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