Takayasu's arteritis

Takayasu's arteritis

Definition:

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 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, called stenoses, or abnormally dilated arteries, called aneurysms. Takayasu's arteritis can also lead to arm or chest pain, high blood pressure, and eventually to heart failure or stroke.

Takayasu's arteritis mainly affects young girls and women under 40. The exact cause of the disease is not known.

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.

Symptoms:

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

• Fatigue
• Rapid, unintended weight loss
• Muscle or joint pain
• Low-grade fever

Not everyone has these initial symptoms, however, and it's possible for inflammation to damage arteries for years before problems appear.

Second-stage symptoms
Second-stage symptoms begin to develop when inflammation has caused arteries to narrow, reducing the amount of blood, oxygen and nutrients reaching certain organs and tissues. These signs and symptoms may include:

• Arm or leg weakness or pain with use (claudication)
• Lightheadedness, dizziness, fainting, headaches
• Difficulty thinking and remembering
• Visual disturbances
• High blood pressure
• Difference in blood pressure between your two arms
• Diminished 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
• Anemia, which may make you feel tired or weak
• Chest pain
• In some people, high blood pressure in the arteries in the lungs (pulmonary hypertension) leading to shortness of breath and fatigue

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 when the disease first began. Also pay attention to any new signs or symptoms. These may indicate either a disease flare or a complication of treatment.

Causes:

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.


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 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 — either of the heart muscle itself (myocarditis), of the sac that surrounds the heart (pericarditis) or of the heart valves (valvulitis)
• 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 — although not common, it 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 delivered a healthy baby. However, the disease may pose risks for you and your baby, and drugs to treat it may also cause problems. 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.


Treatments and drugs:

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, disease activity may still continue "silently." In addition, by the time some people are diagnosed, it's possible that irreversible damage may already have occurred.
On the other hand, if your condition is relatively stable and uncomplicated, 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). About half the people treated with corticosteroids respond well. 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 cataracts, high blood sugar, increased risk of infections, loss of calcium from bones, menstrual irregularities, suppressed adrenal gland hormone production, thin skin, obesity, 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 (Trexall, Rheumatrex) or azathioprine (Imuran, Azasan). These drugs suppress the inflammation in your blood vessels, but they have risks. They can increase your susceptibility to infection, as well as your risk of developing lymph node tumors (lymphoma) and skin cancer.
  
• Transplant medications. 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. Side effects include abdominal pain, fever, constipation, headache and swelling. These drugs may not be taken during pregnancy.

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 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, best performed when inflammation of the arteries is sufficiently suppressed, 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.