Mayo Clinic | American Hospital

Takayasu arteritis (tah-kah-YAH-soo ahr-tuh-RIE-tis) is a rare type of vasculitis. Vasculitis is a group of conditions that causes blood vessel swelling, called inflammation. In Takayasu arteritis, the swelling damages the large artery that carries blood from the heart to the rest of the body, called the aorta, and its main branches.

The condition can narrow or block arteries. The artery walls may bulge and tear, called an aneurysm. It also can cause arm or chest pain and high blood pressure. In time, Takayasu arteritis can cause heart failure or stroke.

People who don't have symptoms may not need treatment. But most people with the condition need medicines to control inflammation in the arteries and prevent complications. Even with treatment, the condition often comes back. And symptoms may come and go.

Overview

Symptoms

The symptoms of Takayasu arteritis often come in two stages.

Stage 1

In the first stage, you're likely to feel not well. Symptoms may include:

Tiredness.
Weight loss.
Muscle and joint aches and pains.
Mild fever, sometimes with night sweats.

Not everyone has these early symptoms. Inflammation can harm arteries for years before symptoms begin.

Stage 2

During the second stage, the swelling causes arteries to narrow. Less blood and oxygen reach the organs and tissues. Stage 2 symptoms may include:

Weakness or pain in the limbs with use.
A weak pulse, trouble getting a blood pressure reading or having a different blood pressure in each arm.
Lightheadedness, dizziness or fainting.
Headaches or changes in vision.
Issues with memory or thinking.
Chest pain or shortness of breath.
High blood pressure.
Diarrhea or blood in stool.
Too few red blood cells, called anemia.

When to see a doctor

Seek medical help right away for shortness of breath, chest or arm pain, or signs of a stroke. Signs of a stroke include face drooping, arm weakness or having trouble speaking.

Make an appointment with your healthcare professional if you have symptoms that worry you. Finding Takayasu arteritis early is key to treating it.

If you've been diagnosed with Takayasu arteritis, your symptoms may come and go even with treatment. Watch for symptoms you had at the start of the condition or any new ones. Tell your healthcare professional right away about any changes.

Causes

No one knows exactly what causes the first swelling, called inflammation, in Takayasu arteritis. It's likely an autoimmune condition. This means the immune system attacks the arteries by mistake. A virus or other infection may set off the condition.

Risk factors

Takayasu arteritis mainly affects people assigned female at birth who are younger than 40. The condition happens around the world. But it's most common in Asia.

Sometimes the condition runs in families. Researchers have found certain genes that are linked with Takayasu arteritis.

Complications

With Takayasu arteritis, cycles of swelling and healing in the arteries might lead to one or more of these complications:

Hard and narrow blood vessels. This can lower blood flow to organs and tissues.
High blood pressure. This most often is due to less blood flow to the kidneys.
Inflammation of the heart. This may harm the heart muscle or the heart valves.
Heart failure. This may be caused by high blood pressure, inflammation of the heart, an aortic valve that allows blood to leak back into the heart or a mix of these.
Stroke. Lower or blocked blood flow in the arteries to the brain can cause this.
Transient ischemic attack. Also called a TIA or ministroke, a transient ischemic attack is a warning. It causes symptoms like those of a stroke. But it doesn't cause long-term damage.
A bulge in the aorta, called an aneurysm. This happens when the walls of the blood vessel weaken and stretch. This forms a bulge that can be life-threatening if it breaks.
Heart attack. This may happen as a result of less blood flow to the heart.

Pregnancy

People with Takayasu arteritis can have a healthy pregnancy. But the condition and medicines that treat it can affect being able to get pregnant and the pregnancy.

If you have Takayasu arteritis and plan to get pregnant, work with your healthcare professional before you get pregnant to make a plan to limit complications of pregnancy. See your healthcare team regularly during your pregnancy.

Diagnosis

Your healthcare professional asks you about your symptoms, does a physical exam, and takes your medical history. You also may have some tests and procedures. They can help confirm the diagnosis and rule out other conditions that are like Takayasu arteritis. Some of these tests also may be used to check your progress during treatment.

Tests and procedures might include:

Blood tests. These tests can be used to look for signs of swelling, called inflammation. They also can check for too few red blood cells, called anemia.
X-rays of your blood vessels, also called angiography. During an angiogram, a healthcare professional puts a long, flexible tube called a catheter into a large artery or vein. A special contrast dye goes into the catheter. Then you have X-rays taken as the dye fills your arteries or veins.

The images show if blood is flowing as it should or if it's being slowed or blocked. The blockage is due to a narrowed blood vessel, called stenosis. People with Takayasu arteritis most often have a few areas of stenosis.
MRA. This is a less invasive form of angiography. It makes detailed images of the blood vessels without the use of catheters or X-rays.

MRA uses radio waves in a strong magnetic field to make detailed images of tissue slices. During this test, a contrast dye put into a vein or artery helps your healthcare professional see the blood vessels better.
Computerized tomography (CT) angiography. This is another noninvasive form of angiography. It combines a computer review of X-ray images with a contrast dye that's put into a vein or artery. This lets your healthcare professional check your aorta and its nearby branches and watch how the blood flows.
Ultrasonography. Doppler ultrasound is an advanced version of the common ultrasound. It can make detailed pictures of the walls of certain arteries, such as those in the neck and shoulder. It may detect slight changes in these arteries earlier than other imaging tests can.
Positron emission tomography. Healthcare professionals often use this imaging test with computerized tomography or magnetic resonance imaging. Also called a PET scan, this test can show how much swelling there is in the blood vessels. A radioactive medicine put into a vein or an artery makes it easier to see areas of less blood flow.

