An unusual presentation of chest pain

Cardiac disease is common among patients with Systemic Lupus Erythematosus (SLE). Pericardial, myocardial, valvular, and coronary artery involvement have been extensively reported. The three major coronary abnormalities associated with myocardial injury in SLE are premature atherosclerosis, coronary arteritis and, less frequently, coronary aneurysms. A 26-year-old black male patient with a 5-year history of anti-phospholipid syndrome sustained a lateral wall myocardial infarct associated to angiographic evidences of multiple, diffuse, saccular coronary aneurysms without evidence of atherosclerotic occlusive disease in all three coronary arteries. Serologic studies were consistent with active SLE. Lupus-associated nephritis was also present. Radiographic studies showed no evidence of brain or thoracic aneurysms. A transthoracic echocardiogram showed an estimated ejection fraction of 35% and a moderate pericardial effusion. High dose i.v. steroids were started along with systemic anticoagulation. The patient had an uneventful clinical course and was discharged by the fourteenth day on high doses of oral steroids (60 mg daily), statins, oral anticoagulation (warfarin 5 mg), antihypertensives and aspirin. The patient was lost to follow-up and developed steroid-induced hyperglycemia, 20 pound weight gain and warfarin intoxication. A three-month follow up coronary angiography showed complete resolution of the aneurysms and serologic studies showed no active autoimmune disorder at that time. Coronary artery aneurysms have previously been reported in 15 cases of patients with SLE. An extensive Medline search of the literature revealed no previous reports of diffuse saccular coronary aneurysms involving all three coronary arteries associated with active SLE. This case highlights the unusual presentation of acute SLE in a young patient complicated by multiple aneurysms and acute myocardial infarction. In this case, we believe that a direct causal association exists between acute SLE-associated aneurysms and myocardial ischemic injury. Early recognition and prompt treatment with large doses of corticosteroids may decrease the mortality and morbidity associated with this condition.