Takayasu's arteritis (tah-kah-YAH-sooz ahr-tuh-RIE-tis) is a rare type of vasculitis, a group of disorders that cause blood vessel inflammation. In Takayasu's arteritis, the inflammation damages the aorta — the large artery that carries blood from your heart to the rest of your body — and its main branches.
The disease can lead to narrowed or blocked arteries, or to weakened artery walls that may bulge and tear (aneurysm). Takayasu's arteritis can also lead to arm or chest pain, high blood pressure, and eventually heart failure or stroke.
If you don't have symptoms, you may not need treatment for Takayasu's arteritis. But most people with the disease need medications to control the inflammation in the arteries and prevent complications. Even with treatment, relapses are common, and your symptoms may come and go.
Takayasu's arteritis is a form of vasculitis — inflammation of the blood vessels — that damages the large arteries, especially the aorta.
A normal heart has two upper and two lower chambers. The upper chambers, the right and left atria, receive incoming blood. The lower chambers, the more muscular right and left ventricles, pump blood out of your heart. The heart valves, which keep blood flowing in the right direction, are gates at the chamber openings.
The signs and symptoms of Takayasu's arteritis often occur in two stages.
In the first stage, you're likely to feel unwell with:
Not everyone has these early signs and symptoms. It's possible for inflammation to damage arteries for years before you realize something is wrong.
During the second stage, inflammation causes arteries to narrow so less blood and oxygen and fewer nutrients reach your organs and tissues. Stage 2 signs and symptoms may include:
Seek immediate medical attention for shortness of breath, chest or arm pain, or signs of a stroke, such as face drooping, limb weakness or speech disturbances.
Make an appointment with your doctor if you have other signs or symptoms that worry you. Early detection of Takayasu's arteritis is key to getting effective treatment.
If you've already been diagnosed with Takayasu's arteritis, keep in mind that your symptoms may come and go even with effective treatment. Pay attention to symptoms similar to those that occurred originally or to any new ones, and be sure to tell your doctor promptly.
With Takayasu's arteritis, the aorta and other major arteries, including those leading to your head and kidneys, become inflamed. Over time the inflammation causes changes in these arteries, including thickening, narrowing and scarring.
No one knows exactly what causes the initial inflammation in Takayasu's arteritis. The condition is likely an autoimmune disease in which your immune system attacks your own arteries by mistake. The disease may be triggered by a virus or other infection.
Takayasu's arteritis primarily affects girls and women younger than 40. The disorder occurs worldwide, but it's most common in Asia. Sometimes the condition runs in families.
With Takayasu's arteritis, extended or recurring cycles of inflammation and healing in the arteries might lead to one or more of the following complications:
A healthy pregnancy is possible for women with Takayasu's arteritis. But the disease and drugs used to treat it can affect your fertility and pregnancy. If you have Takayasu's arteritis and are planning on becoming pregnant, work with your doctor to develop a plan to limit complications of pregnancy before you conceive. See your doctor regularly during your pregnancy for checkups.
An aortic aneurysm occurs when a weak spot in the wall of your aorta begins to bulge (left). This can occur anywhere in your aorta. Having an aneurysm increases the risk of a tear in the lining of the aorta, shown in the image on the right.
Your doctor will ask you about your signs and symptoms, conduct a physical exam, and take your medical history. He or she may also have you undergo some of the following tests and procedures to help rule out other conditions that resemble Takayasu's arteritis and to confirm the diagnosis. Some of these tests may also be used to check on your progress during treatment.
Treatment of Takayasu's arteritis focuses on controlling the inflammation with medications and preventing further damage to your blood vessels. Takayasu's arteritis can sometimes be difficult to treat because even if your symptoms improve, the disease might still be active. In addition, by the time some people are diagnosed, it's possible that irreversible damage has already occurred.
On the other hand, if you don't have a lot of signs and symptoms or serious complications, you may not need treatment.
Talk with your doctor about the drug or drug combinations that are options for you and what the possible side effects are. Your doctor may prescribe:
Corticosteroids to control inflammation. The first line of treatment is usually with a corticosteroid, such as prednisone. Even if you soon start feeling better, you may need to continue taking the drug long term. After a few months, your doctor may gradually begin to lower the dose until you reach the lowest dose you need to control inflammation. Eventually your doctor may tell you to stop taking the medication completely.
Possible side effects of corticosteroids include weight gain, increased risk of infections, bone thinning (osteoporosis) and menstrual irregularities. To help prevent bone loss, your doctor may recommend a calcium supplement and vitamin D.
If your arteries become severely narrowed or blocked, you may need surgery to open or bypass these arteries and allow an uninterrupted flow of blood. Often this helps to improve certain symptoms, such as high blood pressure and chest pain.
In some cases, though, narrowing or blockage may recur, requiring a second procedure. Also, if you develop large aneurysms, surgery may be needed to prevent them from rupturing. Your doctor may suggest putting off surgery until your condition is in remission.
Surgical options, which are best performed when inflammation of the arteries has been reduced, include:
One of the greatest challenges of living with Takayasu's arteritis may be coping with side effects of your medication. The following suggestions may help:
Eat a healthy diet. Eating well can help prevent potential problems that can result from your condition and medications, such as high blood pressure, thinning bones and diabetes. Emphasize fresh fruits and vegetables, whole grains, and lean meats and fish, while limiting salt, sugar and alcohol.
If you're taking a corticosteroid drug, ask your doctor if you need to take a vitamin D or calcium supplement.
If your primary care doctor suspects that you have Takayasu's arteritis, he or she may refer you to one or more specialists with experience in helping people with this condition. Takayasu's arteritis is a rare disorder that can be difficult to diagnose and treat.
You may want to talk with your doctor about a referral to a medical center that specializes in treating vasculitis.
Because appointments can be brief and because there's often a lot of information to discuss, it's a good idea to be prepared. Here's some information to help you get ready for your appointment.
For Takayasu's arteritis, some basic questions to ask include:
Your doctor will likely ask you a number of questions, such as: