Takayasu's arteritis
- Reviewed by Robert H. Shmerling, MD, Senior Faculty Editor, Harvard Health Publishing; Editorial Advisory Board Member, Harvard Health Publishing
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. Researchers have identified a number of genes that appear to increase the risk of developing this disease.
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 two or three of every one million people every year. It is eight times more common in females than in males. Although 75% 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% 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 six 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.
Other criteria have been developed for research purposes but have not been well studied in clinical settings.
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).
In some cases, positron emission tomography (PET), in combination with CT (PET-CT) or MR (PET-MR), may be helpful. When surgery is performed (for example, to repair an aneurysm), the pathology specimen obtained during surgery can help secure the diagnosis.
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 or if there is a high risk of steroid-related complications, another immunosuppressive drug, such as tocilizumab (Actemra), etanercept (Enbrel), or infliximab (Remicade), may be added. Other treatment options include methotrexate (Folex, Methotrexate, and Rheumatrex), azathioprine (Imuran), mycophenolate (CellCept), leflunomide (Arava), or cyclophosphamide (Cytoxan, Neosar). 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, there are several ways this can be accomplished, including:
- 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% 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% to 80% success rate. But, it's common for symptoms to return again. Overall, about 80% survive at least 15 years following the diagnosis, but the prognosis varies depending on whether there are complications (such as the development of aneurysms). The prognosis may be improving due to newer treatment options.
Additional info
American College of Rheumatology
https://www.rheumatology.org/
About the Reviewer
Robert H. Shmerling, MD, Senior Faculty Editor, Harvard Health Publishing; Editorial Advisory Board Member, Harvard Health Publishing
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