Understand this rare disease that damages the aorta, the large artery that carries blood from your heart to the rest of your body.
Takayasu's arteritis (tah-kah-YAH-sooz ahr-tuh-RIE-tis) is a rare type of vasculitis, a group of disorders that causes blood vessel inflammation. In Takayasu's arteritis, the inflammation damages the large artery that carries blood from your heart to the rest of your body (aorta) and its main branches.
The disease can lead to narrowed or blocked arteries, or to weakened artery walls that may bulge (aneurysm) and tear. It can also lead to arm or chest pain, high blood pressure, and eventually heart failure or stroke.
If you don't have symptoms, you may not need treatment. But most people with the disease need medications to control inflammation in the arteries and to prevent complications. Even with treatment, relapses are common, and your symptoms may come and go.
The signs and symptoms of Takayasu's arteritis often occur in two stages.
In the first stage, you're likely to feel unwell with:
Not everyone has these early signs and symptoms. It's possible for inflammation to damage arteries for years before you realize something is wrong.
During the second stage, inflammation causes arteries to narrow so less blood and oxygen and fewer nutrients reach your organs and tissues. Stage 2 signs and symptoms may include:
Seek immediate medical attention for shortness of breath, chest or arm pain, or signs of a stroke, such as face drooping, arm weakness or having difficulty speaking.
Make an appointment with your doctor if you have other signs or symptoms that worry you. Early detection of Takayasu's arteritis is key to getting effective treatment.
If you've already been diagnosed with Takayasu's arteritis, keep in mind that your symptoms may come and go even with effective treatment. Pay attention to symptoms similar to those that occurred originally or to any new ones, and be sure to tell your doctor promptly about changes.
With Takayasu's arteritis, the aorta and other major arteries, including those leading to your head and kidneys, can become inflamed. Over time the inflammation causes changes in these arteries, including thickening, narrowing and scarring.
No one knows exactly what causes the initial inflammation in Takayasu's arteritis. The condition is likely an autoimmune disease in which your immune system attacks your own arteries by mistake. The disease may be triggered by a virus or other infection.
Takayasu's arteritis primarily affects girls and women younger than 40. The disorder occurs worldwide, but it's most common in Asia. Sometimes the condition runs in families. Researchers have identified certain genes associated with Takayasu's arteritis.
With Takayasu's arteritis, cycles of inflammation and healing in the arteries might lead to one or more of the following complications:
A healthy pregnancy is possible for women with Takayasu's arteritis. But the disease and drugs used to treat it can affect your fertility and pregnancy. If you have Takayasu's arteritis and are planning on becoming pregnant, work with your doctor to develop a plan to limit complications of pregnancy before you conceive. See your doctor regularly during your pregnancy for checkups.
Your doctor will ask you about your signs and symptoms, conduct a physical exam, and take your medical history. He or she may also have you undergo some of the following tests and procedures to help rule out other conditions that resemble Takayasu's arteritis and to confirm the diagnosis. Some of these tests may also be used to check on your progress during treatment.
X-rays of your blood vessels (angiography). During an angiogram, a long, flexible tube (catheter) is inserted into a large artery or vein. A special contrast dye 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) of a blood vessel. A person with Takayasu's arteritis generally has several areas of stenosis.
Treatment of Takayasu's arteritis focuses on controlling inflammation with medications and preventing further damage to your blood vessels.
Takayasu's arteritis can be difficult to treat because the disease may remain active even if your symptoms improve. It's also possible that irreversible damage has already occurred by the time you're diagnosed.
On the other hand, if you don't have signs and symptoms or serious complications, you may not need treatment or you may be able to taper and stop treatment if your doctor recommends it.
Talk with your doctor about the drug or drug combinations that are options for you and their possible side effects. Your doctor may prescribe:
Corticosteroids to control inflammation. The first line of treatment is usually a corticosteroid, such as prednisone (Prednisone Intensol, Rayos). Even if you start feeling better, you may need to continue taking the drug long term. After a few months, your doctor may gradually begin to lower the dose until you reach the lowest dose you need to control inflammation. Eventually your doctor may tell you to stop taking the medication completely.
Possible side effects of corticosteroids include weight gain, increased risk of infection and bone thinning. To help prevent bone loss, your doctor may recommend a calcium supplement and vitamin D.
If your arteries become severely narrowed or blocked, you may need surgery 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, the narrowing or blockage may happen again, requiring a second procedure.
Also, if you develop large aneurysms, surgery may be needed to prevent them from rupturing.
Surgical options are best performed when inflammation of the arteries has been reduced. They include:
One of the greatest challenges of living with Takayasu's arteritis may be coping with side effects of your medication. The following suggestions may help:
Eat a healthy diet. Eating well can help prevent potential problems that can 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.
If you're taking a corticosteroid drug, ask your doctor if you need to take a vitamin D or calcium supplement.
If your primary care doctor suspects that you have Takayasu's arteritis, he or she may refer you to one or more specialists with experience in helping people with this condition. Takayasu's arteritis is a rare disorder that can be difficult to diagnose and treat.
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 information to discuss, it's a good idea to be prepared. Here's some information to help you get ready for your appointment.
For Takayasu's arteritis, some basic questions to ask include:
Your doctor will likely ask you a number of questions, such as:
November 10th, 2021