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[Mechanisms of cardiac involvement in systemic scleroderma, apropos of 3 cases]
A M Milesi-Lecat1, P Cauhape, J Schmidt
1Service de médecine interne, CHRU Nord, Clermont-Ferrand.
Insights
Systemic sclerosis can affect the heart, causing myocardial perfusion abnormalities and fibrosis. Unusual HLA DR expression on cardiac fibroblasts was consistently observed in affected patients.
Area of Science:
- Cardiology
- Rheumatology
- Immunology
Background:
- Systemic sclerosis is a multisystem autoimmune disease characterized by fibrosis, vascular dysfunction, and immune dysregulation.
- Cardiac involvement is a significant cause of morbidity and mortality in systemic sclerosis patients.
- Understanding myocardial manifestations is crucial for early diagnosis and management.
Observation:
- Three patients with systemic sclerosis underwent comprehensive cardiac evaluation.
- Diagnostic tools included echocardiography, coronary angiography, cardiac catheterization, and thallium scans.
- Endomyocardial biopsy with immunohistological analysis was performed for detailed myocardial assessment.
Findings:
- No significant coronary artery stenosis was found in any patient.
- All three patients exhibited abnormalities in myocardial perfusion.
- Two patients showed evidence of myocardial fibrosis.
- Consistent and unusual expression of HLA DR on cardiac fibroblasts was noted in all cases.
Implications:
- Myocardial perfusion abnormalities and fibrosis are key cardiac manifestations of systemic sclerosis.
- The aberrant expression of HLA DR on cardiac fibroblasts may play a role in the pathogenesis of cardiac involvement.
- Further research into the mechanisms underlying cardiac fibroblast activation is warranted.
Abstract:
We studied myocardial manifestations of systemic sclerosis in three patients. Two patients were symptomatic. A transthoracic echocardiography, a coronary angiography, a cardiac catheterization, thallium scans with dipyridamol completed by thoracic cold exposure and endomyocardial biopsy with immunohistological study were performed. None of them have coronary stenosis. In the three cases abnormalities of myocardial perfusion were detected, two of them have fibrosis. The immunohistological study always showed an anusual expression of HLA DR on cardiac fibroblasts.