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

• Type of Material: Article
• Authors: Avery E Calhoun, Emory University; Ozlem Bilen, Emory University
• Language: English
• Date: 2021-12-01
• Publisher: Oxford University Press
• Publication Version: Final Published Version
• Copyright Statement: © The Author(s) 2021. Published by Oxford University Press on behalf of the European Society of Cardiology.
• License: Creative Commons Attribution-NonCommercial 4.0 International
• Final Published Version (URL): http://dx.doi.org/10.1093/ehjcr/ytab427
• Title of Journal or Parent Work: European Heart Journal - Case Reports
• Volume: 5
• Issue: 12
• Start Page: ytab427
• End Page: ytab427
• Grant/Funding Information: None declared.
• Keywords: Case report; Valve replacement; Echocardiography; Autoimmune; Aorta; Three-dimensional imaging
• Research Categories: Health Sciences, Medicine and Surgery

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.