IgG4-related aortitis mimicking intramural hematoma

Güler, Gamze Babur; Cantürk, Emir; Güler, Ekrem; Oran, Gülbin; Demir, Gültekin Günhan; Akçevin, Atıf; Barutçu, İrfan

doi:10.14744/anatoljcardiol.2016.7185

Cited by 5 publications

(9 citation statements)

References 5 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: 99%

“…On imaging, CTA demonstrates soft tissue thickening of the outer aortic wall, which can occasionally be mistaken for acute aortic syndrome such as intramural hematoma due to its overlapping appearance [1,4,10].…”

Section: Clinical and Imaging Findingsmentioning

confidence: 99%

“…The presentation and involvement of the ascending aorta can result in a diagnostic dilemma especially in the setting of nonspecific patient presentation. Differential diagnosis of circumferential aortic wall thickening includes intramural hematoma, non-infectious aortitis such as vasculitides, and infectious aortitis [1,10,11]. Intramural hematoma can present as circumferential or crescentic wall thickening, which may or may not be hyperdense on noncontrast CT depending on acuity [12].…”

Section: Differential Diagnosismentioning

confidence: 99%

Section: Treatment and Prognosismentioning

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%

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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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Section: Clinical and Imaging Findingsmentioning

confidence: 99%

Section: Clinical and Imaging Findingsmentioning

confidence: 99%

Section: Differential Diagnosismentioning

confidence: 99%

Section: Treatment and Prognosismentioning

confidence: 99%

Section: Treatment and Prognosismentioning

confidence: 99%

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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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Immunoglobulin G4-related thoracic aortitis

Yuan

2019

Z Rheumatol

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Diagnostic and treatment workup for IgG4-related disease

Abraham

Khosroshahi

2017

Expert Review of Clinical Immunology

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Introduction IgG4-Related Disease is a newly recognized condition which is increasingly diagnosed by practitioners due to improvement in clinical awareness. Men and women have been found to be affected by this disease in various organs, more commonly with involvement of the salivary and lacrimal glands as well as pancreas and liver. Areas Covered The diagnosis and management of this condition remain challenging as biomarkers and therapies are being investigated. Hallmark features on histology are still the gold standard for confirmation of diagnosis, whereas serum IgG4 level has been shown to be neither necessary nor sufficient for the diagnosis. Glucocorticoids remain the most effective initial management for this condition while there are limited clinical trials on the effectiveness of maintenance therapy. Expert Commentary This review serves as an update on approaches for diagnosis and management of IgG4-RD. Most of the known data in this field comes from retrospective cohort studies and expert consensus guidelines but new ongoing prospective studies, clinical trials and better understanding of the pathogenesis of this condition are promising.

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IgG4-related aortitis mimicking intramural hematoma

Cited by 5 publications

References 5 publications

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

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

Immunoglobulin G4-related thoracic aortitis

Diagnostic and treatment workup for IgG4-related disease

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