Granulomatous inflammation of the aorta and its major branches, resulting in thickening and stenosis of the involved blood vessels and subsequent vascular symptoms

Epidemiology


  • Peak incidence: 15–45 years of age
  • Most commonly affects individuals of Asian heritage
  • ♀ > ♂ (9:1)

Etiology


Pathophysiology


Clinical features


  • Nonspecific symptoms: Fever, malaise, arthralgia, night sweats
  • Vascular symptoms
    • Decreased bilateral brachial and radial pulses (so-called pulseless disease)
    • Syncope, angina pectoris
    • Bilateral carotid bruits

Diagnostics


Imaging of the aorta and major branches

  • MR angiography (MRA)
    • Indications: preferred initial study for all patients
    • Findings
      • Thickening and contrast enhancement of the vascular wall (i.e., inflammation)
      • Luminal stenosis or occlusion

Treatment