Background/Purpose: This study is aimed at estimating the prevalence of inflammatory ascending aneurysms, describing clinical and histopathological findings, and assessing whether appropriate follow-up was arranged post discharge

Methods: Data from Enterprise Data Warehouse was retrieved for all cases of ascending aortic aneurysms from Jan 2009-Dec 2015 requiring surgery at the University Health Network for the following diagnostic codes: thoracic aortic aneurysm, ruptured (I71.1) or without mention of rupture (I71.2); aortic aneurysm of unspecified site, ruptured (I71.8) or without mention of rupture (I71.9). After eliminating duplicate retrievals, 743 had their ascending aorta resected. Of these, 730 had aneurysmal dilatation, defined as maximum diameter ≥40mm measured by pre-operative echo or cardiac CT/MRI. All cases with ascending non-infectious inflammatory aneurysms were identified and reviewed for clinical presentation, imaging, histopathological and management method.

Results: Among the 730 cases studied, 53 (7.3%) were of a non-atherosclerotic, non-infectious inflammatory pathogenesis. Mean age was 67 years (18-68), with 50.9% women. Asymptomatic presentation occurred in 52.9%, 7.8% had constitutional symptoms and 43.1% had symptoms that could be attributed to an aneurysm. Isolated aortitis with no other evidence of arterial involvement presented in 41 (77.3%), and 12 had underlying rheumatic disease: Rheumatoid Arthritis (4), Polymyalgia Rheumatica (3), Giant Cell Artertitis (3), Systemic Lupus Erythematosus (1) and Cogan’s Syndrome (1). Echocardiographic findings demonstrated a maximum mean aneurysm diameter of 54.7± 6.8mm, aortic insufficiency (AI) in 73.5%, stenosis (AS) in 9.4% and mixed AS & AI in 3.8%. A bicuspid aortic valve was found in 15.1%. Twenty-five (47.1%) had the aortic aneurysm distal to the ascending aorta and changes of calcification or atherosclerosis seen on CT (10) or intraoperatively (12). Histopathology revealed Giant Cell Aortitis in 35.8% (19), Takayasu’s Arteritis in 5.7% (3), Lymphoplasmacytic Aortitis in 22.6% (12), Mixed Lymphoplasmacytic with Giant Cells in 15.1% (8) and unclassified in 20.8% (11). No lymphoplasmacytic cases were stained for IgG4. Four patients received perioperative steroids. Only 1 had aortitis mentioned in the discharge summary. Of the 53, 24 had a follow-up visit, 8 of which had aortitis diagnosis in the follow-up notes. Two were referred to a rheumatologist, 4 had their family MD notified, and 2 already had a rheumatologist. Rheumatologist referrals status for the remaining 45 (84.9%) patients after discharge is unknown.

Conclusion: Ascending Aortitis is under-recognized as a cause of ascending aortic aneurysms. Diagnosis is often incidental as most patients are asymptomatic. The presence of an aortic bicuspid valve or aortic stenosis and changes of atherosclerosis on imaging or in the intraoperative period do not rule out an inflammatory aortic aneurysm. IgG4 staining should be routine practice for all patients with lymphoplasmacytic aortitis. Cardiovascular surgeons, Pathologists and rheumatologists need to develop protocols for appropriate care of this patient subset. Further multi-center studies are needed.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/inflammation-as-an-under-recognized-cause-of-ascending-aortic-aneurysms-a-single-center-clinical-and-pathological-study-of-53-cases-over-6-years/