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Updated: Jul 18, 2026

Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
Published on: June 16, 2020
[Heart involvement in systemic sclerosis]
Yannick Allanore1, André Kahan
1Université René Descartes, Faculté de Médicine, Service de Rhumatologie A, Hôpital Cochin, AP-HP, Paris. yannick.allanore@cch.aphp.fr
Insights
Systemic sclerosis commonly affects the heart, leading to myocardial fibrosis via microcirculatory impairment. Early treatment with calcium channel blockers and ACE inhibitors is crucial for managing cardiac involvement in systemic sclerosis.
Area of Science:
- Cardiology
- Rheumatology
- Vascular Biology
Background:
- Primary myocardial involvement is frequent in systemic sclerosis.
- Cardiac involvement, particularly myocardial fibrosis from ischemic injury, is a poor prognostic indicator.
- Microcirculatory impairment and abnormal vasoreactivity are implicated in the pathogenesis of cardiac dysfunction.
Purpose of the Study:
- To elucidate the mechanisms of cardiac involvement in systemic sclerosis.
- To highlight the prognostic significance of cardiac manifestations.
- To review current treatment strategies for myocardial involvement.
Main Methods:
- Review of existing evidence on cardiac involvement in systemic sclerosis.
- Analysis of pathological mechanisms including microcirculatory impairment and fibrosis.
- Evaluation of clinical outcomes and treatment efficacy.
Main Results:
- Myocardial fibrosis due to repeated focal ischemic injury is a key feature.
- Pericardial and conduction system abnormalities are common but often asymptomatic or non-serious.
- Arrhythmias can be life-threatening, while valvular involvement is minimal.
- Calcium channel blockers and ACE inhibitors are primary treatment modalities.
Conclusions:
- Cardiac involvement in systemic sclerosis, primarily myocardial fibrosis, significantly impacts prognosis.
- Management requires addressing microcirculatory dysfunction and utilizing specific pharmacotherapies.
- Long-term treatment with vasodilators is essential for patients with systemic sclerosis and cardiac involvement.
Abstract:
Primary myocardial involvement is common in systemic sclerosis. Increasing evidence strongly suggests that this involvement is related to repeated focal ischemic injury causing irreversible myocardial fibrosis. The underlying mechanism appears to be microcirculatory impairment with abnormal vasoreactivity, with or without structural vascular abnormalities. Clinically evident cardiac involvement is recognized to be a poor prognostic factor. Pericardial involvement is frequent but usually asymptomatic. Conduction system abnormalities appear common but not serious, while arrhythmias may be life-threatening. No significant valvular involvement appears to be associated with systemic sclerosis. Treatment for myocardial involvement includes long-term systematic administration of calcium channel blockers and possibly angiotensin-converting enzyme inhibitors, frequently given at high dosage.
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