Many patients with systemic sclerosis may develop a myocarditis. Early recognition and treatment can reduce long-term morbidity and mortality.
Risk factors:
(1) myositis (often with proximal muscle weakness)
(2) possibly Raynaud's phenomenon
Clinical features:
(1) heart failure
(2) dyspnea
(3) angina
(4) heart block or other conduction defect
(5) supraventricular or ventricular arrhythmia
(6) evidence of pulmonary hypertension
Cardiovascular magnetic resonance (CMR) can identify myocardial fibrosis.
Severe cardiac involvement with heart failure may constitute a cardiomyopathy.
Improvement in cardiac findings after immunosuppression favors an immune mechanism.
Differential diagnosis:
(1) viral myocarditis
(2) other causes of myocardial fibrosis, including coronary artery disease
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Specialty: Immunology/Rheumatology
