Inflammatory Diseases of the Aorta: JACC Focus Seminar, Part 2

J Am Coll Cardiol. 2022 Aug 23;80(8):832-844. doi: 10.1016/j.jacc.2022.05.046.

Abstract

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.

Keywords: aneurysm; aortitis; dissection; large vessel vasculitis.

Publication types

  • Review

MeSH terms

  • Aorta / pathology
  • Aortitis* / diagnostic imaging
  • Aortitis* / therapy
  • Giant Cell Arteritis* / diagnosis
  • Giant Cell Arteritis* / drug therapy
  • Humans
  • Immunoglobulin G
  • Takayasu Arteritis* / diagnosis
  • Takayasu Arteritis* / pathology
  • Takayasu Arteritis* / therapy

Substances

  • Immunoglobulin G