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. General Radiology:IgG4 aortitis of the ascending thoracic aorta: A case report and literature review Shilagani et al.

IgG4 aortitis in a patient with coronary artery disease

“…Due to the heterogeneity of its manifestations, it is necessary to integrate clinical, laboratory, instrumental and histopathological data for a definitive identification. The four main pathological signs of IgG4 disease are dense polyclonal lymphoplasmacytic infiltrates with high percentages of IgG4+ plasma cells, storiform fibrosis, obliterative phlebitis and tissue eosinophilia [ 1 , 2 ].…”

“…T helper cells attract into the adventitia IgG4-positive B lymphocytes which subsequently differentiate into plasma cells. Infiltration of plasma cells into the adventitia leads to destruction of the elasticity of the aortic wall and periadventitial remodeling, which could lead to wall weakness and predispose to aneurysm and dissection [ 1 , 3 ]. Aortic dissection associated with IgG4-RD is not a rare finding, but it may often be underdiagnosed, considering the limited available data.…”

“…The differential diagnosis is important and often complicated, especially in patients with non-specific signs. The differential diagnosis of circumferential aortic wall thickening includes intramural hematoma, non-infectious aortitis and infectious aortitis [ 1 ]. Treatment of IgG4 aortitis remains controversial due to the lack of randomized clinical trials.…”