What is Takayasu arteritis?

Takayasu arteritis is a rare, ongoing (chronic) autoimmune disease. It causes redness and swelling (inflammation) of the large arteries. It’s a rare disease that’s much more common in young women. It is also believed to be more common in people from East Asia, India, and South America.

An autoimmune disease is caused by a problem with the immune system. The immune system’s job is to protect the body from disease. It does this by attacking things in the body, such as viruses, that may cause harm. When you have an autoimmune disease, your immune system attacks your own body. With Takayasu arteritis, the immune system attacks the walls of arteries.

Arteries are blood vessels that carry blood throughout your body. Arteries send blood from the heart out to the body. Takayasu arteritis mostly affects the aorta and its main branches. The aorta is the large artery that leads from the heart. When your immune system attacks your arteries, it causes inflammation. This can damage your arteries.

What causes Takayasu arteritis?

Researchers are working to understand what causes the disease. An infection by a virus or bacteria may cause this condition if you have a weak immune system. Genes may be part of the cause.

What are the symptoms of Takayasu arteritis?

Takayasu arteritis causes inflammation of your arteries. This may lead to symptoms such as:

• Tiredness

• Weight loss

• Low fever

Over time, inflammation will damage your arteries. It can cause 1 of your arteries to be partly blocked (stenosis). Or it can cause a complete blockage of 1 of your arteries (occlusion). It also can cause the walls of an artery to become weaker. The weakened artery will then inflate and bulge. This is called an aneurysm. An aneurysm can cause tearing of the artery. Bleeding of your artery will cause serious problems or even death.

Your symptoms will depend on the artery affected. Possible symptoms include:

• Legs and arms feeling cooler than normal

• Pain when you move your legs or arms

• Open sores or dead skin on your arms or legs

• Pain in your muscles or joints

• Belly (abdominal) pain or diarrhea

• Chest pain

• Trouble breathing

• Coughing up blood

• Loss of your sense of balance

• Sudden loss of consciousness

How is Takayasu arteritis diagnosed?

There is no 1 test that can diagnose Takayasu arteritis. And many of its symptoms are a lot like those of other diseases. For these reasons, and because it is so rare, your healthcare provider may not easily recognize and diagnose it.

Your provider will ask about your health history and your symptoms. You will have a physical exam. During the exam, your provider may:

• Measure your blood pressure.  Low blood pressure or different levels of blood pressure in each of your arms are common signs of Takayasu arteritis.

• Feel your pulse. A slower pulse in 1 or both of your arms is another common sign.

• Listen to your arteries.  This is done using a stethoscope. If you have any blocked arteries, they will make a noise called bruit.

You may also have a blood test. This checks your levels of C-reactive protein and alpha-2 globulin protein. Your blood will have higher levels of these if your arteries are inflamed.

You may also have an imaging test of the large arteries. This is an important part of the diagnosis. These tests are done to look for blocked or inflated arteries. Your healthcare provider may use one or more of the below:

• Ultrasound. This uses sound waves to create images of your arteries.

• Angiography. This is done by injecting a dye into your artery while taking X-ray images.

• CT angiography (CTA). This also uses a series of X-rays to create a detailed image, with a dye.

• Magnetic resonance angiography (MRA). This uses magnetic waves with or without contrast dye to take a picture without using X-rays.

How is Takayasu arteritis treated?

A main goal of treatment is to reduce damage to your arteries. This is done by taking medicine that reduces inflammation. You may be given medicine, such as:

• Glucocorticoid medicine. Prednisone is a glucocorticoid medicine that can help treat Takayasu arteritis. Taking this for a long time can damage your bones. Taking calcium and vitamin D supplements may help prevent this damage. This medicine may not work for all people.

• Immunosuppressant medicine. This may be given if prednisone doesn’t work for you, or stops working. This medicine suppresses the immune system and helps prevent the inflammation. The medicines include methotrexate and azathioprine. These medicines may not work for all people.

• Antitumor necrosis factor (anti-TNF). Etanercept and infliximab are two examples of anti-TNF medicine.

What are possible complications of Takayasu arteritis?

Possible complications are caused by blocked arteries. They can vary depending on which artery is blocked and may include problems, such as:

• High blood pressure

• Heart attack

• Stroke

• Gastrointestinal bleeding

• Seizures

Managing this disease can be very stressful for both you and your family or caregiver. If any of you have signs of anxiety or depression, talk to your healthcare provider. They can advise you about getting professional counseling. Some people also find that support groups can offer helpful ways to cope. Ask your provider about online or local support groups.

Key points about Takayasu arteritis

• Takayasu arteritis is a rare, chronic autoimmune disease. It causes inflammation of the large arteries. Over time, this can cause blockage of the arteries.

• Most of the symptoms result from blocked arteries.

• Medicines are given to reduce the inflammation and prevent blockage of the arteries.

• Your arteries will need to be checked regularly.

• If your artery is almost fully blocked, you may need surgery to fix the problem.

Next steps

Tips to help you get the most from a visit to your healthcare provider:

• Know the reason for your visit and what you want to happen.

• Before your visit, write down questions you want answered.

• Bring someone with you to help you ask questions and remember what your provider tells you.

• At the visit, write down the name of a new diagnosis and any new medicines, treatments, or tests. Also write down any new instructions your provider gives you.

• Know why a new medicine or treatment is prescribed and how it will help you. Also know what the side effects are.

• Ask if your condition can be treated in other ways.

• Know why a test or procedure is recommended and what the results could mean.

• Know what to expect if you do not take the medicine or have the test or procedure.

• If you have a follow-up appointment, write down the date, time, and purpose for that visit.

• Know how you can contact your provider if you have questions, especially after office hours or on weekends.