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Takayasu Arteritis

Serious Disorder of the Aorta and Large Arteries

By Mary Kugler, R.N., About.com

Updated: April 26, 2008

About.com Health's Disease and Condition content is reviewed by our Medical Review Board

Takayasu arteritis affects the aorta and its branches

Takayasu arteritis affects the aorta and its branches

Photo © A.D.A.M.
Takayasu arteritis is a serious chronic inflammatory disease of the large arteries, usually affecting the aorta and its large branches. Although the cause of the disease is unknown, studies suggest that it may occur after certain types of infections. It also may be an autoimmune disorder.

It is estimated that Takayasu arteritis affects about only 3 people of every million individuals throughout the world. It affects people of all ethnic backgrounds but is most common among Asian females of childbearing age.

Symptoms

The inflammation caused by Takayasu arteritis can cause partial or complete blockage of blood vessels, narrowing of blood vessels, or weakening of the walls of a blood vessel (known as an aneurysm).

The symptoms depend on how extensive the inflammation is and where it occurs in the body. Symptoms of Takayasu arteritis may follow a pattern:

  • First stage - fatigue, malaise, muscle aches, joint pain, headaches, skin rash, fever or weight loss
  • Second stage - this is known as the vascular inflammatory stage, which may cause pain in the extremities and joints, arm numbness, blurry or double vision, headaches, or shortness of breath. These symptoms occur because of reduced blood supply to the affected area. Occasionally, the first and second stage symptoms occur together.
  • Third stage - generally is associated with remission (the disease going away), but this stage does not occur in all individuals, and the disease process may come back (relapse).
Additional symptoms may occur depending upon which organs of the body are affected by the blood vessel changes. For example, if an artery to the kidney is affected, high blood pressure and kidney problems may occur.

Diagnosis

At first, the vague symptoms of Takayasu arteritis may make it difficult to diagnose. The main sign that would point to the disease is a difference in pulses between the right and left arms. Sometimes no pulse is felt in either or both arms. The diagnosis may be suspected based on other signs or symptoms present, as well as the individual being a young female adult (usually younger than 40 years). Lab tests may show anemia. Other lab tests, such as the erythrocyte sedimentation rate and the c-reactive protein test, also may show signs of inflammation.

Angiography (using radio-opaque dye to take x-ray pictures of an artery) can confirm Takayasu arteritis. According to the American College of Rheumatology, the angiography must show narrowing or blockage of the entire aorta, its primary branches, or large arteries in the arms or legs, and these changes cannot be caused by other diseases, such as heart disease.

Treatment

The goals of treatment include reducing inflammation and slowing or stopping the autoimmune disease process. This is usually done with corticosteroid medication such as prednisone, which may need to be taken for a long time. If corticosteroids do not reduce the symptoms, cytotoxic drugs such as methotrexate (Rheumatrex), azathioprine, or, more recently, cyclophosphamide (Cytoxan) may be used.

Medical care depends on the symptoms and their severity. Complications of Takayasu arteritis may include stroke, bleeding in the brain (intracranial hemorrhage), seizures, or kidney failure. Medication for high blood pressure may be needed. It is possible that a narrowing, blockage, or aneurysm in a major artery may need to be repaired surgically. Treatment is specific to the individual problem.

Source:

"About Takayasu Arteritis." Takayasu's Arteritis Research Association. 14 Aug 2007

"What You Need to Know About Takayasu’s Arteritis." Rheumatic and Immunologic Diseases. 7 Jul 2006. The Cleveland Clinic. 14 Aug 2007

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