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Takayasu's arteritis ( TA), also known as aortic arch syndrome, nonspecific aortoarteritis, and pulseless disease, (2025). 9780323547536, Elsevier. ISBN 9780323547536 is a form of large vessel granulomatous vasculitisAmerican College of Physicians (ACP). Medical Knowledge Self-Assessment Program (MKSAP-15): Rheumatology. "Systemic Vasculitis." Pg. 65–67. 2009, ACP. with massive fibrosis and vascular narrowing, most commonly affecting young or middle-aged women of descent, though anyone can be affected. It mainly affects the aorta (the main blood vessel leaving the heart) and its branches, as well as the pulmonary arteries. Females are about 8–9 times more likely to be affected than males.
Those with the disease often notice symptoms between 15 and 30 years of age. In the Western world, atherosclerosis is a more frequent cause of obstruction of the aortic arch vessels than Takayasu's arteritis. Takayasu's arteritis is similar to other forms of vasculitis, including giant cell arteritis which typically affects older individuals. Due to obstruction of the main branches of the aorta, including the left common carotid artery, the brachiocephalic artery, and the left subclavian artery, Takayasu's arteritis can present as pulseless upper extremities (arms, hands, and wrists with weak or absent pulses on the physical examination) which may be why it is also commonly referred to as the "pulseless disease." Involvement of renal arteries may lead to a presentation of renovascular hypertension.
Of note is the function of renal artery stenosis in the causation of high blood pressure: Normally perfused kidneys produce a proportionate amount of a substance called renin. Stenosis of the renal arteries causes hypoperfusion (decreased blood flow) of the juxtaglomerular apparatus, resulting in exaggerated secretion of renin, and high blood levels of aldosterone, eventually leading to water and salt retention and high blood pressure. The neurological symptoms of the disease vary depending on the degree; the nature of the blood vessel obstruction; and can range from lightheadedness to seizures (in severe cases).
One rare, important feature of the Takayasu's arteritis is human eye involvement in form of visual field defects, vision loss, or retinal hemorrhage. Some individuals with Takayasu's arteritis may present with only late vascular changes, without a preceding systemic illness. In the late stage, weakness of the arterial walls may give rise to localized . As with all aneurysms, the possibility of rupture and vascular bleeding is existent and requires monitoring. In view of the chronic process and good collateral development, Raynaud's phenomenon or digital gangrene are very rare in Takayasu arteritis.
Laser Doppler imaging by near-infrared digital holography can reveal characteristic blood flow waveforms in the central artery and vein of the retina in patients with vascular insufficiency who may exhibit a smooth systo-diastolic pulse in the central retinal artery. This technique enables non invasive functional microangiography by high-contrast measurement of endoluminal blood flow profiles in vessels in the posterior segment of the eye with a spatial resolution comparable to state-of-the-art indocyanine green angiography.
Another gene associated with this condition is MLX (Max-like protein X)Tamura N, Maejima Y, Matsumura T, Vega RB, Amiya E, Ito Y, Shiheido-Watanabe Y, Ashikaga T, Komuro I, Kelly DP, Hirao K, Isobe M (2018) Single-nucleotide polymorphism of the MLX gene is associated With Takayasu arteritis. Circ Genom Precis Med 11(10):e002296. doi: 10.1161/CIRCGEN.118.002296.
Contrast angiography has been the gold standard. The earliest detectable lesion is a local narrowing or irregularity of the lumen. This may develop into stenosis and occlusion. The characteristic finding is the presence of "skip lesions," where stenosis or aneurysms alternate with normal vessels. Angiography provides information on vessel anatomy and patency but does not provide information on the degree of inflammation in the wall.
The age at onset helps to differentiate Takayasu's arteritis from other types of large vessel vasculitis. For example, Takaysu's arteritis has an age of onset of <40 years, while giant cell arteritis has an age of onset >60 years.
Takayasu arteritis is not associated with ANCA, rheumatoid factor, ANA, and anticardiolipin antibodies.
Promising results are achieved with mycophenolate and tocilizumab.Singh, Ambrish et al., Efficacy and safety of tocilizumab in treatment of Takayasu arteritis: A systematic review of randomized controlled trials. Mod Rheumatol. 2020;31:1-20 If treatment is not kept to a high standard, long-term damage or death can occur.
Patients who do not respond to steroids may require revascularization, either via vascular bypass or Angioplasty. Outcomes following revascularization vary depending on the severity of the underlying disease.
It is now known that the blood vessel malformations that occur in the retina are an Angiogenesis response to the arterial narrowings in the neck and that the absence of pulses noted in some people occurs because of narrowings of the blood vessels to the arms. The eye findings described by Takayasu are rarely seen in patients from North America and British Columbia.
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