Related Experiment Videos
Cardiac and skeletal muscle disease in systemic sclerosis (scleroderma): a high risk association
W P Follansbee1, T R Zerbe, T A Medsger
1Division of Cardiology, University of Pittsburgh School of Medicine, PA 15261.
Insights
Systemic sclerosis patients with skeletal myopathy face a higher risk of heart issues, including heart failure, arrhythmias, and cardiac death, especially sudden death.
Area of Science:
- Cardiology
- Rheumatology
- Neurology
Background:
- Systemic sclerosis (SSc) is a multisystem autoimmune disease.
- Cardiac and skeletal muscle involvement can occur in SSc, but their relationship is not fully understood.
Purpose of the Study:
- To investigate the association between skeletal myopathy and cardiac disease in patients with systemic sclerosis.
Main Methods:
- Retrospective review of computerized records of 1095 consecutive systemic sclerosis patients.
- Analysis of skeletal myopathy prevalence and cardiac disease indicators (myocardial disease, congestive heart failure, cardiac death).
Main Results:
- 17% of SSc patients had skeletal myopathy.
- Patients with myopathy showed significantly higher rates of myocardial disease (21% vs 10%), clinical congestive heart failure (CHF) (10% vs 4%), and cardiac death (8% vs 3%).
- A subgroup of 25 patients with coexistent myopathy and myocardial disease experienced severe cardiac dysfunction, conduction abnormalities (60%), and high mortality, with 67% dying suddenly.
Conclusions:
- Skeletal muscle disease is associated with cardiac muscle disease in systemic sclerosis.
- Systemic sclerosis patients with myopathy have an elevated risk of congestive heart failure, arrhythmias, and cardiac mortality, particularly sudden cardiac death.
Abstract:
To examine the possible relationship between cardiac and skeletal muscle disease in systemic sclerosis, we reviewed computerized records of 1095 consecutive patients with systemic sclerosis. One hundred eighty three (17%) had skeletal myopathy. Thirty-nine (21%) of the 183 fulfilled criteria for myocardial disease, compared with 90 (10%) of the 912 without myopathy (p < 0.0001.) Nineteen (10%) of the 183 had clinical CHF compared with 38 (4%) of the remainder (p < 0.002.) Fifteen (8%) of the patients with myopathy died of cardiac causes compared with 27 (3%) of the 912 without myopathy (p < 0.002.) Twenty-five patients with coexistent myopathy and myocardial disease, in the absence of other identifiable contributing causes, were identified. This group was characterized by a high incidence of cardiac conduction abnormalities (60%) and by the severity of the myocardial dysfunction and arrhythmias, both atrial and ventricular that they experienced. Eighteen of these 25 patients died; 12 (67%) died suddenly. Eight of the 18 (44%) had intractable CHF, which directly contributed to their deaths. Myocardial fibrosis was the predominant histologic abnormality at autopsy. However, autopsy of a patient who died in the context of acute "myocarditis" showed severe myocytolysis with contraction band necrosis but without inflammation or fibrosis; this is consistent with possible ischemically mediated injury. We conclude that skeletal and cardiac muscle disease in systemic sclerosis are associated. Patients with myopathy are at increased risk for CHF, sustained symptomatic arrhythmias, and cardiac death, particularly sudden death.