Open surgical treatment for aortic arch and coronary artery aneurysms associated with immunoglobulin G4-related disease: a case report

Abstract

BackgroundImmunoglobulin G4-related disease (IgG4RD) is a systemic autoimmune disease characterized by a dense lymphoplasmacytic infiltrate containing IgG4-positive plasma cells that can cause aneurysms in various organs and tissues. There have been many reports of IgG4RD aneurysms; however, the surgical procedures adopted remain controversial. Herein, we report a case in which surgical treatment for IgG4-related aneurysms was successful.Case presentationAn 83-year-old woman with IgG4-related disease developed multiple aneurysms, including thoracic aortic aneurysm (TAA), right coronary artery aneurysm (CAA), abdominal aortic aneurysm, and common iliac artery aneurysm. Enhanced computed tomography (CT) revealed progressive enlargement of the TAA, while positron emission tomography-CT revealed intense 18F-fluorodeoxyglucose uptake around the TAA and CAA, suggesting the presence of adhesive tissue caused by adventitial inflammation around the aneurysms. Open aortic arch replacement with frozen elephant trunk procedure and coronary artery bypass grafting with ligation of the coronary artery were performed to treat the TAA and CAA. No surgical complications occurred. The postoperative CT scan showed no endoleak around the open stent graft and the coronary artery was patent. The patient is being followed up on an outpatient basis after discontinuing steroid therapy.ConclusionsWe report the successful surgical treatment of IgG4-related multiple aneurysms. Our findings reveal that the main site of inflammation in aneurysms of IgG4RD is the adventitia and the frozen elephant trunk procedure is useful for avoiding complications.