Journal of Invasive Cardiology

Advances in medical therapy and a better understanding of systemic lupus erythematosus (SLE) have contributed to a dramatic improvement in the long-term survival of patients. However, despite the overall long-term improvement, coronary artery disease remains a major cause of morbidity and mortality1 (Table 1) with an incidence that is approximately nine-fold greater than would be expected for this population.2 Following active lupus, coronary artery disease is the second most common cause of hospitalization for SLE patients.3 Manzi et al. found that, when controlled for age and gender, women with SLE who are 35–44 years old have a 50-times higher risk of myocardial infarction (MI).4 Previous autopsy studies have observed that severe coronary artery disease is present in as many as 40% of patients with SLE compared with only 2% of age-matched controls at the time of death.5,6 Etiologies of myocardial damage in SLE patients include premature atherosclerotic disease,7 antiphospholipid antibody syndrome,8 coronary artery spasm,9 coronary artery vasculitis10 and restenosis after percutaneous revascularization procedures. The present case illustrates the importance and challenge of differentiating among these etiologies, especially since the therapies used are different in each situation. The following discussion will focus on the diagnosis and pathogenesis of coronary artery disease with an emphasis on premature atherosclerosis and coronary vasculitis in patients with SLE.
Case Report. A 21-year-old woman presented to the emergency room with a chief complaint of substernal chest pain. The pain was scaled a 9/10, was non-radiating and not associated with diaphoresis, dizziness, nausea or vomiting. The patient had a history of SLE, with a positive ANA and double-stranded DNA antibodies seven years ago. Clinical manifestations included a malar rash and joint swelling. Subsequently, the patient was only treated with hydroxychloroquine. As a child she had several hospitalizations for lupus flares for which she was treated with short courses of steroids. She had no history of pregnancy, deep venous thrombosis, pulmonary embolus or migraine headaches. She had undergone a four-vessel coronary artery bypass graft operation in June of 2000 with separate saphenous vein grafts to the left anterior descending (LAD), obtuse marginal (OM) 1 and 2, and distal right coronary arteries (RCA) 4 months prior to admission. At that time, a biopsy was taken of the internal mammary artery that reported a chronic mural inflammatory infiltrate composed of lymphocytes and occasional eosinophils (Figure 1). Her medications included enteric-coated aspirin 325 mg QD, metoprolol 25 mg BID, omeprazole 30 mg QD and hydroxychloroquine 200 mg QD. She reported allergies to sulfonamides, clarithromycin, metronidazole, nifedipine and ranitidine. The patient denied any family history of hypercholesterolemia or coronary artery disease. She reported a history of tobacco use of one pack per day for 2 years, but this was discontinued one month prior to admission. There was no history of drug or alcohol use.