Noninfectious ascending aortitis: a case series of 64 patients.

Objective: To identify the clinical presentation and histopathologic characteristics of noninfectious ascending aortitis.

Methods: A retrospective medical record and histopathology review was performed of patients with histologic evidence of active noninfectious aortitis who underwent ascending aortic aneurysm resection at Mayo Clinic between January 1, 2000, and February 28, 2006. Clinicopathologic features were recorded, including demographics, clinical presentation, laboratory, imaging findings, histopathology, complications, treatment, and outcome.

Results: Sixty-four patients (50% women) were identified; the majority were Caucasian (83%) and elderly (mean age 69.1 yrs). Upon initial presentation, 45% had aneurysm-related symptoms, 33% were asymptomatic, 12.5% had constitutional symptoms, 4.7% had symptoms referable to cranial arteries, and 9.4% had polymyalgia rheumatica (PMR) symptoms. The majority (81%) were of "isolated" variant, with no rheumatologic history. Mean preoperative erythrocyte sedimentation rate was 16.2 +/- 23.3 mm/h (n = 20). Additional vascular imaging abnormalities were present in 72% of patients, including stenoses and/or ectasia of major aortic branches and descending thoracic or abdominal aneurysms. Giant cells were seen in 71.9%. Median followup time was 15.4 months, during which 6 (9.4%) patients died. Only 22 (34%) patients received corticosteroids, with uncertain effect on development of recurrent aneurysms, rupture, or dissections.

Conclusion: Noninfectious ascending aortitis frequently occurs even in the absence of history, symptoms, or signs of giant cell arteritis (GCA) or PMR. When discovered, such patients should be followed closely, as a majority have additional vascular abnormalities. More studies are needed to determine optimal strategies for surveillance, detection, and treatment of ascending aortitis, which may represent a clinical entity distinct from classical GCA.