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Myocarditis as a complication in scleroderma patients with myositis
1Mount Sinai School of Medicine, Division of Rheumatology, New York, New York 10029.
Clinical Cardiology
|December 1, 1993
Summary
Systemic Sclerosis patients with myositis may develop severe myocarditis. Steroids alone improved myositis but not heart failure, suggesting inflammation is key.
Area of Science:
- Rheumatology and Cardiology
- Connective Tissue Diseases
- Inflammatory Myopathies
Background:
- Systemic Sclerosis (SSc), a diffuse scleroderma, can present with skeletal muscle myositis.
- Myocarditis, inflammation of the heart muscle, is a serious complication that can occur in SSc patients.
- Early diagnosis and appropriate management are crucial for improving outcomes in SSc patients with cardiac involvement.
Observation:
- Six patients with diffuse SSc and skeletal muscle myositis were observed.
- All patients exhibited severe myocarditis, indicated by elevated CPK-MB levels and significant left ventricular (LV) hypokinesis.
- While steroid therapy improved myositis, it did not prevent mortality from progressive LV failure in patients treated with steroids alone.
Findings:
- LV dysfunction in SSc patients with myositis appears to have a significant inflammatory component.
- Myocarditis may be subclinical in the initial stages of SSc-associated myositis.
- Elevated CPK-MB and LV function studies are recommended for all SSc patients diagnosed with myositis.
Implications:
- Aggressive or combination therapy may be necessary for managing SSc-associated myocarditis.
- Further research is needed to determine the optimal treatment strategies for this complex condition.
- Increased awareness and diagnostic vigilance for cardiac involvement in SSc patients with myositis are warranted.