What Is Takayasu's Arteritis?
Arteritis is a general term that refers to the inflammation of arteries — blood vessels that carry blood away from the heart and to the rest of the body. A closely related condition called vasculitis is inflammation of blood vessels in general — including veins and arteries.
Takayasu's arteritis, also sometimes called "pulseless disease" or Takayasu arteritis, is a rare condition in which inflammation damages large and medium-sized arteries. The arteries most commonly affected are the branches of the aorta (the main blood vessel that leaves the heart), including the arteries to the brain , kidneys, and arms. The aorta itself is also often affected.
Less commonly, arteries that provide blood to the heart, intestines, kidneys, and legs may be involved.
Takayasu's arteritis was named after Mikoto Takayasu, the doctor who first described the disorder in 1908. About 2-3 out of every 1 million people in the U.S. are diagnosed with Takayasu's arteritis.
Takayasu's Arteritis Symptoms
Takayasu's arteritis can cause weakening of your artery walls, which can lead to tearing or bulging (aneurysm).
It can also cause narrowing or blocking of your arteries, leading to reduced blood flow. When this happens in blood vessels of your arms or legs, this may cause fatigue, pain, or aching — especially during activities such as shampooing the hair, exercising, or walking. While less common, reduced blood flow may cause a stroke or a heart attack. In some people, poor blood flow to the intestines may lead to abdominal pain, especially after meals.
About half of all people with Takayasu's arteritis will have symptoms, which usually come on gradually. "Initially [a patient] only presents with general symptoms such as fatigue, pain, weakness, weight loss, and pain that makes diagnosis difficult," says Mehrnaz Hojjati, MD, a rheumatologist with Providence St. Joseph Hospital in Orange County, CA.
Other early signs of the condition may include:
- Low-grade fevers
- Swollen glands
- Anemia
- Night sweats
- Muscle aches
- Arthritis
- Reduced appetite
Symptoms of Takayasu's arteritis in later stages might include:
- Dizziness
- Headaches
- Chest pain
- Shortness of breath
- Chest or abdominal pain
- Muscle weakness and arm or leg pain
Reduced blood flow and blood vessel narrowing may cause high blood pressure or kidney failure ; in rare cases, they can cause a heart attack or stroke.
Some people with Takayasu's arteritis may not have any symptoms. It may be diagnosed when their doctor has trouble getting a blood pressure reading in one or both of their arms. Similarly, a doctor may notice that the strength of pulses in their wrists, neck, or groin may not be equal, or the pulse on one side may be absent.
"Absent or weak peripheral pulse is a common finding in patients with TAK due to the inflammation of the arteries that results in narrowing or blockage, making it difficult to feel a pulse in the affected areas. Thus the reason it is sometimes called pulseless disease," Hojjati says.
What Causes Takayasu's Arteritis?
The exact cause of Takayasu's arteritis is unknown. It’s believed that the condition is an autoimmune disease. Your immune system is supposed to fight off attacking microbes, such as viruses and bacteria. With autoimmune diseases, your immune system attacks your body’s healthy cells instead of just the "bad" ones. Although it's not known for sure, some researchers believe that Takayasu's arteritis might be triggered by:
- Infections
- Environment
- Genetics
Who Gets Takayasu Arteritis?
Takayasu's arteritis most often affects people in Asia, but it can also affect children and adults of all ages and ethnic backgrounds around the world. It affects more women than men (eight women for every one man), and three-quarters of people who have the condition start showing symptoms in their teen years. That said, the actual diagnosis is often much later, around age 29.
Every year in the U.S., 2-3 people per 1 million are diagnosed with Takayasu's arteritis.
Takayasu's Arteritis Diagnosis
There's no single test that can tell you that you have Takayasu's arteritis. Diagnosis is based on a combination of findings, including:
- A complete medical history and careful physical exam to rule out other illnesses that may have similar symptoms
- Imaging studies (such as MRI, X-rays, CT scans, and angiograms), which show the location and severity of vessel damage
- Presence of a bruit, which is an unusual sound caused by turbulent blood flow through narrowed vessels
- Blood tests for signs of inflammation, which can suggest that the condition is possible, when considered alongside other test findings
Takayasu's Arteritis Treatment
There is no cure for Takayasu's arteritis, but treatment can help manage symptoms of the condition. Treatment focuses on using medications to lower inflammation as well as lower blood pressure, preventing further damage to the affected arteries and tissues.
Although statistics vary, one study found that about 80% of people with Takayasu's arteritis can manage their condition with proper treatment. Some even go into remission. This doesn't mean they’re cured, but the disease isn’t active, and they might be able to reduce or even stop their medications.
Corticosteroids
Corticosteroids, also called systemic glucocorticoids or just steroids, are usually the first treatment for Takayasu's arteritis. Steroids work within hours after the first dose is given. The most common drug in this class is prednisone. While this drug is often highly effective, it may be only partially effective for some people.
If prednisone works and your disease is under control, your doctor will likely slowly lower the dosage of prednisone (a steroid) to sustain improvement, thereby trying to lessen treatment side effects.
In some people, it is possible to gradually stop medication without a relapse, but about half of all patients will have repeated symptoms or worsening of the illness.
Immunosuppressants
Nonglucocorticoid immunosuppressive agents (drugs) are medications that suppress the immune system but aren't steroids. These drugs fall into one of two classifications: nonbiologics (made purely with synthetic chemicals) and biologics (grown from living cells).
