IgG4-related disease with coronary arteritis.

A 53-year-old male patient with chronic renal failure and biopsy-proven Immunoglobulin G4 (IgG4) renal involvement presented with complains of chest pain and syncope. An ECG showed lateral ST segment depressions concerning for myocardial ischemia. The patient underwent coronary catheterization, which revealed multifocal aneurysms and severe stenosis. A cardiac computerized tomographic angiogram was performed to further characterize aneurysms, which showed aneurysms involving left main, proximal left anterior descending artery, and proximal right coronary artery (Figures 1 and 2). Circumferential mural thickening with associated enhancement of the wall of the coronary arteries, particularly at the site of the stenoses, was concerning for vasculitis. A small pulmonary artery aneurysm was seen in the right upper lobe (Figure 3). Abdominal computerized tomography demonstrated abdominal aortic aneurysm as well as aneurysmal dilatation of dissected superior mesenteric artery (Figure 4). The patient underwent coronary artery bypass grafting surgery with the saphenous vein graft to the mid left anterior descending artery, ramus intermedius, and posterior descending artery. Intraoperative biopsies of the internal mammary artery, pericardium, and aortic adventitia were positive for lymphoplasmacytic infiltrate and IgG4 deposits.