Takayasu Arteritis

Takayasu arteritis is a disease that damages your major arteries throughout your body. It’s a form of vasculitis, which is chronic inflammation in your blood vessels. Arteritis is inflammation in your arteries. These are the vessels that bring oxygenated blood to your organs and tissues. As Takayasu arteritis damages these vessels, it can reduce their blood flow.

Takayasu arteritis starts with general symptoms, like fatigue, loss of appetite and just feeling ill. Eventually, your arteries may become hardened and narrowed, which reduces their blood flow. Your blood flow through these arteries may become so weak that healthcare providers have difficulty finding your pulse. This is why it’s sometimes called “pulseless disease.”

Weak blood flow through your arteries (arterial insufficiency) can cause a variety of other symptoms and complications, some of which are life-threatening. It can affect your brain, heart, kidneys, limbs and other body tissues. It can also make daily life uncomfortable and difficult. Fortunately, once diagnosed, Takayasu arteritis is treatable, and it can even go into remission.

Takayasu arteritis symptoms

Chronic inflammation puts constant stress on your body. You might not notice this right away. But usually, the first symptoms you’ll notice will be vague symptoms of overall illness.

Early symptoms may include:

• Fever
• Fatigue
• Loss of appetite
• Muscle pain
• Joint pain
• Skin rash

Since it’s a chronic disease, the inflammation never stops. Over time, it damages your arteries, restricting blood flow to your organs and tissues. This causes further complications.

Secondary symptoms can include:

• Abdominal pain
• Back pain
• Chest pain
• Cramping pain in your arms or legs (claudication)
• Dizziness
• Fingertips that briefly turn blue (Raynaud’s syndrome)
• Headache
• Heart palpitations
• Pain in the front of your neck (carotidynia)
• Shortness of breath
• Vision changes
• Weak or missing pulse

Takayasu arteritis causes

Healthcare providers don’t know the exact cause of Takayasu arteritis. They believe it’s a type of autoimmune disease. This means your immune system attacks your arteries with inflammation by mistake. Inflammation is usually a tool for fighting infections. Some researchers believe an infection or toxin might trigger the process. Then, somehow, the inflammation continues on autopilot.

Complications of Takayasu arteritis

Takayasu arteritis starts in your aorta. This is the main channel that leaves your heart carrying oxygenated blood. From here, the inflammation spreads into your aorta’s major branches.

Chronic inflammation can have one of two effects on your arteries. It can weaken and stretch them, leading to aneurysms and bleeding. Or it may cause scarring that makes them thicken and narrow (stenosis). This constricts their blood flow. Sometimes, both of these things happen in different places.

Complications can include:

• Aortic stenosis: This condition can cause pulmonary hypertension and heart failure.
• Carotid artery stenosis: This condition raises your risk of stroke.
• Coronary artery disease: This condition raises your risk of heart attack.
• Renal artery stenosis: This can cause high blood pressure and chronic kidney disease.
• Subclavian artery stenosis: This is a form of peripheral artery disease. It can affect your limbs and your brain. It raises your risk of subclavian steal syndrome and transient ischemic attack.

Risk factors

Takayasu arteritis is a rare condition. There are fewer than 2 million cases worldwide. It especially affects:

• Females (about 90%of the time)
• People of childbearing age (average age 15 to 40)
• People of East Asian, Indian and Mexican descent

While it doesn’t seem to run in families, Takayasu arteritis does seem to run in ethnic groups. Researchers have recently identified several genes that might be connected with it.

How is Takayasu arteritis diagnosed?

Diagnosis of Takayasu arteritis is based on a combination of factors, including:

• Medical history and physical exam: Your provider may notice signs like a weak pulse, lower blood pressure in one arm or a whooshing sound (carotid bruit) through their stethoscope.
• Imagining tests: Images of your arteries can help show where and how much they’re damaged. These may include X-rays, a Doppler ultrasound, a CT angiogram or MR angiogram.
• Blood tests: Blood tests can check for certain products in your blood that may suggest you have inflammation. ESR and CRP are two examples. These test results are only suggestive, though.

It can take some time to get the right diagnosis. Since the early symptoms of Takayasu arteritis tend to be mild and vague, you and your provider might not suspect it until you have more advanced symptoms.

How is Takayasu arteritis treated?

Healthcare providers treat Takayasu arteritis with medications to control the inflammation and stop the damage to your arteries. Corticosteroids, like prednisone, are powerful anti-inflammatory medications. Most people start with corticosteroids and slowly reduce the dose as their condition improves. You might need to stay on a low dose, and/or take other medications, to manage your condition long term.

Other long-term medications may include:

• Immunosuppressant drugs: These medications decrease the activity of your immune system that causes the inflammation in your arteries. But as a side effect, you can get sick more easily.
• TNF inhibitors: These are a biologic form of DMARDs (disease-modifying anti-rheumatic drugs) that work to prevent inflammation. Biologics are medications derived from living organisms.
• Blood pressure medications: If you have high blood pressure, you may need medications to reduce it. This helps prevent complications of artery disease.
• Statins: If you have high cholesterol, statins can help prevent plaque buildup in your arteries.

While medications can limit further damage to your arteries, they can’t reverse the damage already done. Some people may need vascular surgery to restore blood flow to their organs and tissues.

These procedures may include:

• Aneurysm surgery: Your surgeon removes the aneurysm and replaces it with a graft.
• Angioplasty: Your surgeon widens your narrowed artery and places a stent to keep it open.
• Aortic valve replacement: Your surgeon replaces your valve with a transplant or prosthesis.
• Arterial bypass surgery: Your surgeon reroutes your blood flow to bypass a blocked area.

Since Takayasu arteritis is a lifelong condition, you’ll need lifelong care, even if you’re feeling well. Your provider will monitor your condition regularly to make sure your treatment plan is up to date. Even when it’s working, you might have times when you feel better or worse. Taking stock of how you feel and letting your provider know can help them identify new complications or treatment side effects.

When should I seek care?

When you have Takayasu arteritis, it’s important to see your provider regularly so they can monitor your condition. You should also be sure to contact them if any new symptoms appear.

Talk with your provider if you become or plan to become pregnant. It’s possible to have a successful pregnancy with Takayasu arteritis, but it’s important to know how to manage it safely.

Seek emergency care if you develop:

• Difficulty breathing
• Difficulty smiling or speaking
• Dizziness or faintness
• Chest pain (angina)
• Loss of feeling in a limb
• Muscle weakness on one side
• Pain when you lift your arms (intermittent claudication)
• A weak or missing pulse

What can I expect if I have Takayasu arteritis?

Without treatment, Takayasu arteritis can be debilitating or even fatal. But with the right diagnosis and treatment, the prognosis (outlook) is good. In the United States and Japan, only about 3% of people with Takayasu arteritis die from it, after an average period of five years. Other parts of the world may have worse outcomes, due to low recognition of the disease, delayed diagnosis or poor access to care.

While there’s no cure for Takayasu arteritis, it usually improves with treatment. Symptoms may seem to come and go. When they’re active, you may find it hard to keep up with your usual activities. But after several years of treatment, many people find that their symptoms seem to go away for good. This is called remission. In this case, you may be able to stop treatment, although you’ll still need checkups.