to focal ischemic injury and myocardial fibrosis.
- Early detection of cardiac dysfunction is vital due to its poor prognostic implications.
Purpose of the Study:
- To review current diagnostic modalities for cardiac involvement in SSc.
- To highlight the importance of pre-clinical identification of myocardial damage.
- To discuss the role of advanced imaging and biomarkers in SSc-related heart disease.
Main Methods:
- Echocardiography with pulsed tissue Doppler for heart assessment.
- Radionuclide ventriculography for ventricular function evaluation.
- Cardiac MRI for comprehensive assessment of ventricular function, perfusion, and tissue characteristics.
- 24-h ambulatory Holter ECG for arrhythmias.
- Natriuretic peptides for early heart dysfunction detection.
Main Results:
- Echocardiography is a cornerstone, while cardiac MRI offers comprehensive multi-parametric assessment.
- Cardiac MRI is poised to become the gold standard, surpassing SPECT for myocardial perfusion.
- Conduction abnormalities are common but generally benign; arrhythmias pose a significant risk.
- Natriuretic peptides may aid in identifying early cardiac dysfunction, especially in primary heart involvement.
Conclusions:
- Cardiac involvement in SSc necessitates thorough evaluation using a combination of imaging and monitoring techniques.
- Cardiac MRI provides unparalleled insights into SSc-related myocardial pathology.
- Prompt identification and management of cardiac dysfunction are essential for improving outcomes in SSc patients.
Abstract:
Primary myocardial involvement is common in SSc. Increasing evidence strongly suggests that this involvement is related to repeat focal ischaemic injury causing irreversible myocardial fibrosis. Clinically evident cardiac involvement is recognized to be a poor prognostic factor, thus pre-clinical identification is highly encouraged. Echocardiography, if possible coupled to pulsed tissue Doppler, is the cornerstone of heart assessment even if radionuclide ventriculography remains the gold standard for evaluation of ventricular function. Myocardial perfusion may be assessed by single photon emission CT, but cardiac MRI will probably supplant this technique; it, furthermore, offers the possibility to concomitantly determine, ventricular function, myocardial perfusion and tissular parameters (i.e. myocarditis or burden of fibrosis). Conduction system abnormalities are common but not serious, while arrhythmias may be life-threatening, necessitating 24-h ambulatory Holter ECG. Natriuretic peptides have been used mainly when the heart has been involved secondary to pulmonary arterial hypertension, but may also be useful for the identification of early heart dysfunction. Their predictive value should also be investigated when there is primary heart involvement.
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