Takayasu arteritis is a vasculitis of large vessels that particularly affects the aorta and its primary branches.
Generally more common in women and typically presents before the age of 40 years.
Typical symptoms include limb claudication on exertion, chest pain, and systemic symptoms of weight loss, fatigue, low-grade fever, and myalgia.
On examination, vascular bruits may be audible over the carotids, abdominal aorta, or subclavian vessels. Unequal blood pressures may be recorded between sides, and a murmur of aortic regurgitation may be heard if there is aortic root dilation.
The diagnosis is usually made by vascular imaging.
Glucocorticoids are initial therapy, with additional use of corticosteroid-sparing immunosuppressants. Biologic agents are often used for refractory disease. Surgery may be required for established complications.
Long-term complications are due mainly to arterial occlusion and related damage, including limb ischemia and cardiac and neurologic manifestations.
Takayasu arteritis is a chronic granulomatous vasculitis affecting large arteries: primarily the aorta and its main branches. Vascular inflammation can cause stenosis, occlusion, and aneurysm formation. Symptoms from vascular ischemia include claudication and stroke. Diminished or absent pulses and hypertension are common. Constitutional symptoms, including fever and weight loss, are often accompanied by elevation of acute phase markers.
History and exam
Key diagnostic factors
- upper or lower limb claudication
- absent pulse(s)
- unequal blood pressures
- vascular bruits
- low-grade fever
Other diagnostic factors
- transient ischemic attack (TIA)
- weight loss
- dizziness on upper-limb exertion
- chest pain
- abdominal pain
- shortness of breath
- night sweats
- heart murmur
- visual symptoms
- erythema nodosum
- pyoderma gangrenosum
- genetic predisposition
- female sex
- age <40 years
- Asian ethnicity
1st investigations to order
- erythrocyte sedimentation rate (ESR)
- C-reactive protein (CRP)
- computed tomography angiography (CTA)
- magnetic resonance angiography (MRA)
Investigations to consider
- Doppler ultrasound
- positron emission tomography with radiolabeled fluorodeoxyglucose (PET-FDG)
- Giant cell arteritis (GCA)
- Essential hypertension
- 2022 American College of Rheumatology/EULAR classification criteria for Takayasu arteritis
- 2022 ACC/AHA guideline for the diagnosis and management of aortic disease: a report of the American Heart Association/American College of Cardiology Joint Committee on Clinical Practice Guidelines
- Log in or subscribe to access all of BMJ Best Practice
Use of this content is subject to our disclaimer