Patient Basics: Takayasu’s Arteritis

Originally published by Harvard Health.

What Is It?

Takayasu’s arteritis is a chronic (long-term) disease in which arteries become inflamed. It is also known as Takayasu’s aortitis, pulseless disease and aortic arch syndrome. The name comes from the doctor who first reported the problem in 1905, Dr. Mikito Takayasu.

In most cases, Takayasu’s arteritis targets the aorta and its major branches, including arteries to the brain, arms and kidneys. The aorta is the body’s main artery, which pumps oxygen-rich blood from the heart to the rest of the body. Less frequently, the pulmonary artery and coronary arteries also are involved. This problem causes damage to the body’s major organs; reduced or absent pulses in the arms and legs; and symptoms of poor circulation, such as a cool or cold arm or leg, muscle pains with use or exertion, or symptoms of stroke if brain arteries are narrowed or blocked.  Over time, Takayasu’s arteritis can cause scarring, narrowing and abnormal ballooning of involved blood vessels. The disease can be fatal.

Although the cause of Takayasu’s arteritis is unknown, it may be an autoimmune disorder, meaning that immune defenses attack the body’s own cells rather than protecting them from outside invaders.

In Asia, Takayasu’s arteritis is a major cause of high blood pressure in teenagers and young adults. In North America and Europe, however, Takayasu’s arteritis is a very rare illness that affects only 2 or 3 of every 1 million people every year. It is 8 times more common in females than in males. Although 75 percent of people begin to have symptoms during their teenage years, the average age at which the problem is diagnosed is usually much later at age 29. This is because early symptoms of Takayasu’s arteritis are more general and common, such as fatigue. Up to 96 percent of people are not diagnosed until they have later, more specific symptoms.

Symptoms

The symptoms of Takayasu’s arteritis vary depending on the stage of the illness and the arteries affected. Early symptoms can include fever, fatigue, poor appetite, weight loss, night sweats, joint pain and chest pain. Later symptoms typically are related to narrowing of the arteries and a decrease in blood flow to certain organs. These later symptoms can include:

• Headache, nausea or fatigue related to high blood pressure and kidney failure
• Chest pain, especially with exertion
• Shortness of breath and fatigue, resulting from congestive heart failure
• Fainting, dizziness, changes in vision, transient ischemic attacks (mini strokes) or a stroke, resulting from poor blood flow to the brain
• Abdominal pain, nausea and vomiting, resulting from poor blood flow to the abdomen
• Muscle weakness and pain in the arms or legs when they are moved

Diagnosis

Because Takayasu’s arteritis is so rare and because the early symptoms are not specific, a doctor may not suspect you have the disease until you develop symptoms of narrowed arteries. This could be months, or even years, after the disease started.

The earliest clues that you may have Takayasu’s arteritis may be high blood pressure that can’t be explained by anything else, difficulty obtaining a blood pressure measurement on one side, a difference in blood pressure reading between the two arms or symptoms of poor circulation – all of these are unusual findings in a younger person.

Your doctor may suspect that you have Takayasu’s arteritis if you have at least three of the 6 criteria developed by the American College of Rheumatology to distinguish it from other forms of vasculitis:

• Symptoms beginning at age 40 or younger
• Muscle weakness and pain when you move your arms or legs
• A weak pulse in the brachial artery (major artery in the upper arm)
• A sound called a bruit, which indicates turbulent blood flow, when your doctor listens to your aorta or subclavian artery with a stethoscope
• Signs of damage to the aorta or other arteries on a test called an angiogram, which is a series of X-rays of arteries
• A difference of at least a 10 mm Hg (millimeters of mercury) in the systolic blood pressure (the first number in your blood pressure reading) in your right and left arms

To confirm the diagnosis, your doctor will review your symptoms and medical history, and examine you, paying special attention to your heart and blood vessels. He or she will measure the blood pressure in your arms and legs and check the strength of pulses in your extremities and neck. To check for bruits in narrowed vessels, your doctor will use a stethoscope to listen to specific areas of your chest, neck and abdomen.

After the physical examination, your doctor may order blood tests. Also, to identify areas where arteries are damaged, your doctor may request one or more of the following studies: standard angiography, magnetic resonance angiography (MRA), Doppler ultrasound, standard magnetic resonance imaging (MRI) or computed tomography (CT).

Expected Duration

How long Takayasu’s arteritis lasts varies. In many cases, the active illness lasts for years, causing continuing inflammation and ongoing damage to blood vessels. If symptoms go away, either on their own or after treatment, they can return later. Damage to arteries may be permanent. Even when Takayasu’s arteritis itself is no longer active, narrowed arteries may continue to disrupt blood flow to organs.

Prevention

There is no way to prevent Takayasu’s arteritis.

Treatment

Doctors usually begin treatment for Takayasu’s arteritis with glucocorticoid medications, such as prednisone. If these medications alone do not work well, then stronger immunosuppressive drugs may be added, including methotrexate (Folex, Methotrexate, and Rheumatrex), azathioprine (Imuran), mycophenolate (CellCept), leflunomide (Arava) or cyclophosphamide (Cytoxan, Neosar). Recent studies suggest that injectable adalimumab (Humira), etanercept (Enbrel) or infliximab (Remicade) may be helpful to control the vascular inflammation of Takayasu’s arteritis when other medications fail. Blood pressure medications also may be necessary to control high blood pressure.

If Takayasu’s arteritis has caused significant narrowing of an artery, the narrowed segment may need to be widened or repaired. Depending on the artery affected, this can be done in one of three ways:

• Traditional (open) surgery
• Percutaneous transluminal balloon angioplasty, in which a small balloon is inserted into the blood vessel, then inflated to dilate it
• Percutaneous placement of a stent, in which a small mesh device is placed in the artery to keep the blood vessel open

Inflamed segments of arteries may become weakened, leading to the formation of an aneurysm, or outpouching of the vessel. These may expand over time and rupture, a potentially life-threatening event; therefore, tests for aneurysm (including MRA) may be recommended for people with Takayasu’s arteritis, and if an aneurysm is large or expanding, surgical repair may be recommended.

When To Call a Professional

Call your doctor if you develop any severe pain, coldness or a blue discoloration in an arm or leg, chest pain, or symptoms of stroke.

Prognosis

Symptoms resolve in about 60 percent of people with Takayasu’s arteritis when they are treated with glucocorticoids alone. However, symptoms return in about half of these patients. When symptoms return, retreatment with a combination of glucocorticoids and other immunosuppressive drugs has a 40 percent to 80 percent success rate.  But, it’s common for symptoms to return again. Overall, about 80 percent to 90 percent of people with Takayasu’s arteritis respond to some form of medical therapy.

In general, about 85 percent of people survive for at least 15 years after diagnosis. This figure drops to 70 percent for those with severe high blood pressure or significant damage to the aorta.

Additional Info

American College of Rheumatology
1800 Century Place
Suite 250
Atlanta, GA 30345-4300
Phone: 404-633-3777
Fax: 404-633-1870
http://www.rheumatology.org/