Aortitis

Clinical Manifestations

• Varied, depending on location and cause
• Classically, back or abdominal pain with fever
• May cause aortic regurgitation and acute aortic syndrome

Differential Diagnosis

• Inflammatory disorders
  • Large-vessel vasculitis: giant cell arteritis, Takayasu arteritis, rheumatoid arthritis, systemic lupus erythematosus, ankylosing spondylitis, Reiter syndrome
  • Other vasculitides: ANCA-associated vasculitis (granulomatosis with polyangiitis, polyarteritis nodosum, microscopic polyangiitis), Behçet disease, Cogan syndrome, relapsing polychondritis
  • Sarcoidosis
  • Isolated aortitis
    • Isolated idiopathic aortitis
    • Chronic periaortitis: idiopathic retroperitoneal fibrosis, inflammatory abdominal aortic aneurysm, perianeurysmal aortitis, idiopathic isolated abdominal periaortitis
• Infections
  • Bacterial: Salmonella, Staphylococcus, Streptococcus pneumoniae, others
  • Syphilis
  • Tuberculous aortitis

Investigations

• CTA or MRA to noninvasively confirm the diagnosis
• Duplex ultrasound of temporal arteries, looking for halo sign to suggest GCA
• CBC, creatinine, liver panel, ESR/CRP
• Blood cultures, for bacterial cause
• Syphilis serology
• Tuberculin skin test
• Rheumatology panel: ANA, ANCA, RF

Further Reading

• Aortitis. Circulation. 2008;117(23):3039–51. doi: 10.1161/CIRCULATIONAHA.107.760686