https://orcid.org/0000-0001-8644-1007
A 78-year-old man was presented with the productive cough to our hospital. A chest radiograph demonstrated the abnormal cardiac silhouettes, and the computed tomography (CT) revealed right and left circumflex coronary aneurysms with circumferential and periarterial soft-tissue enlargements (Panels A and B). The patient also showed mild swelling of bilateral submandibular gland with elevated serum immunoglobulin G4 (IgG4) level (1870 mg/dL; normal, 4.8–105), and its biopsy showed sclerosing sialadenitis infiltrated by various inflammatory cells including CD138-positive plasma cells with over 50% IgG4/IgG positivity (Panels C–F). We diagnosed the patient with IgG4-related disease (IgG4-RD) with coronary aneurysms. He was treated with 30 mg of prednisolone, improving the aneurysmal dilation of coronary arteries and serum IgG4 level. However, soft-tissue mass surrounding the coronary arteries was maintained and the coronary aneurysms were regained along with tapering glucocorticoid dose (Panels G–L). Although there was a gradual progression of chronic heart failure due to low cardiac output caused by direct mass compression on the left ventricle, he had hesitated to undergo surgical removal of the mass. A decade later, he is still alive under prednisolone therapy despite the cardiac mass enlargement.
IgG4-related disease is an insidious immune-mediated fibrotic disorder with multiple organ involvements. IgG4-related arterial involvement is characterized by circumferential wall thickening or aneurysmal dilatation with a predilection for large arteries, which is easily visualized and followed-up by imaging techniques such as CT angiography. The pathogenesis is attributed to an interplay between fibroblasts, M2 macrophage, and adaptive immune cells. The diagnostic delay of treatment might lead to permanent fibrotic change despite appropriate immunosuppression. The systemic work-up would contribute to prompt diagnosis in IgG4-RD.
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