Noninfectious Ascending Aortitis: Staying Ahead of the Curve

Merkel, Peter A.

doi:10.3899/jrheum.090920

Cited by 16 publications

(15 citation statements)

References 19 publications

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“…In a substantial fraction of cases, aortitis is diagnosed in patients with no history or clinical symptoms of a systemic disease. [10][11][12] Although some of these cases may be a sequela of a clinically unapparent or burned-out systemic condition causing few or no symptoms, 13 when no such condition is apparent, the term idiopathic aortitis is used.…”

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confidence: 99%

A case series of surgically diagnosed idiopathic aortitis in a Canadian centre: a retrospective study

et al. 2017

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Background: Idiopathic aortitis became recognized relatively recently, and the body of knowledge concerning this condition is scarce. We aimed to determine the frequency of idiopathic aortitis in aortic specimens, the clinical, laboratory and radiologic characteristics at diagnosis and during follow-up, and the approach to investigation, treatment and monitoring taken by the treating physicians. Methods:We identified cases of aortitis diagnosed on pathological specimens of the aorta between Jan. 1, 2003, and July 31, 2013, at The Ottawa Hospital by reviewing the hospital's pathology database. Charts of identified patients were reviewed, and data on patient demographic characteristics, clinical features, laboratory and imaging tests, treatment and outcomes were analyzed.Results: A total of 684 aortic specimens were analyzed during the study period; 47 cases of aortitis were identified, 32 of which were idiopathic. Twenty-one patients (66%) had complete imaging of branch vessels at baseline, 16 (76%) of whom had additional aortic or branch vessel lesions. Twelve patients (38%) received corticosteroids postoperatively. Over a mean follow-up period of 47.5 months, among the 12 patients (38%) who had complete imaging of branch vessels at least once, new aortic or branch lesions were diagnosed in 5 (42%); 3/32 patients (9%) required additional vascular surgery; and a new systemic condition was diagnosed in 2/32 (6%).Interpretation: Idiopathic aortitis is commonly discovered incidentally on examination of the pathological specimen following ascending aortic aneurysm repair. No guidelines exist for the investigation, treatment and follow-up of this condition, resulting in great variability of practice. Good-quality prospective studies are needed to address the many unanswered clinical questions regarding idiopathic aortitis and to allow formulation of more definitive recommendations. Abstract ResearchResearch CMAJ OPEN E484CMAJ OPEN, 5(2)

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A case series of surgically diagnosed idiopathic aortitis in a Canadian centre: a retrospective study

et al. 2017

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“…Systemic disorders known to be complicated by aortitis were identified in one-quarter of patients in this study [1], and in a quite wide range (5 to 49%) of previously reported patients [4-6]. Such discrepancies may be ascribed to the use of different criteria for disease classification as well as to diverse local ascertainment practices.…”

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confidence: 79%

Idiopathic aortitis: an underrecognized vasculitis

Pipitone

Salvarani

2011

Arthritis Res Ther

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Aortitis is a general term denoting inflammation of the aortic wall. Various infectious and non-infectious diseases can be complicated by aortitis; in addition, isolated idiopathic aortitis has also been described. In a 12-year nationwide Danish population-based study, the prevalence of aortitis among 1,210 resected thoracic aorta samples was 6.1%, with nearly three-quarters of cases being idiopathic. Identified risk factors for aortitis included advanced age, a history of connective tissue disease, diabetes mellitus, and heart valve pathology. As in virtually all pathological studies, this study has a bias toward reporting the most severe cases of aortitis requiring surgical repair.

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“…At least 20% of patients with GCA and 60e80% with TA develop aortitis (8). Many of these develop an aortic aneurysm as a consequence (8).…”

Section: Discussionmentioning

confidence: 99%

“…At least 20% of patients with GCA and 60e80% with TA develop aortitis (8). Many of these develop an aortic aneurysm as a consequence (8). Although there is a risk of spontaneous aortic rupture without aneurysmal dilatation secondary to vessel wall weakening and necrosis, this event is exceedingly rare, with only two previous cases reported in the literature (9,10).…”

Section: Discussionmentioning

confidence: 99%