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Takayasu's Arteritis

Definition:
Takayasu's arteritis is an uncommon form of vasculitis, a spectrum of conditions characterized by inflammation of your blood vessels. Takayasu's arteritis damages the large arteries in your body, particularly the aorta — your body's principal blood vessel, which carries blood from your heart to the rest of your body — and its major branches.

Inflammation of a blood vessel, as occurs in Takayasu's arteritis, can cause layers of the blood vessel to thicken. This narrows the blood vessel, reducing the amount of blood — and thus, the oxygen and nutrients — that reaches your body's tissues. Reduced blood flow can lead to conditions such as high blood pressure, heart failure and other circulatory problems. In some cases, a blood clot may form in an affected artery, obstructing blood flow and causing a stroke or heart attack. Sometimes instead of becoming narrower, a blood vessel may weaken and form a bulge (aneurysm). If the aneurysm bursts, it can be life-threatening.

Treatment usually consists of relieving the inflammation in the arteries and preventing potential complications. With early detection and appropriate treatment, the signs and symptoms of Takayasu's arteritis can be managed successfully for many years.

Causes:
What causes Takayasu's arteritis remains unknown. Some experts believe infection plays a role in the disease, particularly because people who have Takayasu's arteritis often have or have had tuberculosis, but the association between these two illnesses isn't clear. In the United States, for example, most people with Takayasu's arteritis have no history of tuberculosis.

Another theory is 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.

When to seek medical advice:
If you have signs and 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. Nonetheless, 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 signs and symptoms of a disease flare (recurrence) are often similar to those that occurred when the disease first began. In addition, be aware of any new signs or symptoms, as these may indicate either a disease flare or the development of a complication of treatment, such as an infection.

Symptoms:
General signs and symptoms associated with Takayasu's arteritis include:
  • Fever
  • Fatigue
  • Night sweats
  • Weight loss
  • Muscle or joint pain, or both
Narrowing of your arteries due to inflammation may lead to reduced blood flow throughout your body, resulting in signs
and symptoms such as:
  • Arm weakness or pain with use (claudication)
  • Lightheadedness, headaches
  • Visual disturbances
  • High blood pressure
  • Difference in blood pressure between two arms
  • Diminished or absent pulse at the wrist
  • Mild anemia, which may make you feel tired or a little weak
  • Chest pain
In the beginning, you may experience only the more general signs and symptoms, such as fever, fatigue, weight loss and pain. But as your arteries become narrowed and blood flow to other organs and tissues is reduced, other more specific signs and symptoms, such as arm weakness and visual disturbances, may become apparent.

Dignosis:

In addition to taking your medical history and conducting a physical exam, your doctor may use some of the following tests to help rule out other conditions that may be causing your signs and symptoms, and to confirm a diagnosis of Takayasu's arteritis:
  • 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). It measures the speed at which red blood cells sink to the bottom of a glass tube. Generally, when inflammation is present, red blood cells clump together and settle more rapidly than normal, causing the sed rate to increase. Your doctor may also check the number of red blood cells for anemia.
  • Angiography. Traditionally, doctors have used an X-ray test called an angiogram as one of the more definitive tests in 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 these arteries or veins. The resulting images allow your doctor to see if blood is flowing normally through your blood vessels 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. MRA produces detailed images of your blood vessels without the use of catheters or X-rays, although a 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 frequency waves and magnetic fields to create detailed images of organs in the body, such as the brain, and allows your doctor to check for damage, such as from a stroke. It 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 contrast dye to allow your doctor to check the structure of your aorta and its nearby branches, and 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 the common carotid and subclavian arteries. As such, 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.
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 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.

Complications:
The severity of Takayasu's arteritis may vary. In some people, the condition remains mild and doesn't produce complications. But in others, extended cycles of inflammation and healing in the arteries can lead to stiff and narrowed blood vessels, reducing blood flow and its vital supply of oxygen and nutrients to organs and tissues of the body. Complications include:
  • High blood pressure, usually as a result of decreased blood flow to your kidneys
  • Inflammation of the heart, of either the heart muscle itself (myocarditis) or the sac that envelops the heart (pericarditis)
  • Congestive heart failure, due to high blood pressure, myocarditis or aortic regurgitation — a condition in which blood that's just been pumped out of your heart leaks back in due to a faulty valve — or a combination of these
  • Transient ischemic attack, a temporary ministroke caused by a momentary reduction in the supply of blood to your brain
  • Ischemic stroke, a type of stroke that occurs as a result of reduced or blocked blood flow in arteries leading to your brain
  • Aneurysm formation in the aorta — in which the walls of the blood vessel weaken and stretch out, forming a bulge — and potential rupture of the aneurysm
  • Blood clot formation
  • Heart attack, not common but 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
In studies of pregnant women with Takayasu's arteritis, most women had a normal pregnancy and delivery. However, if you have Takayasu's arteritis and are planning on becoming pregnant, talk with your cardiologist and obstetrician first. Active Takayasu's may pose risks for you and your baby, and drugs to treat it may cause problems. A comprehensive plan to limit complications of pregnancy should be in place before you conceive.

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

Still, with early detection and appropriate treatment, the signs and symptoms of Takayasu's arteritis can be managed successfully for many years. If your condition is relatively stable and uncomplicated, you may not need treatment.

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

Medications
Many of these medications have 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 drug such as prednisone or methylprednisolone (Medrol). About half the people treated with corticosteroids respond well. You often start feeling better in just a few days, but you may 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 of corticosteroids you need to control inflammation. Some of your signs and symptoms may return during this tapering period. Long-term side effects of corticosteroids include cataracts, high blood sugar, increased risk of infections, loss of calcium from bones, menstrual irregularities, suppressed adrenal gland hormone production, thin skin, obesity, weakness in shoulders and thighs, easy bruising, and slower wound healing.
  • Cytotoxic drugs. If your condition doesn't respond well to corticosteroids or you have trouble tapering off the medication, you may need treatment with cytotoxic drugs, such as methotrexate (Rheumatrex), azathioprine (Imuran) or cyclophosphamide (Cytoxan). These drugs suppress the inflammation in your blood vessels. Mycophenolate mofetil (CellCept), another immunosuppressant used to prevent transplant rejection, also may be helpful for Takayasu's arteritis, though the Food and Drug Administration hasn't approved it for this purpose. Immunosuppressants have risks. They can increase your susceptibility to infection, as well as your risk of developing lymph node tumors (lymphoma) and skin cancer.
  • TNF-alpha inhibitors. In a small study, a group of medications called tumor necrosis factor-alpha (TNF-alpha) inhibitors were able to help some people who had difficult-to-treat Takayasu's arteritis. This class of medications helps to reduce inflammation by inhibiting the action of TNF-alpha protein, which plays an active role in initiating your body's inflammatory response. Given further confirmation of the drug's effectiveness, doctors hope it will be a useful addition to the treatment options for Takayasu's.
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 signs and symptoms, such as high blood pressure and chest pain. In some cases, though, narrowing or blockage may recur, requiring a second procedure. Procedures used 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.
  • 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.
  • 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.
Please be aware that this information is provided to supplement the care provided by your physician. It is neither intended nor implied to be a substitute for professional medical advice. CALL YOUR HEALTHCARE PROVIDER IMMEDIATELY IF YOU THINK YOU MAY HAVE A MEDICAL EMERGENCY. Always seek the advice of your physician or other qualified health provider prior to starting any new treatment or with any questions you may have regarding a medical condition.
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