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IgG4-Related Aortitis Masking as Intramural Hematoma on Imaging

Year: 2025

Type: article

Abstract: BACKGROUND: IgG4-related aortitis is a rare fibroinflammatory disease characterized by thoracic or abdominal aortic wall thickening and dilation. CASE SUMMARY: A 24-year-old man presented after blunt trauma and was found to have intramural hematoma in the ascending aorta on chest computed tomography. Given the high-impact injury, aortic injury could not be excluded. Therefore, he underwent a hemiarch replacement; intraoperative findings and histopathology were concerning for IgG4-related aortitis. DISCUSSION: IgG4 aortitis of the ascending aorta is rare, with only a few case reports in the literature. On computed tomography, IgG4 aortitis can mimic acute aortic syndrome, which poses a challenge regarding appropriate management. TAKE-HOME MESSAGES: IgG4 aortitis can mimic acute aortic syndrome findings on imaging. This poses a clinical dilemma in the management of these patients, and surgical intervention should only be offered in the appropriate clinical scenario.

Source: JACC Case Reports

DOI: 10.1016/j.jaccas.2025.106128

Authors: Armaan F. Akbar, Atharv Oak, Uzma Rahman, Bryon A. Tompkins, Ahmet Kilic

Institutions: Columbia University, Johns Hopkins University, Johns Hopkins Hospital, Baptist Hospital of Miami

Language: English