Inflammatory Diseases of the Aorta

Inflammatory aortitis is most often caused by large vessel vasculitis (LVV), including giant cell arteritis , Takayasu’s arteritis , immunoglobulin G4–related aortitis, and isolated aortitis. There are distinct differences in the clinical presentation, imaging findings, and natural history of LVV that are important for the cardiovascular provider to know. If possible, histopathologic specimens should be obtained to aide in accurate diagnosis and management of LVV. In most cases, corticosteroids are utilized in the acute phase, with the addition of steroid-sparing agents to achieve disease remission while sparing corticosteroid toxic effects. Endovascular and surgical procedures have been described with success but should be delayed until disease control is achieved whenever possible. Long-term management should include regular follow-up with rheumatology and surveillance imaging for sequelae of LVV. • Aortitis is most often a manifestation of large-vessel vasculitis, and may result in wall thickening, stenosis, aneurysm, or dissection. • Diagnosis and management require an understanding of the clinical spectrum, imaging findings, and histopathology in patients with large vessel vasculitis. • Standardized criteria for diagnosis, imaging findings, and treatment endpoints are needed.