Key result
Pacemaker and steroid therapy successfully manage sick sinus syndrome in AMA-associated myopathy.
Why the study?
Anti-mitochondrial antibody-associated myopathy causes skeletal muscle disorders and arrhythmias, but cases where bradycardia-related symptoms precede skeletal muscle symptoms have not been well described.
Case Report (n=1)
Anti-mitochondrial antibody (AMA)-associated myopathy can initially present with cardiac symptoms such as sick sinus syndrome and arrhythmias before the onset of skeletal myopathy symptoms.
Consider AMA myopathy in unexplained sick sinus syndrome; extends phenotype but leaves association and management open for confirmation.
Background: Anti-mitochondrial antibody (AMA)-associated myopathy is known to be concomitant with primary biliary cirrhosis and to cause both skeletal muscle disorders and arrhythmias, myocardium disorders, and respiratory muscle disorders. We report a case of AMA-associated myopathy in which the bradycardia-related symptoms preceded the skeletal muscle symptoms. Case summary: A 59-year-old woman visited the emergency room in our hospital following a syncopal event. The patient was bradycardiac (45 b.p.m.) with a junctional rhythm resulting from sick sinus syndrome (SSS) and was suffering from heart failure. Blood tests revealed elevated creatine kinase (CK) and hepatic enzymes. She underwent permanent pacemaker implantation. However, it proved difficult to detect the electrical potential in the right atrium. Although successful atrial pacing was achieved at the lower right atrial septum, the atrial threshold was markedly high and she depended on ventricular pacing. One year later, neurological examination and muscle biopsy confirmed the diagnosis of AMA-associated myopathy. Following this diagnosis, steroid pulse therapy was initiated. Steroid administration relieved her symptoms and lowered the CK levels but the atrial standstill persisted. The patient takes low-dose prednisolone and has had an uneventful course for 3 years. Discussion: To the best of our knowledge, this is the first case of AMA-associated myopathy diagnosed by the first symptom related to bradycardia due to SSS. Patients with AMA-associated myopathy can experience a variety of cardiac symptoms, including arrhythmias, and initially complain of cardiac symptoms without symptoms of skeletal myopathy. This disease should be considered when diagnosing patients with arrhythmia and elevated CK.
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Ishiguro et al. (2022) conducted a case report in Anti-mitochondrial antibody (AMA)-associated myopathy with sick sinus syndrome (n=1). Permanent pacemaker implantation and steroid therapy was evaluated on Symptom relief and clinical course. A 59-year-old woman with AMA-associated myopathy initially presented with bradycardia due to sick sinus syndrome, which was successfully managed with pacemaker implantation and steroid therapy.
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