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 primarily damages the aorta — the large artery that carries blood from your heart to the rest of your body — and the aorta's main branches.

The disease can lead to blockages or narrowed arteries (stenosis) or abnormally dilated arteries (aneurysms). Takayasu's arteritis can also lead to arm or chest pain and high blood pressure and eventually to heart failure or stroke.

The goal of treatment is to relieve inflammation in the arteries and prevent potential complications. Even with early detection and treatment, however, Takayasu's arteritis can be challenging to manage.

Stage 1 symptoms

Takayasu's arteritis symptoms often occur in two stages. In the first stage, you're likely to feel unwell with:

  • Fatigue
  • Fast and unintentional weight loss
  • Muscle aches
  • Joint pain
  • Slight fever

Not everyone has these early symptoms, however. It's possible for inflammation to damage arteries for years before you realize something is wrong.

Stage 2 symptoms

Second-stage symptoms begin to develop when inflammation has caused arteries to narrow. At this point, there's less blood, oxygen and nutrients reaching your organs and tissues. These signs and symptoms may include:

  • Arm or leg weakness or pain with use (claudication)
  • Lightheadedness or dizziness
  • Fainting
  • Headaches
  • Memory problems
  • Trouble thinking
  • Shortness of breath
  • Visual problems
  • High blood pressure
  • Difference in blood pressure between your arms
  • A difficult-to-find or absent pulse in the wrists — Takayasu's arteritis is sometimes called pulseless disease because narrowed arteries can make normal pulses difficult or impossible to detect
  • Too few red blood cells (anemia)
  • Chest pain
  • Abdominal pain

When to see a doctor

If you have symptoms that might suggest Takayasu's arteritis, see your doctor. Many signs and symptoms of Takayasu's arteritis are similar to those of other conditions, which can make diagnosis challenging. Still, early detection of the disease is important for getting the most benefit from treatment and preventing complications.

If you've already been diagnosed with Takayasu's arteritis, keep in mind that the symptoms of a disease flare (recurrence) are often similar to those that occurred originally. Also pay attention to any new signs or symptoms. These may indicate either a disease flare or a complication of treatment.

In 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. The result is reduced blood flow to vital tissues and organs, which can lead to serious complications and even death. Sometimes arteries become abnormally dilated, leading to aneurysms that may rupture.

Just what causes the initial inflammation in Takayasu's arteritis isn't known. It's likely that Takayasu's arteritis is an autoimmune disease in which your immune system malfunctions and attacks your own arteries as if they were foreign substances. The disease may be triggered by a virus or other infection.

Takayasu's arteritis primarily affects young girls and women in their 20s and 30s. The disorder occurs worldwide, but it's most common in Asian women.

The severity of Takayasu's arteritis may vary. In some people, the condition remains mild and doesn't produce complications. But in others, extended or recurring cycles of inflammation and healing in the arteries can lead to one or more of the following:

  • Hardening and narrowing of blood vessels, which can cause reduced blood flow to organs and tissues
  • High blood pressure, usually as a result of decreased blood flow to your kidneys
  • Inflammation of the heart, which may affect the heart muscle (myocarditis), the heart valves (valvulitis) or the sac around the heart (pericarditis)
  • Heart failure due to high blood pressure, myocarditis or aortic regurgitation — a condition in which a faulty aortic valve allows blood to leak back into your heart — or a combination of these
  • Ischemic stroke, a type of stroke that occurs as a result of reduced or blocked blood flow in arteries leading to your brain
  • Transient ischemic attack, a temporary stroke that has all the symptoms of an ischemic stroke without causing lasting damage
  • Aneurysm in the aorta, which occurs when the walls of the blood vessel weaken and stretch out, forming a bulge that has the potential to rupture
  • Heart attack, an uncommon event that may occur as a result of reduced blood flow to the heart
  • Lung involvement when the arteries to the lungs (pulmonary arteries) become diseased

Pregnancy

A healthy pregnancy is possible for women with Takayasu's arteritis. However, the disease can affect your fertility and pregnancy. If you have Takayasu's arteritis and are planning on becoming pregnant, it's important to work with your doctor to develop a comprehensive plan to limit complications of pregnancy before you conceive. In addition, you'll be closely monitored throughout your pregnancy.

If your family doctor or general practitioner suspects Takayasu's arteritis, you will likely be referred to one or more specialists for diagnosis and treatment. Takayasu's arteritis is a rare disorder that can be difficult to diagnose and treat. If your case is very serious, 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 there's often a lot of ground to cover, it can help to be well prepared. Here's some information to help you get ready and know what to expect from your doctor.

