Vasculitis Codexery

Takayasu's arteritis

A large-vessel vasculitis causing pulselessness and vascular narrowing.

Takayasu's arteritis

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.

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