IgG4-Aortopathy: An Underappreciated Cause of Non-Infectious Thoracic Aortitis

Aguirre, Victor; Connolly, Catherine; Stuklis, Robert

doi:10.1016/j.hlc.2017.03.156

Cited by 7 publications

(6 citation statements)

References 12 publications

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“…6A) [8,9]. Histologically there is lymphoplasmacytic infiltration with storiform fibrosis as a dominant component, obliterative phlebitis, IgG4/IgG positive plasma cell ratio ≥ 40% and IgG4 positive plasma cell count >10/hpf (high power field) as seen on immunohistochemistry [1][2][3][4][5][6][7][8][9][10].…”

Section: Clinical and Imaging Findingsmentioning

confidence: 98%

“…The 'outside-in' theory suggests that the activated T-helper cells attract IgG4-positive B lymphocytes to the adventitia, which later differentiate into plasma cells [5]. The plasma cell infiltration of the adventitia can result in destruction of aortic wall elasticity and remodeling of the periadventitia via aggregation of cytokines and growth factors, eventually leading to a weakened wall that can predispose to aneurysm [4][5][6]. Risk of aneurysm increases when the inflammation extends to involve the media and lamellar elastic fibers [6,7].…”

Section: Clinical and Imaging Findingsmentioning

confidence: 99%

“…Based on our case, radiological findings such as aortic wall thickening with fat stranding and multifocal involvement may be helpful as a diagnostic feature. Aguirre and colleagues (2015) suggested involvement of other organs, elevated inflammatory markers and PET/CT may be helpful in diagnosis [6].…”

Section: Clinical and Imaging Findingsmentioning

confidence: 99%

“…Surgical versus medical management of IgG4 aortitis currently remains controversial due to lack of randomized clinical trials [10]. Several studies have stated that medical management with steroid therapy can improve aortitis both clinically and radiographically [1][2][3][4]6,10]. However, risk of persistent luminal dilatation and aneurysmal rupture remains high in a patient undergoing high-dose glucocorticoid therapy alone [2].…”

Section: Treatment and Prognosismentioning

confidence: 99%

“…However, risk of persistent luminal dilatation and aneurysmal rupture remains high in a patient undergoing high-dose glucocorticoid therapy alone [2]. Other proposed treatments include immunosuppressive therapy with methotrexate and B-cell depletion therapy with Rituximab [2,6,10]. Surgical management with aortic graft repair has shown improved postoperative course.…”

Section: Treatment and Prognosismentioning

confidence: 99%

See 4 more Smart Citations

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

Shilagani¹,

Lansman²,

Gilet³

et al. 2021

Radiology Case

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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.

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