(Pulseless Disease; Occlusive Thromboaortopathy; Aortic Arch Syndrome; Takayasu's Arteritis)
(See Overview of Vasculitis.)
Takayasu arteritis causes chronic blood vessel inflammation, mainly of the aorta (the artery that connects directly with the heart), the arteries that branch off from it, and the pulmonary arteries.
The cause is unknown.
People may have general symptoms, such as fever or muscle or joint aches, followed by various symptoms, depending on which organs are affected.
An imaging test of the aorta, such as computed tomography angiography or magnetic resonance angiography, is done to confirm the diagnosis.
A corticosteroid and sometimes another drug that suppresses the immune system can usually control the inflammation.
Takayasu arteritis is rare. It is more common among Asians but occurs worldwide. It affects mostly women aged 15 to 30. Its cause is unknown.
The aorta and its branches, including arteries that take blood to the head and the kidneys, become inflamed. In about half of people, the pulmonary arteries are also affected. Inflammation may cause sections of these arteries to become narrow or blocked. The walls of the aorta or its branches may thicken, resulting in a bulge (aneurysm). The affected arteries cannot provide enough blood to the tissues they supply.
Takayasu arteritis is a chronic disorder with symptoms that fluctuate in severity.
Sometimes the disorder begins with fevers, muscle and joint aches, loss of appetite, weight loss, and night sweats. But usually, symptoms occur when an artery narrows, reducing blood flow to part of the body, as in the following:
Arms or legs: The arms ache and tire easily when repetitive arm movements are done or the arms are elevated for a long period of time. When walking, people may feel pain, usually in the calves—a symptom called claudication. The pulse and blood pressure may be decreased in one or both arms or legs.
Head: People may feel dizzy or faint, have headaches, or have problems with vision. Less often, a stroke results.
Heart: Sometimes blood flow to the heart is reduced, and angina or a heart attack results.
Lungs: Blood pressure in the lungs (pulmonary hypertension) becomes very high. People feel short of breath, tire easily, and may have chest pain.
Some people do not have any symptoms. In other people, the disorder progresses, causing serious complications such as strokes, heart failure, heart attacks, kidney failure, and aneurysms.
Doctors suspect the disorder based on the following, especially in young women:
Blood pressure cannot be measured in one or both arms.
Blood pressure is much higher or the pulse is much stronger in one arm or leg than in the other.
Blood pressure is unexpectedly high.
A person has a disorder such as stroke, angina, heart attack, or kidney damage that has no apparent explanation and that is unexpected.
Doctors ask about symptoms, review the person’s medical history, and do a complete physical examination to exclude other disorders that may cause similar symptoms. Doctors measure blood pressure in both arms and both legs. Arms and legs that have lower blood pressure may have narrowed arteries. To get an accurate blood pressure measurement, doctors measure blood pressure in a limb where arteries have not been narrowed by the disease.
Blood tests and urine tests are done. They cannot identify the disorder but may confirm that inflammation is present.
To confirm the diagnosis, doctors may use conventional angiography (also called arteriography), magnetic resonance, or computed tomography (CT) angiography—to evaluate the aorta and its branches. For conventional or CT angiography, a contrast agent that can be seen on x-rays (radiopaque contrast agent) is injected into blood vessels to outline them. Then x-rays are taken. Magnetic resonance angiography does not require the injection of a dye. These procedures can detect aneurysms and show where the arteries are narrowed and where the vessel walls are thickened.
After Takayasu arteritis is diagnosed, regular doctor visits should be scheduled so that the doctor can check whether the disorder is progressing.
For 20% of people, the disorder occurs once and does not return. For the rest, the disorder goes away and returns or is chronic and progressively worsens. Even when symptoms and laboratory test abnormalities suggest the disorder is not active, new symptoms occur or an abnormality is seen during imaging studies. The prognosis is poorer for people whose disorder is progressively worsening and who have complications (such as high blood pressure, heart failure, or aneurysms).
Corticosteroids (such as prednisone) are usually used. They effectively reduce inflammation in most people. Sometimes another drug that suppresses the immune system (immunosuppressant), such as azathioprine, cyclophosphamide, mycophenolate mofetil, or methotrexate, is also used. Tumor necrosis factor inhibitors, such as infliximab and etanercept, may also be effective. However, drugs cannot control symptoms in about one fourth of people.
How long drugs should be given has not been determined. The dose of the corticosteroid is gradually reduced, and the drug is eventually stopped because these drugs, especially when used for a long time, can have serious side effects. When drugs are stopped, symptoms return in about one half of people, so the drugs may need to be restarted.
High blood pressure must be controlled to prevent complications (see High Blood Pressure : Treatment). Angiotensin-converting enzyme (ACE) inhibitors are often used. Taking a low dose of aspirin is usually recommended to help decrease the risk of clotting in the inflamed artery, which can lead to blockages. If an artery that supplies the heart is blocked, a heart attack can result.
If people have difficulty using their arms or walking, bypass surgery may be done to restore the blood flow to the affected limb. Other procedures to restore blood flow (such as coronary artery bypass surgery or percutaneous transluminal coronary angioplasty) may be needed, depending on the symptoms, but may not work as well as bypass surgery.