Takayasu's Arteritis

Takayasu's arteritis is a rheumatic disease that causes the aorta and its branches to become inflamed (swollen and tender).

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Symptoms

About half the people who have Takayasu's arteritis develop a general feeling of being unwell, a fever, night sweats, tiredness, weight loss and joint pain. This phase gradually goes away and is replaced by chronic inflammation and damaging changes to the aorta and its branches.

The other half of the people who have this disease have symptoms only of chronic inflammation and damage to the aorta.

In advanced stages of the disease, the walls of the arteries may become weak and develop aneurysms. These are weak spots that bulge out from the artery walls due to the pressure of the blood flowing through the artery. If they aren't discovered, they can burst and cause bleeding inside the body.

The blood vessels that branch off of the aorta are particularly at risk. Any or all of them may become blocked. Fainting and temporary interruptions of the blood flow to the brain and head may occur along with weakness of the jaws when chewing or speaking or the arms.

The face and arms may lose muscle mass. Disturbances in a person's ability to see are common. Sometimes the portion of the aorta that runs through the abdomen may be affected. This can cause serious increases in the blood pressure of the kidneys.

The involvement of the coronary arteries, severe high blood pressure or changes in the aorta's ability to work efficiently can lead to heart failure. Much less often, blockages in the arteries of the lungs can cause high blood pressure in the lungs.

Causes and Risk Factors

Takayasu's arteritis occurs throughout the world. However, persons with the following characteristics have a higher risk of developing Takayasu's arteritis:

  • Being between the ages of 15 and 30
  • Being a woman. (Women are about eight times more likely than men to develop this condition.)
  • Being a young Asian woman.
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Diagnosis

During a physical examination, the doctor will check for the blood pressure rate in the involved arteries arising from the aorta. In Takayasu's arteritis, pulses and blood pressure will be low or impossible to find. By contrast, there will be generally brisk pulses in the legs. Bruits may be heard over partially narrowed arteries.

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Treatments

In many patients, Takayasu's arteritis goes away without major complications. Such patients may do well for years. In other cases, complications such as stroke, severe high blood pressure, heart attack, heart failure or aneurysms may occur.

During the acute stage of the symptoms, corticosteroids such as prednisone may be used. Once the symptoms start to go away, the amount of prednisone is tapered off, although treatment may be needed for several months. In cases where a person can't take corticosteroids or where they are not effective, cyclophosphamide may be given. Some studies show that vigorous treatment with corticosteroids and cyclophosphamide when the condition is acute may reduce long-term complications of the blood vessels.

Aspirin or drugs that prevent the blood from clotting such as warfarin may be recommended for ischemia. Hypertension should be treated aggressively. ACE inhibitors may be particularly effective because hypertension is frequently of renovascular origin.

In the later stages of the disease, surgery may be needed to reestablish blood flow in blocked arteries. Balloon angioplasty may provide temporary improvement in selected cases. Arterial and aortic aneurysms may require surgery as well.