Takayasu's Arteritis

Takayasu's arteritis is a rheumatic disease that causes the aorta and its branches to become inflamed (swollen and tender). The aorta is the largest artery in the body. It rises from the heart's left ventricle (the major chamber that pumps blood out of the heart) and is filled with oxygen-rich blood that travels throughout the body.

Inflammation of the aorta and its branches can lead to narrowing and blockages in the artery that can cause tissue damage or death if left untreated.


Symptoms of Takayasu's arteritis can vary depending on whether the condition is in the first or second stage. The first stage is not always noticed because inflammation in the artery can exist for years before patients are aware of symptoms. Only about 50 percent of patients experience symptoms of the first stage. When symptoms are present during the first stage, they may include:

  • Fatigue
  • Fast and unintentional weight loss
  • Muscle aches
  • Joint pain
  • Slight fever

This phase gradually goes away and is replaced by chronic inflammation and damaging changes to the aorta and its branches.

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. Temporary interruptions of blood flow to the brain and head may occur, or the portion of the aorta that runs through the abdomen may be affected.

When the condition progresses to the second stage, the affected blood vessels have begun to narrow and blood flow to tissues and organs has become limited. Symptoms associated with this stage of the condition include:

  • Arm or leg weakness or pain with use (claudication)
  • Lightheadedness
  • Dizziness
  • Fainting
  • Headaches
  • Memory problems
  • Trouble thinking
  • Shortness of breath
  • Visual changes
  • High blood pressure
  • Difference in blood pressure between each arm
  • A very feint or absent pulse in the wrists
  • Too few red blood cells (anemia)
  • Chest pain
  • Abdominal pain
  • Skin rash
  • Night sweats

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

It is unknown what causes the initial inflammation in the aorta. Some research suggests that it is an autoimmune disorder in which the body attacks the aorta as if it is a foreign object. This may be triggered by an infection.

Takayasu's arteritis occurs throughout the world. However, it is most common in Asian women. Other patients at an increased risk are young women between the ages of 15 and 30 years old.


Diagnosis of Takayasu's arteritis will begin with the physician performing a physical examination and recording the patient's medical history. During the physical examination, the physician will check the blood pressure in the involved arteries arising from the aorta. In Takayasu's arteritis, pulses and blood pressures in these arteries will be low or impossible to find. By contrast, there will be generally brisk pulses in the legs. The physician may hear the sound of blood rushing past a blockage (bruits), over partially narrowed arteries.

Imaging tests may be done to look at the blood vessels and the blood flow through them. These test may include magnetic resonance angiography (MRA), MRI, CT scans, or angiography. These imaging tests will also highlight any areas of inflammation.

Other diagnostic tests may include blood tests to look for an elevated "sed rate," which looks at how fast the patient's red blood cells settle in a tube. When inflammation is present the cells settle much more quickly.


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 patient can't take corticosteroids, or where they are ineffective, 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 caused by the kidney's hormonal reaction to the limited blood supply that is reaching them (renovascular).

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.

The knowledgeable and highly trained staff at the Cedars-Sinai Heart Institute will work with each patient to determine the best treatment option.