Takayasu's arteritis
A large-vessel vasculitis causing pulselessness and vascular narrowing.
Takayasu's arteritis (TA), also known as aortic arch syndrome, nonspecific aortoarteritis, and pulseless disease, is a form of large vessel granulomatous vasculitis with massive intimal fibrosis and vascular narrowing. It most commonly affects young or middle-aged women of Asian descent, though anyone can be affected, and primarily involves the aorta and its branches as well as the pulmonary arteries.
- First described
- 1908
- Described by
- Mikito Takayasu
- Commonly affects
- Young or middle-aged women of Asian descent
- Gender ratio
- Females 8–9 times more likely than males
- Typical age of onset
- 15–30 years
- Key feature
- Pulseless upper extremities
- Genetic associations
- HLA-B*52, FCGR2A/FCGR3A, IL12B, IL6, RPS9/LILRB3
Lore & Background
Takayasu's arteritis is characterized by an initial inflammatory phase with systemic symptoms such as malaise, fever, night sweats, weight loss, joint pain, fatigue, and fainting, often accompanied by anemia and elevated inflammatory markers. This is followed by a pulseless phase marked by vascular insufficiency from intimal narrowing, leading to arm or leg claudication, renal artery stenosis causing hypertension, and neurological manifestations due to decreased blood flow to the brain. Ocular involvement can include visual field defects, vision loss, or retinal hemorrhage.
Reader's Guide
Takayasu's arteritis is significant as a rare but serious cause of large-vessel obstruction, particularly in young women. Its recognition is crucial for early treatment with steroids such as prednisone, which can control inflammation and prevent long-term damage. The disease's genetic associations, including HLA-B*52 and multiple other loci, highlight its autoimmune nature. Diagnosis relies on imaging techniques like angiography, CT, MRI, or FDG PET, which reveal characteristic vessel narrowing and skip lesions. Without adequate treatment, the condition can lead to severe complications such as aneurysms, renovascular hypertension, and neurological deficits. The disease's historical description by Mikito Takayasu in 1908 underscores its long-standing recognition in medicine.
Did You Know?
- Takayasu's arteritis is also called pulseless disease due to weak or absent pulses in the upper extremities.
- The condition is about 8–9 times more common in females than males.
- Renal artery stenosis in Takayasu's arteritis can cause renovascular hypertension through exaggerated renin secretion.
- Laser Doppler imaging by near-infrared digital holography can reveal characteristic blood flow waveforms in the retina of affected patients.
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