Takayasu’s Arteritis (TA) is an inflammation of large blood vessels, that is more common in women than in men. 80% to 90% of cases occur in women. People who get this form of vasculitis are usually between the ages of 10 and 40 years when the disease starts. Rarely, however, even children and young infants develop TA. The disease affects people around the world, but is most common in Asians, especially in Japan.

TA primarily affects the aorta, the major blood vessel that carries blood from the heart to supply oxygen to the body. Inflammation from the disease may be localized to one small part of the aorta, or may involve the entire length of the vessel. As the disease progresses, other major blood vessels may also become inflamed. The abdominal aorta, the part of the aorta that runs through the middle of the body and supplies blood to the stomach, intestines, and kidneys is involved in about 50% of patients. The inflammatory process causes the walls of the aorta to become thick, which makes it difficult for blood to flow easily. This may cause a wide variety of symptoms, depending upon which organs have their blood flow affected.

The cause of TA, and the reason that it develops in some people is not understood. Several studies suggest that genetic factors are involved. Nonetheless, the risk that more than one person in a family will develop TA is very small.

Symptoms: Symptoms that are common when TA first develops include lack of energy, weight loss, weakness and low-grade fever. Symptoms that affect the blood vessels take time to develop. As the disease progresses the arms and legs may become cool. Later, claudication or pain with use may develop. Other symptoms include joint pain, muscle weakness and pain, and skin lesions. If the vessels supplying blood to the heart or lungs become affected, people may develop chest pain, a myocardial infarction (heart attack). Anemia (low blood count) is present in most patients and results in a general feeling of weakness and fatigue.

Involvement of the major blood vessel that carries blood to the head and brain (the carotid artery) causes decreased blood flow that can lead to dizziness, fainting, headaches,seizures and difficulties thinking and remembering. Difficulty with seeing may also develop. Stomach pain, diarrhea, or ulcers can result from inflammation in the blood vessels supplying the stomach or intestines.

Diagnosis: Because the symptoms of TA may be so variable, the most importants step in diagnosis is considering the possibility of vasculitis. Physical examination often demonstrates decreased pulses in the arms or legs, and abdominal turbulence (bruits) in the neck or abdomen. Laboratory studies confirm the presence of inflammation. The next step is for doctors to visualize what is happening to the aorta and other blood vessels. Several different imaging methods are available. Radiography (x-ray) of the chest
may be used to look for changes in the aorta. Other types of
pictures show the actual flow of blood within the vessels, and
require drinking or injecting something called contrast material. This material travels with the blood and helps to outline location and appearance of vessels affected by the disease. Such procedures include angiography, computed tomographic (CT) or magnetic imaging resonance (MR) imaging scans of the chest, abdomen, head and neck, or other areas. Positron emission tomography (PET scanning) can also be used to take pictures of the aorta and other large vessels. More than one of these types of tests and pictures may be needed in order to diagnose and manage TA optimally.

Treatment: Medications used to treat TA include drugs that decrease inflammation or weaken the part of the immune system causing inflammation in the blood vessels. The main drug used to treat TA is corticosteroids, also known as glucocorticoids. Treatment with this medicine usually stops this disease from getting worse by reducing the inflammation associated with the disease. However, use of this medicine, especially for long periods of time, can cause many adverse reactions or side affects.

Approximately one-half of all patients with TA have disease that is ongoing and does not get completely better with the use of corticosteroids. When this is the case, other drugs such as methotrexate, cyclophosphamide, and azathioprine may reduce the inflammation and be helpful in controlling symptoms of the disease. A new drug that may be helpful for patients with TA who do not get better with the treatments listed above is called an anti-tumor necrosis factor alpha (anti-TNF) agent. More information about the role of this type drug in treating TA is needed, a large study is planned.

Several types of medical procedures may be used to help repair damaged blood vessels and to restore blood flow if major damage from the disease occurs. Angioplasty is a medical procedure in which a balloon is used to open narrowed or blocked blood vessels. A by-pass graft, in which blood is surgically redirected around a clogged or damaged vessel may also be done.

Prognosis: TA is a chronic disease. The degree of new disease involvement over time can be quite different between patients, and the inflammation may never completely go away. In some patients the disease eventually burns out and symptoms and inflammation do not ever return. Despite this, most people with TA may be treated effectively, and new and better medications continue to improve their prognosis.

Read stories about people with Takayasu's Disease.

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