How IgG + Complement = Coronary Inflammation + Aneurysm in Lupus

July 3, 2014

Another case report describes coronary aneurysms in a lupus patient. These were discovered serendipitously, and she recovered after aggressive treatment.

Famularo G, Vallone A, Compagnucci M, et al.Tiny bubbles: coronary aneurysms and lupus. Am J Med (2014) Online: June 11, . doi: http://dx.doi.org/10.1016/j.amjmed.2014.06.005

This makes the twentieth case report to describe coronary aneurysms in patients with systemic lupus erythematosus (SLE).

Almost all aneurysms were clinically significant with stenosis, thrombosis, distal embolism, dissection or rupture occurring at coronary artery sites, causing luminal compromise and downstream ischema. Presentations included myocardial infarction, intermediate coronary syndromes, acute ventricular dysfunction, malignant ventricular arrhythmias, and sudden death.

The mechanism is thought to be the deposition of immune globulin and complement, which causes coronary inflammation, which leads to necrosis and scars the tunica media, weakens the coronary wall, and forms aneurysms.

In this case, a 32-year-old woman with SLE was referred for treatment of New York Heart Association class III heart failure. She had diffuse proliferative lupus nephritis (class IV), chronic renal failure, hypertension, chronic anemia, and lung tuberculosis.

Coronary aneurysms were detected serendipitously in a work-up for possible valve replacement. Chest x-ray showed cardiomegaly and pulmonary congestion, and echocardiography showed severe mitral regurgitation. A computed tomography coronary angiogram showed many fusiform and saccular aneurysms, 3-10 mm, in all coronary territories. Several aneurysms were partially or completely calcified with internal thrombosis. The left main coronary artery was spared.

There are no formal guidelines for managing patients with SLE and coronary aneurysms. This patient recovered fully after aggressive treatment with fluid restriction, diuretics, and transfusion of packed red cells.