Takayasu's arteritis

Takayasu's arteritis (TA), also known as aortic arch syndrome, nonspecific aortoarteritis, and pulseless disease,[2] is a form of large vessel granulomatous vasculitis[3] with massive intimal fibrosis and vascular narrowing, most commonly affecting young or middle-aged women of Asian descent, though anyone can be affected.

In the Western world, atherosclerosis is a more frequent cause of obstruction of the aortic arch vessels than Takayasu's arteritis.

Some people develop an initial "inflammatory phase" characterized by systemic illness with signs and symptoms of malaise, fever, night sweats, weight loss, joint pain, fatigue, and fainting.

[3] 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.

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 ocular involvement in form of visual field defects, vision loss, or retinal hemorrhage.

In view of the chronic process and good collateral development, Raynaud's phenomenon or digital gangrene are very rare in Takayasu arteritis.

[8] 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.

[citation needed] Although the cause of Takayasu arteritis is unknown, the condition is characterized by segmental and patchy granulomatous inflammation of the aorta and its major derivative branches.

[12] Seeing abnormal diffuse arterial wall thickening, the 'macaroni sign', with ultrasound is highly suggestive of the condition.

[12] The age at onset helps to differentiate Takayasu's arteritis from other types of large vessel vasculitis.

[citation needed] Patients who do not respond to steroids may require revascularization, either via vascular bypass or angioplasty and stenting.

Blood flow pulse wave in the central retinal artery (red) and vein (blue), measured in the eye fundus of a patient with Takayasu arteritis by laser Doppler imaging .
Axial T1-weighted post- gadolinium MRI in a patient with Takayasu arteritis showing thickened, enhancing aortic wall, consistent with large vessel vasculitis