What you can do

  • Be aware of any pre-appointment restrictions. At the time you make the appointment, be sure to ask if there's anything you need to do in advance, such as restrict your diet or your use of certain vitamins or medications.
  • Write down any symptoms you're experiencing, even if they seem unrelated to arteritis. Symptoms of Takayasu's arteritis, especially in the early stages, are similar to those of a number of other illnesses, so it's important that your doctor knows what you're experiencing.
  • Write down key personal information, including any major stresses or recent life changes.
  • Make a list of all medications, vitamins or supplements that you're taking. Even better, take the original bottles or a written list of the dosages and directions.
  • Take a family member or friend along. Sometimes it can be difficult to remember all the information provided during an appointment. Someone who accompanies you may remember something that you missed or forgot.
  • Write down questions to ask your doctor.

Your time with your doctor is limited, so preparing a list of questions can help you make the most of your time together. For Takayasu's arteritis, some basic questions to ask your doctor include:

  • What is likely causing my symptoms?
  • Are there other possible causes for my symptoms?
  • What kinds of tests do I need?
  • Is my condition likely temporary or long lasting?
  • What treatments are available? Which do you recommend for me?
  • I have these other health conditions. How can I best manage these conditions together?
  • Are there any dietary or activity restrictions that I need to follow?
  • Is there a generic alternative to the medicine you're prescribing me?
  • What if I can't or don't want to take steroids?
  • Are there any brochures or other printed material that I can take with me? What websites do you recommend?

In addition to the questions that you've prepared to ask your doctor, don't hesitate to ask additional questions.

What to expect from your doctor

Your doctor is likely to ask you a number of questions. Being ready to answer them may reserve time to go over points you want to spend more time on. Your doctor may ask:

  • When did you first start having symptoms?
  • Do you have your symptoms all the time, or do they come and go?
  • How severe are your symptoms?
  • What, if anything, seems to improve your symptoms?
  • What, if anything, appears to worsen your symptoms?

Takayasu's arteritis can be challenging to detect, and some people go years without an accurate diagnosis. Your doctor may use some of the following steps and tests to help rule out other conditions that closely resemble Takayasu's arteritis and to confirm the diagnosis:

  • Medical history and physical exam. Your doctor will examine you and ask you questions about your general health, including questions about heart and vascular disease.
  • Blood tests. Your doctor may order blood tests to check for signs of inflammation in your body, such as a high white blood cell count or high levels of C-reactive protein, an inflammatory substance produced by your liver. Another blood test commonly used to help identify inflammatory disorders is called the erythrocyte sedimentation rate (sed rate). Your doctor may also check for anemia.
  • Angiography. Traditionally, doctors have used an X-ray test called an angiogram as one of the more definitive tests for diagnosing Takayasu's arteritis. During an angiogram, a thin, flexible tube called a catheter is inserted into a large blood vessel. A special dye (contrast medium) is then injected into the catheter, and X-rays are taken as the dye fills your arteries or veins. The resulting images allow your doctor to see if blood is flowing normally or if it's being slowed or interrupted due to narrowing (stenosis) or blockage of a blood vessel. A person with Takayasu's arteritis generally has several areas of stenosis.
  • Magnetic resonance angiography (MRA). Increasingly, doctors are using this less invasive form of angiography in place of traditional angiography as a test for Takayasu's arteritis. MRA produces detailed images of your blood vessels without the use of catheters or X-rays, although an intravenous contrast medium generally is used. MRA works by using radio waves in a strong magnetic field to produce data that a computer turns into detailed images of tissue slices.
  • Magnetic resonance imaging (MRI). An MRI is similar to an MRA in that it uses radio waves and a magnetic field to create detailed images of organs in the body and allows your doctor to check for possible damage. MRI doesn't use a contrast medium, however.
  • Computerized tomography (CT) angiography. This is another noninvasive form of angiography combining computerized analysis of X-ray images with the use of intravenous contrast dye to allow your doctor to check the structure of your aorta and its nearby branches and to monitor blood flow.
  • Ultrasonography. Doppler ultrasound, a more sophisticated version of the common ultrasound, has the ability to produce very high-resolution images of the walls of certain arteries, such as those in the neck (carotid arteries) and those in the shoulder (subclavian arteries). It may be able to detect subtle changes in these arteries before other imaging techniques can. Doppler ultrasound can also help distinguish between Takayasu's arteritis and atherosclerosis, a much more common condition caused by the buildup of cholesterol particles and other cellular debris in your arteries.

