Takayasu's 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 form the mainstay of treatment, with the additional use of steroid-sparing immunosuppressive agents for resistant disease. Surgery may be required for established complications.
Long-term complications are due mainly to arterial occlusion and related damage, including limb ischaemia and cardiac and neurological manifestations.
Takayasu's 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 ischaemia 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
- presence of risk factors
- upper or lower limb claudication
- absent pulse(s)
- unequal blood pressures
- vascular bruits
- low-grade fever
Other diagnostic factors
- transient ischaemic 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
- catheter angiogram
- Doppler ultrasound
- positron emission tomography with radiolabelled fluorodeoxyglucose (PET-FDG)
- Giant cell arteritis (GCA)
- Essential hypertension
- Guideline for the management of giant cell arteritis and Takayasu arteritis
- Criteria for the classification of Takayasu arteritis
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