DRUG INDEX
Takayasu Disease
(Takayasu Arteritis)
What is Takayasu disease?
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.
What are symptoms of Takayasu disease?
The inflammation of the aorta and its branch arteries can lead to poor blood supply to tissues of the body in patients with Takayasu disease. This can cause painful, cool or blanched extremities, dizziness , headaches, chest pain, and abdominal pain . Other early symptoms can include fatigue, weight loss, and low-grade fever.
How is Takayasu disease diagnosed?
The doctor can find elevated blood pressure in over half of the patients with Takayasu disease. Abnormal sounds of blood movement through blood vessels can sometimes be heard with a stethoscope. Small notches in the ribs of the back of the chest have sometimes been detected on routine chest x- rays and are felt to be a result of abnormal pulsations of blood vessels in these areas.
The diagnosis is supported by the blood tests, such as a sedimentation rate (sed rate), that suggest inflammation in the body. In fact, elevated blood pressure with an elevated sedimentation rate is distinctly uncommon in children and very helpful in suggesting Takayasu disease as a possible cause. Anemia (low red blood cell count) is frequent.
Takayasu arteritis is ultimately diagnosed with an angiogram of the arteries (arteriogram) whereby a contrast material is injected into the blood vessels which makes them visible by x-ray. With the arteriogram the doctor can visualize the abnormally narrowed and constricted arteries. Recent reports have shown successful diagnosis of Takayasu disease using magnetic resonance angiography (MRA). MRA, the combination of an MRI scan with angiogram, could be used as a non-invasive method of diagnosing patients with this illness.
How is Takayasu disease treated?
Takayasu disease is treated by suppressing the inflammation with cortisone medication. While most patients can improve, some do not or relapse. In cortisone-resistant patients stronger medications which suppress the immune system (immunosuppressive drugs), thereby further decreasing active inflammation of the arteries, have been used. Examples include prednisone , prednisolone , methotrexate (Rheumatrex, Trexall), cyclosporine, cyclophosphamide (Cytoxan), and azathioprine (Imuran). Strict control of elevated blood pressure (hypertension) is important.
What is the longterm outlook for patients with Takayasu disease?
The long-term prognosis is not predictable. Some researchers have found that the prognosis is worse when the retina of the eyes are affected or if the aorta is damaged or when aneurysms develop.
The effects of Takayasu arteritis vary greatly from patient to patient. These effects frequently depend on the impaired blood supply to body tissues (such as the brain leading to strokes or spinal cord leading to paralysis).
Medical Author: William C. Shiel Jr., MD, FACP, FACR
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