Juvenile dermatomyositis (JDM) is an auto-immune inflammatory condition associated with cardiac disorders including conduction abnormalities and myocardial dysfunction. The time of presentation of cardiac abnormalities can range from disease onset to after long-term follow-up, emphasising the importance of screening for cardiac involvement in JDM. A previously healthy 10-year-old girl presented with syncope, fatigue and weakness associated with a heliotrope rash. JDM was diagnosed based on the clinical, laboratory and imaging findings. An ECG demonstrated complete heart block (CHB). All symptoms resolved following treatment with parenteral corticosteroids. In JDM, it is important to investigate for cardiac manifestations and in CHB to consider administering corticosteroids.
Keywords: Juvenile dermatomyositis; MRI; arrhythmia; cardiac; heart block; immunosuppression; myopathy.