Severe Coronary Vasculitis During a Systemic Lupus Erythematosus Flare Improved Angiographically With Immune-Suppressant Therapy

A 47-year-old woman with systemic lupus erythematosus with nephritis, maintained on prednisone, experienced transient chest pain radiating to her left arm and shoulder.She was hospitalized for a potassium level of 6.1 mEq/dL.She lost consciousness after receiving sodium polystyrene enemas.Cardiopulmonary resuscitation was performed briefly.Her troponin-I was 16 ng/mL, and echocardiography revealed inferior, posterior-lateral, and apical hypokinesia; normal chamber sizes; and moderate mitral regurgitation.Coronary angiography revealed multiple small saccular aneurysms scattered along the smaller epicardial arteries and occlusion in the distal obtuse marginal branch (Figure , A andB).Hemodynamics of the right side of the heart were normal.Left ventriculography demonstrated moderate mitral regurgitation and ventricular dilatation with anterior, apical, and inferior hypokinesis.She received intravenous methylprednisolone and cyclophosphamide.Fifteen months later, she had severe mitral regurgitation with worsening left ventricular function.Coronary angiography before mitral valve repair showed complete resolution of the coronary aneurysms and only mild diffuse luminal irregularities of the coronary arteries (Figure, C and D).Valve repair was uneventful, with subsequent improvement in left ventricular function and resolution of mitral regurgitation.