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结节性多动脉炎合并主动脉夹层:滋养血管的坏死性血管炎。

Polyarteritis nodosa with aortic dissection: necrotizing vasculitis of the vasa vasorum.

作者信息

Iino T, Eguchi K, Sakai M, Nagataki S, Ishijima M, Toriyama K

机构信息

First Department of Internal Medicine, Nagasaki University School of Medicine, Japan.

出版信息

J Rheumatol. 1992 Oct;19(10):1632-6.

PMID:1361205
Abstract

A 59-year-old woman was admitted to our hospital with acute onset of chest pain. She had experienced high fever, weight loss, polyarthralgia, myalgia, polyneuropathy and hallucinations for 3 years before admission. The diagnosis of polyarteritis nodosa with hepatitis B surface antigenemia was made by muscle biopsy and serological examinations, and administration of prednisolone induced remission of all the manifestations. After developing the acute attack of severe chest pain, she died suddenly. At autopsy, a DeBakey type 1 aortic dissection was found and the immediate cause of death was found to be cardiac tamponade secondary to rupture of the aortic dissection. Microscopically, necrotizing inflammatory lesions were present in the medium sized vascular arteries throughout her body. Furthermore, necrotizing vasculitis was also found in the vasa vasorum of the adventitia and media of the thoracic aorta. The dissecting lesion was seen in the outer layer of the media. Our results suggest that spontaneous dissection of the aorta may be attributed to necrotizing vasculitis of the vasa vasorum.

摘要

一名59岁女性因突发胸痛入院。入院前3年,她出现高热、体重减轻、多关节痛、肌痛、多发性神经病及幻觉。经肌肉活检和血清学检查诊断为结节性多动脉炎合并乙肝表面抗原血症,给予泼尼松龙治疗后所有症状缓解。在发生严重胸痛急性发作后,她突然死亡。尸检发现为DeBakey Ⅰ型主动脉夹层,死亡直接原因是主动脉夹层破裂继发心脏压塞。显微镜下,全身中等大小血管动脉存在坏死性炎性病变。此外,在胸主动脉外膜和中膜的滋养血管中也发现坏死性血管炎。夹层病变见于中膜外层。我们的结果提示,主动脉自发夹层可能归因于滋养血管的坏死性血管炎。

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