Aortitis and periaortitis: The puzzling spectrum of inflammatory aortic diseases

Presse Med. 2020 Apr;49(1):104018. doi: 10.1016/j.lpm.2020.104018. Epub 2020 Mar 28.


Aortitis and periaortitis are inflammatory diseases of the aorta and its main branches; they differ in the extension of inflammation, which is confined to the aortic wall in aortitis, and spreads to the periaortic space in periaortitis. Aortitis is classified as non-infectious or infectious. Non-infectious aortitis represents a common feature of large-vessel vasculitides but can also be isolated or associated with other rheumatologic conditions. Periaortitis can be idiopathic or secondary to a wide array of etiologies such as drugs, infections, malignancies, and other proliferative diseases. Notably, both aortitis and periaortitis may arise in the context of IgG4-related disease, a recently characterised fibro-inflammatory systemic disease. Prompt recognition, correct diagnosis and appropriate treatment are essential in order to avoid life-threatening complications.

Publication types

  • Review

MeSH terms

  • Aorta / pathology
  • Aortitis* / classification
  • Aortitis* / diagnosis
  • Aortitis* / etiology
  • Aortitis* / pathology
  • Diagnostic Imaging / methods
  • Giant Cell Arteritis / diagnosis
  • Giant Cell Arteritis / etiology
  • Giant Cell Arteritis / pathology
  • Humans
  • Immunoglobulin G4-Related Disease / complications
  • Retroperitoneal Fibrosis / complications
  • Retroperitoneal Fibrosis / pathology
  • Takayasu Arteritis / diagnosis
  • Takayasu Arteritis / etiology
  • Takayasu Arteritis / pathology