Kavita Sharma1, Ana-Maria Orbai2, Dipan Desai2, Oscar H Cingolani2, Marc K Halushka3, Lisa Christopher-Stine2, Andrew L Mammen4, Katherine C Wu2, Sammy Zakaria2. 1. Department of Medicine, Johns Hopkins University School of Medicine, Baltimore, Maryland. Electronic address: ksharma8@jhmi.edu. 2. Department of Medicine, Johns Hopkins University School of Medicine, Baltimore, Maryland. 3. Department of Pathology, Johns Hopkins University School of Medicine, Baltimore, Maryland. 4. Department of Medicine, Johns Hopkins University School of Medicine, Baltimore, Maryland; Department of Neurology, Johns Hopkins University School of Medicine, Baltimore, Maryland.
Abstract
BACKGROUND: The antisynthetase (AS) syndrome is characterized by autoimmune myopathy, interstitial lung disease, cutaneous involvement, arthritis, fever, and antibody specificity. We describe 2 patients with AS syndrome who also developed myocarditis, depressed biventricular function, and congestive heart failure. METHODS AND RESULTS: Both patients were diagnosed with AS syndrome based on clinical manifestations, detection of serum AS antibodies, and myositis confirmation with the use of skeletal muscle magnetic resonance imaging and skeletal muscle biopsy. In addition, myocarditis resulting in heart failure was confirmed with the use of cardiac magnetic resonance imaging and from endomyocardial biopsy findings. After treatment for presumed AS syndrome-associated myocarditis, one patient recovered and the other patient died. CONCLUSIONS: AS syndrome is a rare entity with morbidity and mortality typically attributed to myositis and lung involvement. This is the first report of AS syndrome-associated myocarditis leading to congestive heart failure in 2 patients. Given the potentially fatal consequences, myocarditis should be considered in patients with AS syndrome presenting with heart failure.
BACKGROUND: The antisynthetase (AS) syndrome is characterized by autoimmune myopathy, interstitial lung disease, cutaneous involvement, arthritis, fever, and antibody specificity. We describe 2 patients with AS syndrome who also developed myocarditis, depressed biventricular function, and congestive heart failure. METHODS AND RESULTS: Both patients were diagnosed with AS syndrome based on clinical manifestations, detection of serum AS antibodies, and myositis confirmation with the use of skeletal muscle magnetic resonance imaging and skeletal muscle biopsy. In addition, myocarditis resulting in heart failure was confirmed with the use of cardiac magnetic resonance imaging and from endomyocardial biopsy findings. After treatment for presumed AS syndrome-associated myocarditis, one patient recovered and the other patient died. CONCLUSIONS: AS syndrome is a rare entity with morbidity and mortality typically attributed to myositis and lung involvement. This is the first report of AS syndrome-associated myocarditis leading to congestive heart failure in 2 patients. Given the potentially fatal consequences, myocarditis should be considered in patients with AS syndrome presenting with heart failure.
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