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Updated: May 28, 2026

Scanning Electron Microscopy of Macerated Tissue to Visualize the Extracellular Matrix
Published on: June 14, 2016
[Primary scleroderma myocardial disease]
1Rhumatologie A, Hôpital Cochin, AP-HP, 27 rue du Faubourg Saint-Jacques,--75014 Paris, Université Paris Descartes, France. andre.kahan@cch.aphp.fr
Insights
Systemic sclerosis commonly causes myocardial disease, often due to coronary artery vasospasm. Treatments targeting vasospasm may prevent heart complications in these patients.
Area of Science:
- Cardiology
- Rheumatology
- Pathophysiology
Context:
- Systemic sclerosis frequently involves the heart, impacting patient prognosis.
- Myocardial disease in systemic sclerosis is linked to coronary microvascular dysfunction.
Purpose:
- To explore the mechanisms of cardiac involvement in systemic sclerosis.
- To evaluate the efficacy of vasodilatory therapies for myocardial dysfunction.
Summary:
- Primary myocardial disease is common in systemic sclerosis, often caused by coronary artery vasospasm.
- Impaired myocardial perfusion and ventricular dysfunction are confirmed by advanced imaging.
- Vasodilators show promise in improving cardiac function and preventing complications.
Impact:
- Highlights the role of vasospasm in systemic sclerosis-related heart disease.
- Suggests potential therapeutic strategies for managing cardiac complications.
- Emphasizes the need for early detection and intervention in affected patients.
Abstract:
"Primary" myocardial disease is common in patients with systemic sclerosis and appears to be a factor of poor prognosis when clinically evident. An increasing body of evidence suggests that myocardial involvement is due, at least in part, to abnormal vasospasm, with or without associated structural abnormalities, of the small coronary arteries or arterioles. Impaired myocardial perfusion, as well as left and right ventricular dysfunction, have recently been confirmed in this setting, using more sensitive techniques such as tissue Doppler echocardiography and magnetic resonance imaging. Vasodilators such as calcium channel blockers, angiotensin-converting-enzyme inhibitors and an endothelin receptor antagonist improve both myocardial perfusion and functional abnormalities, and their systematic long-term administration might help to prevent these major complications.
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