Systemic Lupus Complicated by Myocardial Vasculitis

Abstract

A 20-year-old woman was admitted to the intensive care unit due to heart failure. Her past medical history included only systemic lupus erythematosus (SLE) with multiple complications. She had been suffering from asthenia, fever and abdominal pain, followed by dyspnea, global heart failure and a persistent fever. Electrocardiogram (ECG) showed sinus tachycardia and echocardiography showed dilated cardiomyopathy with significant systolic alteration and an estimated left ventricular ejection fraction (LVEF) of 40%. The level of troponin I was elevated. A total body scan excluded the presence of any deep abscesses. The treatment consisted of intravenous corticosteroids, cyclophosphamide and finally plasma exchange, which achieved clinical and echographic improvement. Mycophenolate mofetil and rituximab were later introduced. We herein report a rare case of multiple infarcts in an apparently healthy young woman. SLE is known to be a cardiovascular risk factor in this population; however, we report a rare case of multiple infarcts and vasculitis in a young woman suffering from severe LED, in spite of the use of immunosuppressive treatment. A cardiac MR scan confirmed the presence of left ventricular global hypokinesia (Picture A, arrows). Several areas of late gadolinium enhancement (LGE) were visible, indicating small and patchy subendocardial infarcts, without a relationship with coronary segmentation (Picture B, arrows). Furthermore, a diagnosis of myocarditis was excluded based on the lack of delayed enhancement in the subepicardial layers. Altogether, the clinical and MR scan findings were in suggested a diagnosis of myocardial vasculitis, supported by the fact that the coronary arteries were normal.