Background/Purpose:  Idiopathic Aortitis (IA) is poorly defined, with no specific criteria for its classification or diagnosis, except for presence of aortic inflammation and the absence of clinical features of another systemic condition. We aim to establish a prospective cohort of patients with surgically diagnosed aortitis and compare disease features in patients with IA and aortitis secondary to a systemic inflammatory condition (SA).

Methods:  This is a single-center, prospective cohort study. Patients are recruited following identification of aortitis on surgical specimen. Baseline assessment includes clinical assessment, inflammatory markers (ESR and CRP), serology to exclude a connective tissue disease and infectious causes, and full imaging of the aorta and its branches. Patients are classified as either IA or SA, and followed prospectively. Clinical assessments and measurements of ESR and CRP are done at least yearly, and full imaging of the aorta and branches at least every 2 years. Primary outcome of interest is radiographic progression of aortitis (new aortic or branch lesions or progression of existing lesions). Secondary outcomes include the need for delayed immunosuppressive therapy, need for aortitis-related surgeries, or a change in diagnosis (from IA to SA).

Results: Fourteen patients have been enrolled to date, 8 IA, 6 SA (5 giant cell arteritis (GCA), 1 rheumatoid arthritis (RA)). The majority of patients are female (7/8 IA, 6/6 SA); average age 74 and 75 years in IA and SA respectively. Four out of eight patients with IA and 2/6 with SA had aortic branch lesions at baseline. Mean follow-up is 2.3 and 1.8 years in IA and SA groups, respectively. One patient (with SA) was found to have worsening aortic dilatation 15.4 months post-biopsy (PB); new aortic branch involvement was observed in 3/8 patients with IA (identified at mean 9.3 months PB) and in 3/6 SA (identified at mean 13.3 months PB). Surgical re-intervention was required in 2 patients with IA (mean 6.5 months PB). Two patients with IA received a combination of prednisone (initiated at a mean dose of 45 milligrams (mg) daily) and methotrexate (20mg weekly) starting at 36.0 months 38.2 months PB respectively for worsening aortitis. Two patients with SA received prednisone for worsening of their underlying condition, not related to diagnosis of aortitis: one was finishing a course of prednisone initiated for flare of RA prior to diagnosis of aortitis and the other was treated for symptomatic GCA 3.2 months PB (starting dose of 40 mg daily). One patient with IA died due to a cause unrelated to aortitis 17.5 months PB.

Conclusion:  Preliminary data suggest patients with IA and SA may experience a significant rate of radiographic vascular progression, particularly in terms of development of branch vessel abnormalities. The number of events was too low to detect significant differences between IA and SA with respect to any of the studied parameters. Further enrollment and a longer duration of follow-up are needed. Controlled studies would be required to assess whether treating IA with corticosteroids at the time of diagnosis would reduce radiographic progression or other complications.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/prospective-cohort-of-surgically-diagnosed-aortitis-at-the-ottawa-hospital/