Takayasu arteritis
Takayasu arteritis is a large-vessel vasculitis of the aorta and its primary branches, occurring almost exclusively in women under 40, that produces the pulseless disease phenotype of absent pulses, an interarm blood pressure differential, and bruits. Magnetic resonance or computed tomographic angiography makes the diagnosis, and treatment is high-dose glucocorticoid induction paired with a steroid-sparing agent such as methotrexate.
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What this article covers
- Overview
Definition: Takayasu arteritis is a chronic, idiopathic large-vessel vasculitis of the aorta and its primary branches, with granulomatous wall inflammation similar to giant cell arteritis.
- Epidemiology
Rare disease: prevalence is about 40 per million in Japan and up to 8 per million elsewhere, far less common than giant cell arteritis.
- Risk factors
Female sex: the great majority of patients are women.
- Pathophysiology
Granulomatous panarteritis: inflammation targets the aorta most commonly, followed by the subclavian, common carotid, and renal arteries; the pulmonary arteries may also be involved.
- Clinical features
Systemic inflammatory prodrome: an early phase of fatigue, malaise, arthralgias, and myalgias, easily mistaken for another cause of unexplained fever.
- Differential diagnosis
Giant cell arteritis: the other large-vessel vasculitis with similar histopathology, but it occurs in patients over 50, targets the cranial branches of the external carotid artery (temporal…
- Diagnosis
The diagnosis is made by vascular imaging demonstrating characteristic large-vessel involvement, supported by classification criteria: a patient is classified as having Takayasu arteritis when at…
- Management
First and immediate step, start high-dose oral glucocorticoids: prednisone 1 mg/kg/day PO once daily, up to 60 mg/day, maintained about 1 month until symptoms and markers improve.
- Complications
Aortic aneurysm and dissection: dilatation develops in roughly one third of patients and requires long-term surveillance; an aneurysmal segment carries a risk of dissection, a catastrophic…
- Prognosis
Overall survival is favorable: the 10-year survival rate is approximately 90%.
- Prevention
No primary prevention exists; efforts focus on limiting vascular and treatment-related damage once diagnosed.
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Related articles
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Giant cell arteritis is a granulomatous large- and medium-vessel vasculitis of patients over 50 that threatens irreversible blindness through ischemic optic neuropathy. Diagnosis is clinical and must not delay treatment: start high-dose glucocorticoids immediately on suspicion, intravenous methylprednisolone pulse for visual loss, then confirm with temporal artery biopsy within 1 to 2 weeks and add tocilizumab as glucocorticoid-sparing therapy.
- Vasculitis: an overview
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- Renal artery stenosis
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- Aortic regurgitation
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- Aortic dissection
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