Takayasu's Arteritis

What is Takayasu’s arteritis?

Takayasu's arteritis (also called Takayasu disease, pulseless disease or TAK) is an uncommon form of vasculitis or inflammation in your blood vessels. It affects your biggest arteries, like your aorta (the main blood vessel that leaves your heart) and arteries that branch off of your aorta and bring oxygenated blood from your heart to your body tissues.

Affected arteries include the ones that supply blood to your arms, and travel through your neck to provide blood to your brain.

With Takayasu’s arteritis, inflammation damages your arteries, making parts of them weak and stretched out. This can lead to an aneurysm. Your arteries can also become inflamed and narrowed, resulting in restricted blood flow. Also, blood vessels can become completely blocked (called an occlusion). Less commonly, arteries that provide blood flow to your heart, intestines, kidneys and legs may be involved.

Who does Takayasu’s arteritis affect?

TAK often affects children and people who are between the ages of 20 to 40 and assigned female at birth. At diagnosis, people with Takayasu’s arteritis are often between 15 and 35 years old. About 80% to 90% of people with this condition were assigned female at birth.

Healthcare providers see Takayasu’s arteritis more often in Japan and in people of Mexican, Indian and East Asian descent.

Every year in the United States, providers diagnose 2 to 3 new cases of TAK per 1 million Americans.

How does Takayasu’s arteritis affect my body?

Narrowed vessels cause decreased blood flow to the areas they supply with blood.

The changes that happen in TAK are often gradual, allowing alternate routes of blood flow to develop. These alternate routes are often smaller "side roads" that may or may not be able to carry as much blood as the original arteries did.

In general, blood flow beyond an area of narrowing is almost always enough to allow tissues to survive. In rare cases, if there aren’t enough alternate blood vessels, tissue that doesn’t get blood and oxygen will die.

What are the symptoms?

Some people with TAK may not have any symptoms, but about half of all people who have TAK will have a sense of generalized illness.

Early-stage or first-stage symptoms of Takayasu’s arteritis include:

• Fever.
• Fatigue.
• Pain in your muscles or joints.
• Not much desire to eat.

Later-stage or second-stage symptoms of Takayasu’s arteritis include:

• Chest pain.
• Shortness of breath.
• Headaches.
• Dizziness.

Narrowing of blood vessels and reduced blood supply can cause:

• Fatigue, pain, or aching in your arms and legs.
• Belly pain from decreased blood flow to your intestines.
• High blood pressure from decreased blood flow to your kidneys.
• Stroke (rarely).
• Heart attack (rarely.)

What causes Takayasu’s arteritis?

The exact cause of TAK is unknown. Takayasu’s arteritis may be an autoimmune condition, which means your immune system is harming healthy tissue that it should leave alone.

Researchers think some cases of Takayasu’s arteritis may come from a gene you get from both of your parents. They may not have symptoms because they’re just carrying one copy of the gene. When you inherit that specific gene from both parents, you can get the condition and its symptoms.

How is Takayasu’s arteritis diagnosed?

Your healthcare provider’s diagnosis of Takayasu’s arteritis is based on a combination of factors, including:

• Complete medical history and physical exam to exclude other illnesses that may have similar symptoms. Your provider may hear an unusual sound called a bruit through their stethoscope. If you have a lot of narrowing in your blood vessels, it can be hard for blood to move through. This creates that sound.
• X-rays that show where your artery has damage and the severity of it.
• Procedures to detect blood vessel narrowing or aneurysm.

What tests will be done to diagnose Takayasu’s arteritis?

Several tests can help your healthcare provider make a diagnosis, including:

• Magnetic resonance imaging (MRI): a test that produces images without using X-rays. It uses a large magnet, electromagnetic energy waves and a computer to produce these images.
• Computed tomography (CT scan): X-rays and computers produce images of internal organs, including large blood vessels.
• Angiography: X-ray pictures of the inside of your blood vessels. Your provider inserts a long, slender tube called a catheter into a large artery in your groin area or arm and threads it through to the artery they want to see. They use the catheter to inject contrast material into your blood vessel, and then take X-rays. The contrast agent makes the blood vessels show up on the X-ray pictures. Your provider may also use an MRI for angiography.
• Positron emission tomography (PET): Your provider gives you a radioactive substance through a needle in your arm. This substance travels through your body, and a scanner detects it and creates images.
• Ultrasound: Sound waves help create images of your blood vessels.

How is Takayasu’s arteritis treated?

Medicines can help control inflammation, but some people may need surgery to create a way around a blocked artery.

Corticosteroids like prednisone (Rayos® or Sterapred®) or prednisolone (Flo-Pred® or Orapred®) are the most common treatments for TAK.