Unlike other types of vasculitis, the removal and analysis of tissue (biopsy) is not usually used to diagnose Takayasu's arteritis.

Because Takayasu's arteritis has a tendency to recur or flare up after being in remission for a while, these tests may be used not only for diagnosis but also for monitoring the progress of the disease and following up on effectiveness of treatment. Some of the medications used for Takayasu's arteritis may have potentially harmful effects over the long run, so it's important for you and your doctor to know when medication is beneficial and when its risks outweigh its benefits.

The goal of treatment is to control inflammation and prevent further damage to your blood vessels with the fewest long-term side effects. Takayasu's arteritis can sometimes be difficult to treat because even if you appear to be in remission the disease may still be active. In addition, by the time some people are diagnosed, it's possible that irreversible damage may already have occurred.

On the other hand, if you don't have a lot of signs and symptoms or serious complications, you may not need treatment at all.

Treatment usually consists of medications and, in some cases, surgery.

Medications

Many of these medications have serious long-term side effects, so your doctor will try to balance their benefits against their potential risks by controlling dosing of medications and the length of time you take them.

  • Corticosteroids. The first line of treatment is usually with a corticosteroid, such as prednisone or methylprednisolone (Medrol). You often start feeling better in just a few days, but you usually need to continue taking medication for an extended period of time. After the first month, your doctor may gradually begin to lower the dose until you reach the lowest dose you need to control inflammation. Some of your symptoms may return during this tapering period. Long-term side effects of corticosteroids include weight gain, high blood sugar, increased risk of infections, osteoporosis, menstrual irregularities and slower wound healing.
  • Immune-suppressing medications. If your condition doesn't respond well to corticosteroids or you have trouble tapering off the medication, you may need treatment with drugs that suppress immune system function, such as methotrexate (Trexall, Rheumatrex) or azathioprine (Imuran, Azasan). Some people respond well to medications that were developed for people receiving organ transplants. These drugs, including mycophenolate (CellCept), work by suppressing the immune system, and they have effectively reduced blood vessel inflammation in people with Takayasu's arteritis. The most common side effect is an increased risk of infection.
  • Drugs that regulate the immune system. In people who don't respond to standard treatments, drugs that correct abnormalities in the immune system (biologics) may be used. Examples of these drugs include adalimumab (Humira), etanercept (Enbrel), infliximab (Remicade) and tocilizumab (Actemra). Small studies have found these medications effective at controlling signs and symptoms, as well as at reducing the need for corticosteroid treatments. However, the studies have been small, and more research is needed. The most common side effect is an increased risk of infection.

Surgery

If your arteries become severely narrowed or blocked, surgery may be necessary to open or bypass these arteries to 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. These procedures, which are best performed when inflammation of the arteries has been reduced, include:

  • Bypass surgery. In this procedure, an artery or a vein is removed from a different part of your body and attached to the blocked artery, providing a bypass for blood to flow through.
  • Blood vessel widening (percutaneous angioplasty). During this procedure, a tiny balloon is threaded through a blood vessel and into the affected artery. Once in place, the balloon is expanded to widen the blocked area, then it's deflated and removed.
  • Stenting. Tiny wire mesh coils called stents may be inserted into the area widened by angioplasty. The stents help to prop open the artery to prevent the blood vessel from narrowing again.

When Takayasu's arteritis is identified and treated early, the prognosis is usually good. One of your greatest challenges may be coping with side effects of your medication. The following suggestions may help:

  • Understand your condition. Learn everything you can about Takayasu's arteritis and its treatment. Know the possible side effects of any medications you take and report any changes in your health to your doctor.
  • Eat a healthy diet. Eating well can help prevent potential problems that may 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.

    Be sure to get adequate amounts of calcium and vitamin D to help prevent osteoporosis, a primary side effect of treatment with corticosteroids. Ask your doctor what the proper amount of these nutrients is for you. If you find it hard to get calcium from your diet because you don't eat dairy products, for example, talk with your doctor about trying calcium supplements, which are often combined with vitamin D and may help combat thinning bones.

  • Exercise regularly. Regular aerobic exercise, such as walking, can help prevent bone loss, high blood pressure and diabetes. It also benefits your heart and lungs. In addition, many people find that exercise improves their mood and overall sense of well-being.
  • Avoid all tobacco products. It's important to stop using all forms of tobacco to reduce the risk of injuring your blood vessels and tissues even more.
Mar. 13, 2013