Multiple Coronary Artery Aneurysms and Thoracic Aortitis Associated with IgG4-related Disease

Abstract

A 60-year-old man was admitted due to the onset of right coronary artery (RCA) aneurysms. Coronary angiography showed two RCA aneurysms and focal stenosis with limitations in the blood flow. Balloon angioplasty was performed. However, the follow-up coronary angiography showed restenosis, an enlarged proximal aneurysm and a newly formed aneurysm. The serum immunoglobulin G4 level was elevated to 1,350 mg/dL and fluorodeoxyglucose positron emission tomography showed increased uptake in the ascending aorta, so the patient was diagnosed with immunoglobulin G4-related vascular disease. The prevention of further enlargement of the aneurysms and an improvement in the RCA flow were achieved with steroid therapy. Steroid therapy may therefore be effective for immunoglobulin G4-related vascular disease.

Early Detection and Intervention of Coronary Artery Involvement in Immunoglobulin G4-related Disease

“…Cardiac surgery was necessary in three of the eight cases (6, 9-15, Table 2 ). All patients who had coronary stenosis or aneurysms were resistant to corticosteroid ( 6 , 9 - 15 ). In contrast, the patients without coronary stenosis or aneurysms responded to corticosteroid (18, the present case).…”

“…The pathological characteristics include diffuse lymphoplasmacytic infiltration, fibrosis, obliterative phlebitis, and eosinophilic infiltration in the involved organs ( 7 , 8 ). Many reports indicate that coronary artery involvement is related to a poor prognosis ( 6 , 9 - 15 ).…”

Clinical Utility of Diffusion-weighted Whole-body Magnetic Resonance Imaging with Background Body Signal Suppression for Assessing and Monitoring IgG4-related Disease

“…Recently, several cases have been reported that involved IgG4-related coronary disease. Some patients had pseudotumors surrounding coronary arteries (10)(11)(12)(13)(14), and others had aneurysms of coronary arteries (15)(16)(17)(18)(19)(20). The present patient had pseudotumors surrounding two coronary arteries with stenosis.…”

A Case of Aortic Stenosis with Serum IgG4 Elevation, and IgG4-Positive Plasmacytic Infiltration in the Aortic Valve, Epicardium, and Aortic Adventitia

“…It may then be questioned whether the current patient can be diagnosed with IgG4-related disease that affected multiple cardiovascular tissues. In several previous reports, IgG4-related cardiovascular disease was diagnosed when either IgG4-positive plasmacytic infiltration or elevated serum IgG4 was present alongside the suspected radiologic findings, 5,14) or when IgG4-related disease-like radiologic findings were observed in the heart or vessels in association with IgG4-related disease diagnosed elsewhere in non-cardiovascular organs. 15,16) On the other hand, cur- rently, unlike several other organs, 17) cardiac valve-specific diagnostic criteria for the diagnosis of IgG4-related disease do not exist in Japan.…”