Takayasu's Arteritis Causes, Symptoms, Diagnosis, Treatment, Prevention

Takayasu's Arteritis Causes, Symptoms, Diagnosis, Treatment, Prevention

What is Takayasu's arteritis?

Takayasu's arteritis is an uncommon form of vasculitis. Inflammation damages large, and medium-sized blood vessels. The vessels most commonly affected are the branches of the aorta (the main blood vessel that leaves the heart), including the blood vessels that supply blood to the arms and travel through the neck to provide blood to the brain. The aorta itself is also often affected.

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.

Takayasu's arteritis was named for Dr. Mikoto Takayasu, the doctor who first described the disorder in 1908.

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.

What Causes Takayasu's Arteritis

The cause of Takayasu arteritis is unknown. The disease occurs mainly in children and women younger than 30, especially those who are of Asian or African descent.

It appears to be an autoimmune condition, where immune cells are wrongly targeted against the body's own tissues, and it may involve other systems.

Who gets Takayasu's Arteritis?

Takayasu's Arteritis is rare, affecting perhaps one in 200,000 people. It most often occurs in people ages 15–40 years, but sometimes affects younger children or middle-aged adults. Nine of 10 patients are female. Takayasu's Arteritis seems to be more common in East Asia, India and, perhaps, Latin America, than in other regions. Yet, it is rare even in these regions and occurs in a wide range of ethnic groups.

Every year in the United States, two to three new cases of Takayasu's Arteritis per million Americans are diagnosed.

What Are the Symptoms of Takayasu's Arteritis?

Approximately half of all patients with Takayasu's Arteritis will have a sense of generalized illness. This may include fevers, anemia, muscle aches, arthritis and/or headaches. Narrowed vessels cause decreased blood flow to the areas that are supplied "downstream" from the narrowed area.

The changes that occur in Takayasu's Arteritis are often gradual, allowing alternate (or collateral) routes of blood flow to develop. These alternate routes are often smaller "side roads." The collateral vessels may or may not be adequate to carry as much blood as was present normally.

In general, the blood flow that occurs beyond an area of narrowing is almost always adequate to allow tissues to survive. In rare cases, if collateral blood vessels are not available in sufficient quantity, the tissue that is no longer supplied by blood and oxygen will die. This is called an "infarction."

Narrowing of blood vessels to the arms or legs may cause fatigue, pain, or aching due to reduced blood supply -- especially during activities such as shampooing the hair, exercising or walking. It is much less common for decreased blood flow to cause a stroke or a heart attack (myocardial infarction). In some patients, decreased blood flow to the intestines may lead to abdominal pain, especially after meals.

Decreased blood flow to the kidneys may cause high blood pressure, but rarely causes kidney failure.

Some patients with Takayasu's Arteritis may not have any symptoms. Their diagnosis may be stumbled upon by a doctor who has difficulty measuring blood pressure in one or both arms. Similarly, a doctor may notice that the strength of pulses in the wrists, neck or groin may not be equal, or the pulse on one side may be absent.

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.

How is Takayasu's Arteritis diagnosed?

Doctors most often find Takayasu's Arteritis on an angiogram, a test that shows how well blood flows in arteries. A doctor often orders an angiogram when a patient has symptoms and abnormal results of the physical exam. These include loss of pulse or low blood pressure in an arm, or abnormal sounds ("bruits") heard over large arteries with a stethoscope.

There are various types of angiograms, including standard ones that involve injection of dye directly into an artery while X-ray test are taken. Less invasive types of angiography use another imaging technique such as computed tomography, and this is CT angiography or CTA. When MRI—magnetic resonance imaging— is used, it is called magnetic resonance angiography or MRA.

Angiograms may show narrowing of one or more large arteries. It is important for the doctor to try to distinguish between narrowing due to vasculitis (inflammation of arteries) and narrowing due to atherosclerosis ("hardening" of the arteries). At times, this can be challenging. There are other causes of arterial narrowing as well, including fibromuscular dysplasia, another rare disease that mainly affects women.

Large arteries can also become inflamed in a few other diseases. Examples include other types of vasculitis: giant cell arteritis (a disease of older adults), relapsing polychondritis, Cogan's syndrome and Beh├žet's disease. Some infections can also cause inflammation in large arteries.

Blood tests for inflammation include measurements of the erythrocyte sedimentation rate (sometimes referred to as the "sed rate" or ESR) and C-reactive protein (often called CRP). Results of these tests are often, but not always, high in patients with Takayasu's Arteritis. However, these tests are also abnormal in a large number of other inflammatory diseases. Patients with Takayasu's Arteritis may also have anemia due to chronic (long-term) inflammation. Anemia is also tested for with a blood test. None of these blood tests can tell you for sure if you have Takayasu's Arteritis, and these blood tests may be abnormal in many other diseases.

