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