Treatment

Treatment of Takayasu arteritis aims at controlling inflammation with medicines and preventing more damage to the blood vessels.

Takayasu arteritis can be hard to treat. The condition can stay active even if symptoms improve. It's also possible that you already have damage that can't be fixed by the time you're diagnosed.

If you don't have symptoms or serious complications, you may not need treatment. Or your healthcare professional may have you taper and stop treatment over time.

Medicines

Talk with your healthcare professional about what medicines you might take and their possible side effects. Medicines may include:

Corticosteroids to control inflammation. The first line of treatment most often is a corticosteroid, such as prednisone (Rayos). Even after you feel better, you may need to keep taking the medicine long term.

After a few months, your healthcare professional may begin to lower the dose little by little until you reach the lowest dose you need to control inflammation. In time, you might be able to stop taking the medicine.

Possible side effects of corticosteroids include weight gain, increased risk of infection and bone thinning. To help prevent bone loss, you might take a calcium supplement and vitamin D.
Other medicines that lower immune system responses. If your condition doesn't respond well to corticosteroids or you have trouble as you lower the dose, your healthcare professional may prescribe medicines such as methotrexate (Trexall, Xatmep, others), azathioprine (Azasan, Imuran) and leflunomide (Arava).

Some people respond well to medicines made for people who get organ transplants. Mycophenolate (CellCept, Myhibbin) is such a medicine. The most common side effect is a greater risk of infection.
Medicines to regulate the immune system. If you don't respond to standard treatments, your healthcare professional may suggest medicines called biologics that affect the immune system. Biologics include etanercept (Enbrel), infliximab (Remicade) and tocilizumab (Actemra).

The most common side effect with these drugs is a higher risk of infection. More research is needed on these medicines for treatment of Takayasu arteritis.

Surgery

For very narrow arteries, surgery can open or go around, called bypass, these arteries to allow blood to flow better. Often this helps improve certain symptoms, such as high blood pressure and chest pain. But sometimes, the narrowing or blockage may happen again. This needs a second procedure.

Also, surgery may prevent large aneurysms from tearing.

It's best to have surgery after the swelling in the arteries has gone down. Surgical options include:

Bypass surgery. In this procedure, an artery or a vein is removed from another part of the body and attached to the blocked artery. This gives blood a bypass to flow through. Surgeons most often do bypass surgery when the narrowed artery can't be fixed or when blood flow is very blocked.
Blood vessel widening, called percutaneous angioplasty. Surgeons may use this procedure if the arteries are very blocked. During percutaneous angioplasty, a surgeon puts a tiny balloon through a blood vessel into the affected artery. Once in place, the surgeon expands the balloon to widen the blocked area. Then the surgeon deflates the balloon and removes it.
Aortic valve surgery. For an aortic valve that is leaking greatly, a surgeon may repair or replace the valve.

Coping and support

One of the hardest things about living with Takayasu arteritis may be coping with side effects of medicine. The following tips may help:

Know about your condition. Learn all you can about Takayasu arteritis and its treatment. Know the possible side effects of the medicines you take. Tell your healthcare professional about any changes in your health. Ask if you should take low-dose aspirin daily.
Eat healthy food. Eating well can help prevent issues that your condition and medicines can cause. These include high blood pressure, thinning bones and diabetes. Eat plenty of fresh fruits and vegetables, whole grains, and lean meats and fish. Limit salt, sugar and alcohol.

If you take a corticosteroid medicine, ask your healthcare professional if you need to take a vitamin D or calcium supplement.
Exercise regularly. Regular aerobic exercise, such as walking, can help prevent bone loss, high blood pressure and diabetes. It also helps your heart and lungs. And many people find that exercise improves their mood and overall sense of well-being.
Don't use tobacco products. Stop using all forms of tobacco to lower the risk of injuring your blood vessels and tissues even more.
Stay up to date on vaccines. Your medicines may make it harder for your body to fight infection. Talk with your healthcare professional about getting vaccinated against the flu, pneumonia, shingles and other diseases.

Preparing for your appointment

For Takayasu arteritis, your main healthcare professional may send you to one or more specialists in helping people with this condition. Takayasu arteritis is a rare condition that can be hard to diagnose and treat.

You may want to talk with your healthcare professional about sending you to a medical center that specializes in treating vasculitis.

Here's some information to help you get ready for your appointment.

What you can do

When you make the appointment, ask if you need to do anything before it, such as restrict your diet. Ask a family member or friend to go with you to help you keep track of what you learn during your visit.

Make a list of:

All your symptoms, and when they began.
Key personal information, including major stresses and recent life changes.
All medicines, vitamins and supplements you take, including doses.
Questions to ask during your visit.

For Takayasu arteritis, some questions to ask include:

What's the most likely cause of my symptoms?
What are other possible causes for my symptoms?
What tests do I need? How do I prepare for them?
Is my condition likely to be brief or long lasting?
What treatments do you advise?
I have another medical condition. How can I best manage these conditions together?
Do you have any brochures or other printed material that I can have? What websites do you suggest?