Summary

A 36-year-old man with systemic lupus erythematosus (SLE) presented with chest pain, infero-lateral ST segment elevation on ECG and elevation of cardiac biomarkers and inflammatory markers. Coronary angiography ruled out obstructive coronary artery disease (CAD) but echocardiography showed impairment of regional and global left ventricular (LV) function. He was treated for SLE myocarditis but institution of aggressive immunosuppressant therapy only partially improved his condition, which followed a relapsing and remitting course in subsequent months, with progressive impairment of LV function. Cardiac MRI showed active inflammation and extensive transmural scarring. Endomyocardial biopsy (EMB) demonstrated patchy myocardial fibrosis and low-grade myocarditis and PCR assays excluded viral causes. The lack of response to immunosuppression and the detection of the sign of En coup de Sabre were suggestive of scleroderma as the underlying cause of the myocarditis.