Patients with Takayasu's Arteritis may have no symptoms, and the disease is so rare that doctors may not easily recognize it. Thus, there is often a delay in detecting it, sometimes several years.

How Is Takayasu's Arteritis Treated?

Corticosteroids -- often called simply "steroids" -- are the most common treatment for Takayasu's arteritis. Steroids work within hours after the first dose is given. While this drug often is dramatically effective, it may be only partially effective for some people.

Once it is apparent that the disease is under control, doctors slowly reduce the dosage of prednisone (a steroid) to sustain improvement, thereby trying to minimize treatment side effects. In some people, it is possible to gradually discontinue medication without a relapse.

As the dosage of prednisone is gradually reduced, about half of all patients will have recurrent symptoms or progression of illness. This has led researchers to explore additional treatments to produce remission. Among medications that have been tried, with varying degrees of success, are immunosuppressive drugs such as methotrexate.

When these drugs are added to prednisone to treat Takayasu's arteritis, 50% of patients who had previously relapsed will achieve remission and be able to gradually discontinue prednisone. Overall, about 25% of patients will have disease that is not entirely controlled without continued use of these treatments. This emphasizes the need for continuing research to identify better and less toxic treatments for Takayasu's arteritis and other forms of vasculitis.

Many patients with Takayasu's arteritis have high blood pressure. Careful control of blood pressure is very important. Inadequate treatment of high blood pressure may result in stroke, heart disease, or kidney failure. In some cases, it is desirable to stretch narrow vessel openings with a balloon (a technique known as "angioplasty") or to do a bypass operation to restore normal flow to the kidney. This may result in normal blood pressure without the need to use blood pressure drugs.

Some patients may have serious disabilities because of narrowed blood vessels that supply other sites, such as the arms or legs. Bypass operations may correct these abnormalities. Aneurysms also can be surgically repaired.

Possible Complications of Takayasu's Arteritis

Prevention for Takayasu's Arteritis

As the precise etiology of Takayasu arteritis and causes of flare-ups in disease activity are unknown, there are no known specific preventive actions. Management of hypertension is important to prevent further vascular damage. Attention to osteoporosis screening and management is crucial, given the need for corticosteroid therapy. Patients require influenza and pneumococcal immunizations annually. Use of prophylactic antibiotic therapy to prevent Pneumocystis jirovecii pneumonia is important, especially when the prednisone dose is more than 20 mg daily. Atherosclerotic vascular disease can further complicate the vascular damage caused by Takayasu arteritis; thus, control of other risk factors is important.

Living with Takayasu's Arteritis

Takayasu's Arteritis is a chronic disease and may need long-term treatment. Some patients have no symptoms or only mild symptoms, but others are disabled or need surgery more than once. Side effects from medicines, mainly glucocorticoids, can be troubling. Patients taking immunosuppressants are at risk of infections.

Blood pressure measurement is often not correct (falsely low due to blocked arteries) in the arm. So, your health care provider may need to measure your blood pressure in a leg.

The disease can recur after treatment or can silently get worse. It is often very hard to know whether Takayasu's Arteritis is active again. Thus, most patients need frequent doctor visits and angiograms.

Will People With Takayasu's Arteritis Be Able to Live Normal Lives?

Takayasu's arteritis is clearly a treatable disease and most patients improve. However, it is apparent that many patients have to deal with consequences of this illness that may be partially or, less often, completely disabling. Close follow-up is required as drugs used to treat the disease have side effects that should be regularly monitored by a doctor.

What is the outlook?

In the United States and Japan, only about 3% of patients with Takayasu's Arteritis die after having the disease for an average period of 5 years. This encouraging statistic is the product of recognizing the disease and treating it appropriately. Reports from certain other parts of the world have been less optimistic. This may be the result of delayed recognition and treatment or poor access to care.

For patients who live long lives, in spite of having Takayasu's disease, there are significant problems that must be recognized. Having a chronic illness requires periodic evaluation and adjustment of drugs whenever necessary. The drugs for Takayasu's Arteritis have side effects, and these must be monitored by a doctor, as well as by blood tests. The effects of illness on function may be significant.

In our experience, 25% of patients have an entirely normal lifestyle. Another 25% have had to make some adjustments in their activities. About half of our patients had to modify their jobs and some within that group were occupationally disabled.

Takayasu's Arteritis is clearly a treatable disease, and most patients improve. However, it is apparent that many patients have to deal with consequences of this illness that may be partially, or less often, completely disabling. These effects can be minimized by a team of doctors that includes specialists in vascular and immunologic diseases (rheumatology, immunology, radiology, vascular medicine, vascular and cardiac surgery). For best results, a team leader should coordinate diagnostic tests and the different forms of treatment that Takayasu's Arteritis patients may require.