A 54-year-old man with remote large cell non-Hodgkin’s lymphoma in remission following R-CHOP and severe atopic dermatitis was transferred from another hospital with a non-ST elevation myocardial infarction. Over the preceding year, the patient had suffered recurrent admissions for acutely decompensated heart failure with a newly depressed left ventricular ejection fraction (LVEF) of 20% by echocardiography and rapidly progressive end-stage renal disease of unclear etiology requiring the initiation of hemodialysis. Prior workup had demonstrated an infrarenal abdominal aortic aneurysm and bilateral common iliac artery aneurysms with subsequent computed tomography (CT) additionally demonstrating a superior mesenteric artery aneurysm. The patient was taken for immediate coronary arteriography, which demonstrated giant aneurysms in the left main and right coronary arteries, as well as multivessel severe stenoses. CT coronary angiogram demonstrated significant circumferential wall thickening throughout the coronary vasculature. Given concern for IgG4-related disease (IgG4-RD), a renal biopsy was pursued that confirmed the diagnosis. 18F-fluorodeoxyglucose positron emission tomography-CT identified only mild aortic inflammation. The patient was treated with high-dose steroids and rituximab. The serological inflammatory markers improved, and he underwent coronary artery bypass grafting. Pericardial, aortic adventitial, left internal mammary artery, and saphenous vein biopsies showed cardiovascular involvement of IgG4-RD. The patient has been maintained on rituximab with normalization of his LVEF and no recurrence of chest pain over the past eighteen months. IgG4-RD is a fibroinflammatory systemic disease newly described in 2003 and only recently found to involve the cardiovascular system with several reports of peripheral aneurysmal disease. To our knowledge, the current case represents the first report of a patient successfully treated for biopsy-proven IgG4-RD associated with coronary artery disease and left ventricular systolic dysfunction. IgG4-RD may represent a novel mechanism underlying some forms of peripheral and coronary arterial disease and may offer new insights into vascular biology.
Blunted cyclic variation of heart rate predicts mortality risk in post-myocardial infarction, end-stage renal disease, and chronic heart failure patients