Benefits of corticosteroids:

• Often dramatically effective in bringing about remission (the absence of active blood vessel inflammation).
• Your healthcare provider can slowly reduce the dose of prednisone to the lowest effective amount to minimize side effects. Some people can gradually discontinue medication without symptoms returning.

Drawbacks of corticosteroids:

• Only partially effective for some people.
• More than half of all people taking it will have symptoms that come back or illness that progresses.

Usually, providers also prescribe immunosuppressive medications such as:

• Methotrexate (Rheumatrex® or Trexall®).
• Azathioprine (Imuran® or Azasan®).
• Mycophenolate (Cellcept® or Myfortic®).
• Leflunomide.
• Cyclophosphamide.

Taking immunosuppressive drugs in addition to prednisone can help 50% of people who previously relapsed get rid of their symptoms and gradually stop taking prednisone.

Some people may also take tumor-necrosis factor (TNF) inhibitors to fight inflammation. This class of medicines includes:

• Etanercept.
• Infliximab.
• Tocilizumab.

Overall, about 25% of people who have Takayasu’s arteritis won’t be able to manage their disease completely without continued use of medication.

Side effects of the treatment

Medicines for treating Takayasu’s arteritis can have serious side effects, but other medicines can help with these side effects.

Side effects may include:

• Being less able to fight an infection.
• Losing bone mass.
• Having a hard time seeing.
• Having seizures.

How soon after treatment will I feel better?

Corticosteroids work within hours after you receive your first dose.

What treatments are used?

In some instances, narrowing of arteries to your kidney may cause hypertension (high blood pressure). Your provider can stretch narrow vessel openings with a balloon (angioplasty) or do a bypass operation to restore normal flow to your kidney. This may result in normal blood pressure without the need for antihypertensive medications.

Some people may have serious mobility issues because of narrowed blood vessels in their arms or legs. Bypass operations may correct these problems. A surgical repair or bypass may correct an aneurysm.

Some people with Takayasu’s arteritis may need an aortic valve replacement.

How to take care of myself

Many people with Takayasu’s arteritis have high blood pressure (hypertension), which is important to manage. If you don’t treat your high blood pressure, you could have:

• Stroke.
• Heart disease.
• Kidney failure.

If you’re taking medicine that holds back your immune system, consider vaccinations that can protect you from an infection, such as:

• Flu.
• Pneumonia.
• Shingles.

What can I expect if I have Takayasu’s arteritis?

Although there is no cure for Takayasu’s arteritis, it is a treatable disease. Most people who have it improve with treatment. However, for many people, TAK can be partially, or less often, completely disabling. The effects of illness on function may be significant.

About half of people with Takayasu’s arteritis have to modify their jobs. But about 25% of people with this condition have an entirely normal lifestyle. Another 25% have had to make some adjustments in their activities.

People who live long lives with this chronic illness need to have regular checkups and adjustments to their medications if needed. Providers need to monitor side effects from medications and do blood tests.

Complications of Takayasu’s arteritis may include:

• Heart failure.
• Stroke.
• Blood clots.
• Heart attack.

How long Takayasu’s arteritis lasts

Takayasu’s arteritis is a chronic disease that progresses slowly. You’ll need long-term treatment, most likely with medicine.

Outlook for Takayasu’s arteritis

In the United States and Japan, only about 3% of people with Takayasu's arteritis die after having the disease for an average period of five years. This may be due to healthcare providers recognizing the disease and treating it correctly. Other parts of the world may have worse outcomes because of delayed diagnosis and treatment or poor access to care. Without treatment, TAK can be fatal.

How do I take care of myself?

Being aware of how you’re feeling and staying in touch with your healthcare provider can help you catch any symptoms that are just starting or are getting worse.

When should I see my healthcare provider?

Contact your provider if your symptoms of Takayasu’s arteritis come back during or after treatment.

Talk with your healthcare provider if you’re planning a pregnancy. It’s possible to have a successful pregnancy with Takayasu’s arteritis, but it’s important to know how to manage it.

When should I go to the ER?

Call 911 if you have:

• Chest pain.
• Difficulty breathing.
• A weak pulse.
• Signs of a heart attack.
• Signs of a stroke.

What questions should I ask my doctor?

• How long will I need to take medication for Takayasu’s arteritis?
• How often do I need to go to follow-up appointments?
• Can I manage my condition without surgery?
• How often do I need to have imaging tests ?

A note from Cleveland Clinic

Because Takayasu’s arteritis is a chronic disease, you’ll need regular checkups with your healthcare provider. Over time, you’re likely to have times when your symptoms are under control and other times when they’re not. This is why having a provider to follow up with consistently is important. Don’t be afraid to say something if you notice something that’s different about your health. Your provider will want to know about it so they can help you.