Tuesday, December 13, 2011

Aortitis: Differential Diagnosis

Aortitis, the inflammation of the aortic wall, can be due to infectious or noninfectious conditions. Patients present with nonspecific signs, symptoms, and laboratory findings that can include pain, fever, vascular insufficiency, and elevated levels of acute phase reactants.

Differential considerations include:

  • Large-vessel vasculitides (shown above):
    • Giant cell arteritis: Affects large and medium-sized vessels. Often involves the superficial cranial arteries.
    • Takayasu arteritis: Abdominal aorta most commonly affected. Descending thoracic aorta and aortic arch can also be involved. Look for stenosis or luminal narrowing of aorta and branch vessels. Aneurysmal dilatation less common, but can be seen after destruction of media. Arterial wall calcification (can be seen in chronic cases) is typically linear and spares the ascending aorta.
    • Rheumatoid arthritis: Aortitis is rare. Heart, aortic valve, and great vessels can be affected.
    • Systemic lupus erythematosus: Aortitis uncommon.
    • Ankylosing spondylitis: Aortic root and valve disease seen in 80% of cases. Aortic wall thickening is seen in 60% of affected patients.
    • Reiter syndrome:
  • Medium- and small-vessel vasculitides:
    • Wegener arteritis:
    • Polyarteritis nodosa:
    • Behçet disease: Wall-enhancing saccular pseudoaneurysms can be seen in the aorta and branch vessels in 20% of patients.
    • Relapsing polychondritis: May manifest as aortic root dilatation and aortitis.
    • Cogan syndrome: Ocular, inner ear, and vascular inflammation. Patients are usually white young adults. Aortitis and valvulitis seen in ~10% of patients.
  • Isolated aortitis: Isolated idiopathic (thoracic) aortitis, Chronic periaortitis (Idiopathic retroperitoneal fibrosis, inflammatory abdominal aortic aneurysm, perianeurysmal aortitis, idiopathic isolated abdominal periaortitis).
  • Radiation-induced: Usually years after exposure to high-dose radiation. Can manifest as thrombosis, pseudoaneurysm, rupture, stenosis, and accelerated wall calcification.
  • Bacterial: Salmonella, Staphylococcus, Streptococcus pneumoniae
  • Syphilis: The typical calcification of the ascending aorta is uncommon.
  • Mycobacterial: Mycobacterium tuberculosis
  • Viral: HIV.


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