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非感染性升主动脉炎的一种不寻常表现:一例无症状杂音的病例报告

An unusual presentation of non-infectious ascending aortitis: a case report of an asymptomatic murmur.

作者信息

Calhoun Avery E, Bilen Ozlem

机构信息

Department of Internal Medicine, Emory University, 100 Woodruff Circle, Atlanta, GA 30322, USA.

Division of Cardiology, Department of Internal Medicine, Emory University, Atlanta, GA, USA.

出版信息

Eur Heart J Case Rep. 2021 Oct 23;5(12):ytab427. doi: 10.1093/ehjcr/ytab427. eCollection 2021 Dec.

DOI:10.1093/ehjcr/ytab427
PMID:34993398
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC8728718/
Abstract

BACKGROUND

Aortitis refers to pathologic inflammation of the aortic wall and is broadly categorized into inflammatory (or non-infectious) and infectious aortitis. While rare, isolated non-infectious ascending aortitis (I-NIAA) is a clinical entity that is becoming increasingly recognized but remains poorly understood.

CASE SUMMARY

A 72-year-old man presented with an asymptomatic murmur and was found to have severe aortic insufficiency second to a large ascending aortic aneurysm. He underwent surgical repair and pathology revealed isolated non-infectious ascending aortitis. Following successful surgical repair, he developed joint pains which were successfully treated with glucocorticoids.

DISCUSSION

Isolated non-infectious aortitis is a rare entity that warrants further investigation. This case highlights the importance of sending surgical specimens for histopathologic evaluation even when a systemic process is not evident at the time of surgical repair. The development of systemic symptoms following surgical repair in this patient emphasizes the importance of thorough rheumatologic evaluation in patients found to have I-NIAA. Isolated non-infectious ascending aortitis remains poorly understood, and further study is needed to evaluate both its existence as a distinct clinical entity and the role of immunosuppressive therapy.

摘要

背景

主动脉炎是指主动脉壁的病理性炎症,大致分为炎症性(或非感染性)主动脉炎和感染性主动脉炎。孤立性非感染性升主动脉炎(I-NIAA)虽罕见,但作为一种临床实体正日益受到认可,不过人们对其仍知之甚少。

病例摘要

一名72岁男性因无症状杂音就诊,检查发现因巨大升主动脉瘤导致严重主动脉瓣关闭不全。他接受了手术修复,病理检查显示为孤立性非感染性升主动脉炎。手术修复成功后,他出现关节疼痛,使用糖皮质激素治疗成功。

讨论

孤立性非感染性主动脉炎是一种罕见的疾病,值得进一步研究。该病例强调了即使在手术修复时未发现全身性病变,也应将手术标本送检进行组织病理学评估的重要性。该患者术后出现全身性症状,这凸显了对确诊为I-NIAA的患者进行全面风湿学评估的重要性。孤立性非感染性升主动脉炎仍未被充分了解,需要进一步研究以评估其作为一种独特临床实体的存在情况以及免疫抑制治疗的作用。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4843/8728718/60c71b1b466f/ytab427f7.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4843/8728718/37512d04f098/ytab427f1.jpg
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https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4843/8728718/574f2c39317c/ytab427f4.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4843/8728718/926bf6fdf154/ytab427f5.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4843/8728718/93a229656393/ytab427f6.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4843/8728718/60c71b1b466f/ytab427f7.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4843/8728718/37512d04f098/ytab427f1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4843/8728718/c74e9a6523d5/ytab427f2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4843/8728718/1decc797e2ca/ytab427f3.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4843/8728718/574f2c39317c/ytab427f4.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4843/8728718/926bf6fdf154/ytab427f5.jpg
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https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4843/8728718/60c71b1b466f/ytab427f7.jpg

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本文引用的文献

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Ascending Aortic Aneurysm Secondary to Isolated Noninfectious Ascending Aortitis.孤立性非感染性升主动脉炎致升主动脉瘤。
J Clin Rheumatol. 2019 Jun;25(4):186-194. doi: 10.1097/RHU.0000000000000948.
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Arthritis Rheum. 2000 Apr;43(4):901-7. doi: 10.1002/1529-0131(200004)43:4<901::AID-ANR23>3.0.CO;2-U.