Takayasu syndrome

Takayasu Syndrome

Description, Causes and Risk Factors:

ICD-9-CM: 446.7.

A progressive obliterative arteritis of unknown origin involving chronic inflammation of the aortic arch with fibrosis and marked luminal narrowing that affects the aorta and its branches, often with complete or near complete occlusion of segments of the aorta; more common in females.

Alternative Names: Pulseless disease, Takayasu arteritis.

Takayasu disease or Takayasu arteritis is a chronic inflammation of the large blood vessel that distributes blood from the heart, called the aorta. It is most common in women (90%) of Asian descent. It usually begins between 10 and 30 years of age. The cause is unknown. Takayasu disease has also been referred to as pulseless disease and aortic arch syndrome

Takayasu Syndrome is a rare form of vasculitis. It causes the inflammation of the aorta and its major branches . It can also affect pulmonary and coronary arteries. The inflammation narrows the lumen of the arteries and can eventually cause thrombosis. In some cases, the weakness of the walls results in aneurysm leading to death. The disease was discovered by Dr. Mikito Takayasu, an ophthalmologist, in 1908 in Japan. He noticed a “wreathlike” formation of blood vessels behind the retina. This new blood vessel growth is a response to the narrowing of the arteries in the neck. Some of Takayasu's colleagues also found the pulse in the wrist to be absent due to the narrowing of the arteries in the arm.

Takayasu Syndrome is mostly prevalent in Asians although cases have been reported all over the world. Females are eight times for likely to be affected and the typical age of onset is between 15 and 30 years old.

The cause of Takayasu's is unknown. Some researches believe it is an autoimmune disease in which the body attacks its own cells. Some other studies suggest it may be caused by an infection (viral or bacteria) occurring in a person with predisposing genetic factors. Research, however, is still being conducted to find a definite cause.


Symptoms at first include myalgia, arthralgia, malaise, fever and arthritis. However since blood flow is being obstructed weakness in the legs and arms eventually result.

Other symptoms include:

    Arm weakness or pain with use.

  • Light-headedness.

  • Muscle or joint pain.

  • Skin rash.

  • Chest pain.

  • Dizziness.

  • Fatigue.

  • Night sweats.

  • Vision changes.

  • Weight loss.


Although a diagnosis of Takayasu Syndrome is hard to determine some tests can be used to reach a diagnosis. Blood tests can be used to check for a high white blood cell count or high levels of C-reactive protein, which are signs of inflammation. An increased erythrocyte sedimentary rate is another sign of inflammation. An angiography can be done to see if blood flow is normal. A sign of Takayasu's will be stenosis of several arteries. A CT angiography can be used to look at the structure of the aorta and monitor its blood flow. Doppler ultrasound can produce high-resolution images and detect subtle changes of artery walls. It can be used to distinguish between Takayasu Syndrome and arthrosclerosis.

Treatment Options:

Early detection and proper treatment can make the chronic Takayasu Syndrome very manageable. Treatment can include medications and/or surgery. The most common form of treatment is administering the corticosteroids, such as prednisone. After about a month, the does is gradually lowered to the lowest level possible to control the inflammation. If corticosteroids are not effective, the patient may be given cytotoxic drugs to reduce blood vessel inflammation. Examples of cytotoxic drugs include Rheumatrex, Imuran and Cytoxan. A more recent treatment used is the use of TNF-alpha inhibitors. These medications reduce inflammation by inhibiting the TNF-alpha protein. This protein plays a role in initiating an immune response. Since many of the medications mentioned about have side effects if taken long-term the dosage must be monitored by the physician. Sometimes surgery is necessary to fix narrowed or blocked arteries. These procedures include bypass surgery, percutaneous angioplasty and stenting.

Since the cause is still unknown, nobody is completely sure if it can be prevented. What researchers do know is complications can be prevented with early diagnosis and proper treatment

DISCLAIMER: This information should not substitute for seeking responsible, professional medical care.


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