IgG4 aortitis of the ascending thoracic aorta: A case report and literature review

Abstract

IgG4 aortitis is a recently recognized entity that can have clinical and imaging features that mimic acute aortic syndrome. Therefore, it is imperative for radiologists to be aware of how to potentially differentiate the two. Although this entity has been previously described via case reports and meta-analysis in the context of inflammatory abdominal aortic aneurysm, very few cases of ascending aortic involvement have been reported. In this case report, we present a case of a 60-year-old female transferred from another facility for an initial diagnosis of intramural hematoma of the ascending aorta and later found to have IgG4 aortitis post aortic root repair. This is a histologically confirmed case of multi-segmented IgG4 aortitis with rare involvement of both ascending and infra-renal aorta. We will briefly discuss the pathophysiology of IgG4 aortitis, along with review of literature.