Isolated aortitis - is it truly isolated? An approach to diagnosis and management.

Objective: With the rise in incidence of aortic aneurysm surgeries and the advances in large vessel imaging's ability to detect vessel wall inflammation, rheumatologists can expect to see an increase in isolated aortitis (IA) cases in their clinics. The purpose of this article is to review the latest data on IA, discuss its natural history and to provide an approach on how to diagnose and manage this inflammatory aortic disease.

Results: IA can be diagnosed on surgical histology or on imaging studies. Preoperative imaging in patients with thoracic aortic aneurysms does not detect all aortitis cases. Patients with IA have a high risk (up to 50%) of developing new aortic and branch lesions. Histologic and mechanistic studies show an overlap with giant cell arteritis.

Conclusions: Evaluation for underlying infections and systemic diseases is recommended for diagnosis. Surveillance of patients with IA with repeated clinical assessments and imaging is recommended.