Inflammatory disease of the aorta: Patterns and classification of giant cell aortitis, Takayasu arteritis, and nonsyndromic aortitis

Svensson, Lars; Arafat, Amr A.; Roselli, Eric E.; Idrees, Jahanzaib; Clifford, Alison; Tan, Carmela D.; Hoffman, Gary S.; Eng, Charis; Langford, Carol A.; Rodríguez, E. René; Gornik, Heather L.; Blackstone, Eugene H.; Sabik, Joseph F.; Lytle, Bruce W.

doi:10.1016/j.jtcvs.2014.08.003

Cited by 54 publications

(57 citation statements)

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“…[10][11][12]17,18 Most cases of aortitis in our series were idiopathic, also consistent with the existing literature. 3,11,12 The mean age of our patients with idiopathic aortitis was 66 years, within the reported range of 63-71 years in studies from 2000-2014. [10][11][12]14 Our population had high prevalence rates of smoking, hypertension and other cardiovascular risk factors that are typically associated with noninflammatory aortic aneurysms; notably, a case-control study that compared features of idiopathic aortitis with noninflammatory aortic aneurysms identified current smoking as well as female sex as independent risk factors for idiopathic aortitis.…”

Section: Discussionsupporting

confidence: 79%

“…1 It is known to be associated with a variety of systemic vasculitides (most commonly giant cell arteritis 2,3 and Takayasu's arteritis 3,4 ), other systemic inflammatory diseases such as rheumatoid arthritis, 5 systemic lupus erythematosus, 6 ankylosing spondylitis 7 and infections (especially bacterial infections, syphilis 8 and tuberculosis 9 ). In a substantial fraction of cases, aortitis is diagnosed in patients with no history or clinical symptoms of a systemic disease.…”

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confidence: 99%

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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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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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Section: Discussionsupporting

confidence: 79%

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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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“…Therefore, it is no surprise that subsequent surgeries were most commonly undertaken to repair the distal aorta. Further details of the surgical outcomes of these patients have been described in a separate study .…”

Section: Discussionmentioning

confidence: 99%

Outcomes Among 196 Patients With Noninfectious Proximal Aortitis

Clifford

Arafat

Idrees

et al. 2019

Arthritis & Rheumatology

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Objective. Noninfectious aortitis may occur in the context of a recognized systemic disease or as a topographically limited lesion without systemic features, which is called clinically isolated aortitis (CIA). This study was undertaken to better define and stress the limitations of this diagnostic category in a large population of patients in a single center dedicated to aortic diseases and to suggest recommendations for care.Methods. Records of patients undergoing thoracic aortic surgery (1996)(1997)(1998)(1999)(2000)(2001)(2002)(2003)(2004)(2005)(2006)(2007)(2008)(2009)(2010)(2011)(2012) at the Cleveland Clinic were reviewed to identify 196 patients with histopathologically proven aortitis. Clinical diagnoses (giant cell arteritis [GCA], Takayasu arteritis [TAK], CIA, or Other) were determined at the time of surgery. Clinical features, laboratory findings, and imaging results were recorded throughout the follow-up period. At least 6 months of follow-up data were available for 73 CIA patients.Results. The mean age of the patients at time of surgery was 65.6 years (range 15-88 years); 67% of patients were female, and 90.3% were white. At the time of surgery, 129 patients (65.8%) met criteria for CIA, 42 (21.4%) for GCA, 14 (7.1%) for TAK, and 11 (5.6%) met criteria for other systemic inflammatory diseases. During a mean followup period of 56.2 months, 19% of CIA patients developed new symptoms, 45% developed new radiographic vascular lesions, 40% underwent additional vascular surgery, and 12% died (n = 9). Eleven of 73 patients (15%) initially classified as having CIA developed features of a systemic disease, most often GCA.Conclusion. The majority of patients (66%) with histopathologically proven aortitis have CIA at the time of surgery. CIA patients infrequently report new symptoms over time, but new vascular lesions requiring surgery commonly occur. Serial follow-up including large vessel imaging is strongly advised for all aortitis patients.

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“…In the latest review discussing the management strategies of 156 histologically proven cases of aortitis, type 3 disease i.e. thoraco-abdominal aortic disease was treated by bypassing and stenting of greater vessels, before carrying out thoracoabdominal aortic repairs to the greater vessels or concurrent prosthetic material bypasses to visceral arteries or left subclavian bypass (Svensson et al, 2015). In our case, we deployed aortic stent graft intentionally covering left subclavian artery after confirming the patent communication of the right and left vertebral arteries at the basilar confluence.…”

Section: Resultsmentioning

confidence: 99%

Thoracic endovascular repair of a rare case of leaking aortic arch intramural hematoma secondary to Giant cell arteritis

et al. 2019

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Background: Traditionally thoracic aortic aneurysms (TAA) secondary to Giant Cell Arteritis (GCA) were treated with resection and open repair. However no prior studies have reported an aortic intramural hematoma (IMH) as a presentation of GCA or outcome of thoracic endovascular aortic repair (TEVAR) in TAA or IMH secondary to GCA. Case presentation: A 59 year old female, nonsmoker, non-hypertensive, non-diabetic with a known history of GCA, temporal arteritis on prednisone presented with shortness of breath & chest pain. Chest CT revealed aortic arch IMH and large left hemothorax. CTA confirmed distal aortic arch focal dilation, a focal intimal irregularity in the distal aortic arch and extensive IMH without any active extravasation or signs of aortitis. Patient underwent an urgent TEVAR without oversizing the aortic landing zones. Post TEVAR aortogram showed exclusion of the site of IMH origin and dilated aortic arch segment by the stent and absence of active extravasation. One month post-TEVAR CTA showed patent stent graft with resolution of IMH and hemothorax. One year after TEVAR, patient remained asymptomatic. Conclusion: GCA can present as an IMH secondary to underlying chronic vasculitis. When endovascular repair is considered, great care should be taken not to grossly oversize aortic landing zones.

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Inflammatory disease of the aorta: Patterns and classification of giant cell aortitis, Takayasu arteritis, and nonsyndromic aortitis

Abstract: Aortitis continues to be a conundrum; however, good results are achievable with surgery. Intervention should be based on a clearer understanding of the histologic pattern and extent of disease, which helps in subsequent targeted disease management.

Cited by 54 publications

References 30 publications

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

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

Outcomes Among 196 Patients With Noninfectious Proximal Aortitis

Thoracic endovascular repair of a rare case of leaking aortic arch intramural hematoma secondary to Giant cell arteritis

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