Ishida Mitsuaki, Hotta Machiko, Kushima Ryoji, Asai Tohru, Okabe Hidetoshi
Department of Clinical Laboratory Medicine, Shiga University of Medical Science, Shiga, Japan.
Pathol Int. 2009 Apr;59(4):269-73. doi: 10.1111/j.1440-1827.2009.02363.x.
IgG4-related sclerosing disease can occur in the cardiovascular system and some inflammatory abdominal aortic aneurysms have been shown to belong to IgG4-related sclerosing disease. Herein is reported a case of IgG4-related inflammatory aortic aneurysm of the aortic arch. A 71-year-old Japanese man was found to have an aneurysm of the aortic arch with maximum dimension of 5.5 cm. The surgically resected aneurysm wall had conspicuous fibrosclerotic changes, dense lymphoplasmacytic infiltration and occasional obliterative phlebitis in the adventitia; the thickness of the adventitia was 6.5 mm. Immunohistochemistry indicated numerous IgG4-positive plasma cell infiltrates; 84% of the IgG-bearing cells were IgG4 positive. The diagnosis of IgG4-related inflammatory aortic aneurysm of the aortic arch was made. Although previously reported IgG4-related inflammatory aortic aneurysms were confined to the abdominal aorta, the present case report demonstrates that IgG4-related inflammatory aortic aneurysm can occur in the aortic arch, thereby extending the spectrum of IgG4-related periaortitis. Further studies are needed to clarify the spectrum of IgG4-related sclerosing disease in the cardiovascular system.
IgG4相关性硬化性疾病可发生于心血管系统，一些炎症性腹主动脉瘤已被证实属于IgG4相关性硬化性疾病。本文报道一例主动脉弓IgG4相关性炎性动脉瘤。一名71岁的日本男性被发现患有主动脉弓动脉瘤，最大直径为5.5厘米。手术切除的动脉瘤壁有明显的纤维硬化改变，外膜有密集的淋巴浆细胞浸润及偶见的闭塞性静脉炎；外膜厚度为6.5毫米。免疫组化显示有大量IgG4阳性浆细胞浸润；84%的IgG阳性细胞为IgG4阳性。诊断为主动脉弓IgG4相关性炎性动脉瘤。尽管先前报道的IgG4相关性炎性主动脉瘤局限于腹主动脉，但本病例报告表明IgG4相关性炎性主动脉瘤可发生于主动脉弓，从而扩展了IgG4相关性主动脉周炎的范围。需要进一步研究以阐明心血管系统中IgG4相关性硬化性疾病的范围。
