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[Mechanisms of cardiac involvement in systemic scleroderma. Apropos of 3 cases]
A M Milesi-Lecat1, O Aumaitre, J Schmidt
1Service de médecine interne, CHRU Nord, Clermont-Ferrand, France.
Insights
Progressive systemic sclerosis can affect the heart, causing myocardial perfusion abnormalities and fibrosis even without coronary artery narrowing. Unusual HLA class II antigen expression on cardiac fibroblasts was consistently observed.
Area of Science:
- Cardiology
- Rheumatology
- Immunology
Background:
- Progressive systemic sclerosis (PSS) is an autoimmune disease that can affect multiple organs.
- Cardiac involvement in PSS is a significant cause of morbidity and mortality.
- Understanding myocardial manifestations is crucial for patient management.
Observation:
- Three patients with PSS underwent comprehensive cardiac evaluation.
- Methods included echocardiography, cardiac catheterization, coronary angiography, thallium scans, and endomyocardial biopsy.
- Two patients presented with symptoms related to cardiac dysfunction.
Findings:
- All patients exhibited myocardial perfusion abnormalities without significant coronary stenosis.
- Myocardial fibrosis was present in two patients, with coronary spasm noted in one.
- Immunohistology consistently revealed abnormal HLA class II antigen expression on cardiac fibroblasts.
Implications:
- Myocardial perfusion defects and fibrosis in PSS may occur independently of coronary artery disease.
- Cardiac fibroblasts expressing HLA class II antigens could play a role in the pathogenesis of PSS-related cardiomyopathy.
- Further research into these mechanisms may lead to targeted therapies for cardiac manifestations in PSS.
Abstract:
To investigate myocardial manifestations in progressive systemic sclerosis, we studied three patients with transthoracic echocardiography, cardiac catheterization with coronary angiography, thallium scans with dipyridamol completed by thoracic cold exposure and endomyocardial biopsy with immunohistology. Two patients were symptomatic. In the three cases, abnormalities of myocardial perfusion were detected without coronary stenosis. Two patients had myocardial fibrosis with a coronary spasm in one case. The immunohistological study always showed an unusual expression of HLA class II antigen on cardiac fibroblasts.