Nonbiologics. If corticosteroids don't work on their own or if the symptoms return after tapering, your doctor may recommend these nonbiologic drugs to suppress your immune system and the inflammation:
- Azathioprine (Imuran)
- Cyclophosphamide
- Leflunomide (Arava)
- Methotrexate
- Mycophenolate mofetil
Biologics. Researchers looking at treatment for Takayasu's arteritis found that patients responded better if they took biologics combined with the steroid rather than nonbiologics with the steroid, Hojjati says. Biologics used to treat Takayasu's arteritis include:
- Etanercept (Enbrel)
- Infliximab (Remicade)
- Tocilizumab (Actemra)
Other medications
Other medications that your doctor may prescribe would be based on the risk of complications from Takayasu's arteritis. For example, if your doctor is concerned about blood clots causing a stroke, you might take heparin, warfarin, or some other type of medication that keeps your platelets from forming clots (called antiplatelets).
Managing high blood pressure caused by Takayasu's arteritis
Many patients with Takayasu's arteritis have high blood pressure. Careful control of blood pressure is also important when treating Takayasu's arteritis. If your blood pressure isn't controlled, it could lead to stroke, heart disease, or kidney failure. If you have hypertension, your doctor will likely prescribe antihypertensive medications to bring it down. Some doctors also prescribe low-dose aspirin.
In addition to taking medications, you might have to make some lifestyle changes to help manage your blood pressure. This could mean doing more physical activity and eating a heart-healthy diet.
Surgery
Sometimes, taking medications isn't enough to help manage Takayasu's arteritis, and you might need surgery to help widen the arteries to allow for a healthy blood flow or to repair an aneurysm. Recommended surgeries might include:
- Open surgery. Traditional surgery where the surgeon makes an incision to do the procedure.
- Percutaneous transluminal balloon angioplasty. A procedure that stretches narrow vessel openings with a balloon that's inserted through a tiny incision in the artery and threaded through to the right place.
- Percutaneous placement of a stent. A small mesh device is placed in the artery to keep the blood vessel open, usually done through a small opening in the artery.
- Bypass surgery. This might be done to bypass the blocked or narrowed artery, supplying blood through a new route. It could be done to restore blood flow to a kidney or other places, such as the arms and legs.
Takayasu's Arteritis Complications
The disease is often diagnosed late. As a result, the long-term reduced blood flow can cause complications. Complications can also occur because of the repeating cycle of inflammation and healing. For example, inflammation can cause segments of the arteries to weaken and stretch. This can result in an aneurysm (bulging blood vessel). When this happens in the aorta, it's called an aortic aneurysm. The arteries can also become narrowed or even completely blocked (called an occlusion).
Other complications can include:
- Damage to the heart muscle or heart valves
- Hardening and narrowing of blood vessels
- Heart attack
- Heart failure
- High blood pressure
- Stroke
- Transient ischemic attacks (TIA), also called ministrokes
Takayasu's Arteritis Outlook
The outlook or prognosis for people with Takayasu's arteritis varies from person to person. "It can be a debilitating disease, mainly [because it] is usually diagnosed late after affecting multiple organs and potentially causing permanent damage," says Hamid Mojibian, MD, an interventional radiologist with Yale Medicine. Statistics about how people manage with Takayasu's arteritis are hard to find because it is such a rare disease.
One small study shows that about half of those who have it relapse (their symptoms return), and they have complications within 10 years of diagnosis. Another slightly larger study shows that about 5% of people die six years after diagnosis. But according to Hojjati, "The short-term prognosis is favorable and several studies have reported up to 90% of five-year survival."
Will People With Takayasu's Arteritis Be Able to Live Normal Lives?
Many people with Takayasu's arteritis respond well to treatment. About 25% of people with the condition can continue living their usual lifestyle, while another 25% need to make adjustments in their activities.
"Most patients have an almost normal life span with adequate treatment," Hojjati says. Although if there are complications, such as aneurysms or a severe lack of blood flow to a limb, or if the disease gets worse, this usually changes the outlook.
Another problem might be the side effects from some of the drugs used to treat Takayasu's arteritis, especially the steroids. These can negatively affect quality of life.
Staying in touch with your doctor for close follow-up is vital. The medications used to treat Takayasu's arteritis can have serious side effects, so your doctor must monitor long-term use. Your doctor will want to watch for any signs of relapse or worsening symptoms.
Takeaways
Takayasu's arteritis is a very rare disease that causes inflammation and narrowing of your major arteries, the blood vessels that supply your body's tissues with oxygen and other nutrients. Treatment can help reduce the inflammation and damage to the blood vessels and organs, but complications of the condition include hypertension, stroke, and heart damage.
Takayasu's Arteritis FAQs
Why is Takayasu's arteritis called the pulseless disease?
If you have Takayasu's arteritis, it can be hard to find a pulse in the affected arm or leg. The limb can seem pulseless.
Can Takayasu's arteritis be cured?
There aren't any cures for Takayasu's arteritis. But treatment can help slow down or stop the disease for a while.
What is the life expectancy of someone with Takayasu's arteritis?
The disease is complicated, but many people live normal lives with proper treatment. There are few accurate statistics, but it could be that as many as 90% of people with effective treatment are still living five years after their diagnosis. This is called the five-year survival rate.
What is the difference between arthritis and arteritis?
Some people can confuse "arteritis" with "arthritis" because the two words sound and look very similar. Both involve inflammation, but in different parts of the body. "Arteritis is the inflammation of the wall of blood vessels, such as the aorta, while arthritis is the inflammation of joints," Mojibian says.