Study of 52 Patients with Idiopathic Aortitis from a Cohort of 1,204 Surgical Cases

Objective: To determine 1) the frequency of idiopathic aortitis in a large surgical cohort, 2) how often aortitis was associated with a systemic disease, and 3) whether the findings of aortitis in resected specimens predicted future occurrences of clinically apparent vascular injury due to vasculitis.

Methods: Retrospective chart and pathology review of 1,204 aortic surgical specimens that were gathered over a period of 20 years at a tertiary care medical center. A standardized database was used to compare features of aortitis patients with those of controls in whom inflammation was not present.

Results: Among 1,204 aortic specimens, 52 (4.3%) were clinically and pathologically classified as idiopathic aortitis. Sixty-seven percent of patients with idiopathic aortitis were women. In 96% of idiopathic aortitis patients with aneurysm formation, aortitis was present only in the thoracic aorta. Among 383 thoracic aortic aneurysms, 12% had idiopathic inflammatory features. In 96% of patients with idiopathic aortitis, symptoms of systemic illness had not been present at the time of surgery. In 31%, aortitis was associated with a remote history of vasculitis and a variety of other systemic disorders. During a mean followup period of 41.2 months, new aneurysms were identified among 6 of 25 patients who were not treated with glucocorticoids. None were identified among 11 patients who were treated with glucocorticoids (mean followup 35.5 months).

Conclusion: The frequency of idiopathic aortitis in a large surgical cohort was found to be 4.3%. Thoracic aorta aneurysm formation, in the absence of systemic illness, was the most common manifestation. In the setting of a cardiovascular surgery practice, aortitis may first become apparent only after pathologic evaluation of excised specimens. The appropriate medical treatment for patients with incidentally discovered aortitis is not known. Because 17% of our patients subsequently developed new aneurysms, we suggest that it would be prudent for patients with idiopathic aortitis identified at the time of surgery to be periodically evaluated for recurrent or persistent disease.