IgG4-aortitis among thoracic aortic aneurysms

Abstract

ObjectiveThe incidence of aortitis in patients with thoracic aortic diseases is not well established. The aim of this study was to analyse the frequency and clinical course of patients with aortitis in a surgical series.Methods320 consecutive patients with ascending aorta/aortic arch aneurysm or acute aortic syndrome who underwent surgery from 2012 to 2017 in a single tertiary referral hospital were retrospectively reviewed. Epidemiological data, clinical course and variables related to diagnosis, treatment and follow-up were collected from patients with histologically proven aortitis.ResultsFrom 320 examined aortic samples, 279 (87.2%) thoracic aneurysms and 41 acute aortic syndromes (12.8%), 9 (2.8%) were aortitis: 3 cases of Takayasu’s arteritis, 3 of IgG4-related aortitis, 2 of giant cell, and 1 classified as idiopathic. Median age at surgery was 53.4 (51–69.2) years and six cases were female. Seven patients presented with non-specific symptoms and the diagnosis was made at pathology. Surgery was elective in eight patients and emergent in one case of IgG4-related aortitis. 18F-fluorodeoxyglucose positron emission tomography/CT (18F-FDG PET/CT) was performed for disease extension study and as a monitoring technique during the follow-up of five patients, with just one case performed presurgically. All the patients with IgG4-related disease showed extrathoracic aortic involvement. There were no deaths, neither in-hospital nor during the 1.7 years of median follow-up.ConclusionsIn surgically treated thoracic aorta pathology, the frequency of aortitis is low; IgG4-related disease is among the most common aetiologies with a frequency similar to other types of aortitis, such as Takayasu’s and giant cell arteritis, and clinical manifestations are non-specific making presurgical diagnosis difficult. 18F-FDG PET/CT allows a better assessment of disease extension and therapeutic response. Surgery can be successfully performed and corticosteroid therapy ensures a good mid-term follow-up.

How to mimic a type A intramural hematoma: A case of IgG4‐related aortitis associated with cerebral hemorrhage

“…Sixty percent to eighty‐five percent of the patients affected by IgG4‐related vasculitis are males, usually older than 50 years, as in the case described 1,2 . In a series of 320 surgically treated thoracic aortic aneurysms, a low frequency of aortitis was found (2.8%), with IgG4‐related aortitis as one of the most common etiologies 3 …”

MDCT Imaging of Non-Traumatic Thoracic Aortic Emergencies and Its Impact on Diagnosis and Management—A Reappraisal

“…Notably, both aortitis and periaortitis may arise in the context of IgG4-related disease that is defined as idiopathic inflammatory and sclerosing lesions infiltrated by numerous IgG4-positive plasma cells with multi-organ effects, including the vascular system [ 130 , 131 ]. More common CTA findings are severe, often circumferential, aortic wall thickening with homogenous delayed-phase aortic mural enhancement and minor or major irregular extension of periaortic inflammatory tissue into adjacent adipose tissue.…”

“…More common CTA findings are severe, often circumferential, aortic wall thickening with homogenous delayed-phase aortic mural enhancement and minor or major irregular extension of periaortic inflammatory tissue into adjacent adipose tissue. Luminal changes (often dilation and more rarely stenosis) may also be present [ 131 ].…”

<p>Diagnosing Aortic Intramural Hematoma: Current Perspectives</p>

“…Differential diagnosis of AIH must include the aortitis. 61 Although the clinical picture is generally different and is helpful to guide the diagnosis, there are several cases reported in the literature of aortitis simulating an AIH. There are some clues that could be useful for radiological diagnosis.…”

“…An increased glucose uptake and subsequent hyper-enhanced corresponding to the thickened aortic wall is diagnostic of aortitis. 61 AIH could also be present in patients with a known medical history of vasculitis, which makes it even more difficult to diagnose. 64 The clinical picture is that D-dimer levels and inflammatory markers could be helpful.…”