Oddly Shaped Lumpy Thoracic Aortic Aneurysm Associated with IgG4 Related Disease: Report of a Case

• Published in: Japanese Journal of Vascular Surgery Vol. 31; no. 2; pp. 61 - 65
• Main Authors: Shimauchi, Kota, Hayashi, Nagi, Yoshikai, Masaru, Nishida, Naoyo, Sato, Hisashi
• Format: Journal Article
• Language: Japanese
• Published: JAPANESE SOCIETY FOR VASCULAR SURGERY 19.04.2022; 特定非営利活動法人 日本血管外科学会
• Subjects: IgG4 related disease; IgG4関連疾患; inflammatory aneurysm; thoracic aortic aneurysm; 炎症性大動脈瘤; 胸部大動脈瘤
• ISSN: 0918-6778; 1881-767X
• DOI: 10.11401/jsvs.22-00009

IgG4 related disease (IgG4-RD) is clinically characterized by elevated serum levels of IgG4, dense lymphoplasmacytic infiltration of IgG4 positive plasma cells, and fibrosis of the affected organs. Regarding IgG4-RD related cardiovascular lesions, while reports have predominantly focused on cases involving the abdominal aorta or the iliac artery, the literature on thoracic aortic aneurysms is limited. We herein present a case of an oddly shaped, lumpy thoracic aortic aneurysm associated with IgG4-RD. A 69-year-old male presented to our facility with pyoderma on his hip. Preoperative contrast enhanced computed tomography (CT) showed an oddly shaped aneurysm extending from the ascending aorta to the aortic arch, and subsequent blood tests showed elevated CRP and IgG4 levels (CRP 8.1 mg/dL, IgG4 207 mg/dL). Since the aneurysm was determined to be at a high risk of rupture, we performed a prosthetic graft replacement of the aorta from the ascending aorta to the aortic arch after resecting the aneurysmal aortic wall. On distal anastomosis of the graft, an open stent graft was deployed into the descending aorta to reinforce the anastomosis. According to the results of the pathological study of the resected aortic wall, which showed an abundant infiltration of IgG-positive plasma cells, the CT findings, and elevated serum IgG4 levels, we confirmed the definite diagnosis of IgG4-RD. In considering the increased risk that corticosteroid therapy brings to both the aortic wall’s fragility and the associated postoperative infectious complications, corticosteroid therapy was not introduced. We conclude that careful long-term follow-up should be mandatory, especially paying attention to exacerbation of the inflammation or the development of a pseudoaneurysm at the anastomotic sites.