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Aortitis

Inflammation of the aorta is discovered as an incidental histologic finding in 3% to 10% of patients undergoing aortic aneurysm surgery. It is representative of a cluster of large-vessel diseases that have various or unknown etiologies.

Causes and Risk Factors

  • Systemic lupus erythematosus

  • Rheumatoid arthritis

  • The HLA-B27–associated spondyloarthropathies

  • Antineutrophil cytoplasmic antibody–associated vasculitides

  • Behçet's disease

  • Cogan syndrome

  • Sarcoidosis

  • Tuberculosis

  • Syphilis

  • Salmonella

  • Other bacteria

The classification is based on location:

Isolated idiopathic thoracic aortitis

  • Giant cell aortits: inflammation indistinguishable from GCA

  • Lymphoplasmacytic infiltrate: a significant percentage (75%) of these patients have IgG4-related systemic disease

Chronic periaortitis (abdomen)

  • Idiopathic retroperitoneal fibrosis (Ormond disease)

  • Inflammatory abdominal aortic aneurysm: associated with smoking and family history of aortic aneurysm and can be associated with retroperitoneal fibrosis, some of these patients have IgG4-related systemic disease

  • Idiopathic isolated abdominal periaortitis: occurs without associated aneurysm or retroperitoneal fibrosis



Pubmed

UpToDate

Images


Web:

Medscape

Vasculitis Foundation

Radiopaedia

Cedars-Sinai

Imaging Spectrum - RadioGraphics

Multimodality Imaging of Aortitis - JACC


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