Takayasu’s Arteritis

Takayasu arteritis, Takayasu’s disease, pulseless disease, nonspecific aortoarteritis, aortic arch syndrome

Takayasu’s arteritis is a rare inflammatory disease that attacks the body’s largest arteries, causing them to narrow, weaken, or develop dangerous bulges. This condition mainly affects young women, particularly those of Asian descent, and can lead to serious complications including stroke and heart failure if left untreated.

Table of contents

• What Is Takayasu’s Arteritis?
• Which Blood Vessels Are Affected?
• Who Gets This Disease?
• Signs and Symptoms
• What Causes Takayasu’s Arteritis?
• Possible Complications
• How Is It Diagnosed?
• Treatment Options
• Living With Takayasu’s Arteritis

What Is Takayasu’s Arteritis?

Takayasu’s arteritis is a rare form of vasculitis, which means inflammation of blood vessels. In this disease, the body’s immune system mistakenly attacks the walls of the largest arteries in the body^[1]. Arteries are blood vessels that carry oxygen-rich blood from the heart to the rest of the body^[2].

The disease was first described in 1908 by Japanese physician Dr. Mikito Takayasu, who noticed unusual blood vessel patterns in the back of a patient’s eye. At the same meeting, two colleagues reported similar findings in patients whose wrist pulses could not be felt^[4]. This is why the condition is sometimes called “pulseless disease” — the inflammation can narrow arteries so severely that doctors have difficulty detecting a pulse in the arms or legs^[2].

• Aorta
• Aortic arch and branches
• Carotid arteries
• Subclavian arteries
• Renal arteries
• Coronary arteries
• Pulmonary arteries

Which Blood Vessels Are Affected?

Takayasu’s arteritis primarily damages the aorta, which is the main blood vessel that leaves the heart and carries blood throughout the body. From there, the inflammation spreads to the aorta’s major branches^[2]. The disease most commonly affects the arteries that supply blood to the arms (subclavian arteries), neck (carotid arteries), and kidneys (renal arteries)^[3].

Doctors classify Takayasu’s arteritis into six types based on which arteries are involved. Type I affects the branches of the aortic arch, Type III involves the thoracic and abdominal aorta, and Type V affects the entire aorta and all its branches. The other types represent combinations of these patterns^[8].

Who Gets This Disease?

Takayasu’s arteritis is extremely rare, affecting only about 1 to 2 people per million in the population worldwide^[3]. The disease shows a strong pattern of affecting certain groups of people more than others.

Women are much more likely to develop Takayasu’s arteritis than men, with approximately 9 out of every 10 patients being female^[4]. The disease typically appears in young women under the age of 40, though it has been diagnosed in people ranging from 6 months old to the elderly^[3].

While the disease has been found worldwide, it occurs more frequently in people of Asian descent, particularly those from Japan, Southeast Asia, India, and Mexico. The highest known rate is in Japan, where about 40 cases occur per million people. It is relatively rare in North America^[3].

Signs and Symptoms

The symptoms of Takayasu’s arteritis typically develop in two stages, though not everyone experiences both phases clearly^[4].

Early Stage: The Systemic Phase

In the first stage, people often feel generally unwell. This phase includes symptoms that could be caused by many different illnesses, which is why the disease can be difficult to recognize early^[1]. Common early symptoms include:

• Extreme tiredness or fatigue
• Unexplained weight loss
• Muscle and joint aches and pains
• Mild fever, sometimes with night sweats
• Loss of appetite

Some people do not experience these early warning signs, and inflammation can damage arteries for years before symptoms become noticeable^[1].

Later Stage: The Occlusive Phase

As the disease progresses, the narrowing of blood vessels begins to cause problems throughout the body. This second phase occurs when arteries become so damaged that blood flow is significantly reduced^[4]. Symptoms in this stage may include:

• Weak or absent pulses in the arms or legs
• Different blood pressure readings in the left and right arms
• Pain in the limbs during activity (claudication), such as arm pain while using tools or leg pain when walking
• Dizziness or lightheadedness, especially when standing up
• Headaches
• Vision changes or vision loss
• Chest pain
• Shortness of breath
• Abdominal pain
• High blood pressure
• Pain in the front of the neck along the path of the carotid arteries

A doctor may hear abnormal sounds called bruits (pronounced “brew-eez”) when listening to arteries with a stethoscope. These are harsh, whooshing sounds that indicate turbulent blood flow through narrowed vessels^[4].

What Causes Takayasu’s Arteritis?

The exact cause of Takayasu’s arteritis remains largely unknown^[3]. Medical researchers believe it is an autoimmune disease, meaning the body’s immune system mistakenly attacks its own tissues. In Takayasu’s arteritis, the immune system targets the walls of large arteries with inflammation^[2].

Normally, inflammation is the immune system’s way of fighting infections and protecting the body from harm. Some researchers think that an infection or exposure to a toxin might trigger the inflammatory process in Takayasu’s arteritis. Once started, the inflammation somehow continues on its own, even after the original trigger is gone^[2].

At its core, Takayasu’s arteritis is characterized as an inflammatory disease involving abnormal cell-mediated immunity. The chronic inflammation leads to thickening of the arterial walls throughout their entire depth, which eventually results in narrowing of blood vessels, blockages, and reduced blood flow to organs and tissues^[3].

Possible Complications

Takayasu’s arteritis can lead to serious, even life-threatening complications when inflammation damages major arteries and reduces blood flow to vital organs^[2].

The chronic inflammation can have two different effects on arteries. It may weaken and stretch the artery walls, leading to bulging areas called aneurysms that can rupture and cause dangerous bleeding. Alternatively, inflammation may cause scarring that makes artery walls thicken and narrow, a condition called stenosis, which restricts blood flow. Sometimes both types of damage occur in different locations^[2].

Specific complications include:

• High blood pressure (hypertension): This occurs when narrowing affects the arteries to the kidneys, causing the kidneys to release substances that raise blood pressure^[6]
• Heart problems: Narrowing of the aorta can cause aortic stenosis, leading to heart failure. Damage to coronary arteries increases the risk of heart attack^[2]
• Stroke: Narrowing of the carotid arteries reduces blood flow to the brain and significantly raises stroke risk^[2]
• Vision problems or loss: Reduced blood flow to the eyes can cause retinal damage and vision changes^[6]
• Kidney disease: Narrowing of renal arteries can lead to chronic kidney disease^[2]
• Aneurysm rupture: Weakened artery walls may tear, causing potentially fatal internal bleeding^[1]

Approximately 10 percent of patients with Takayasu’s arteritis have no symptoms at all, while others may be severely disabled and require surgery^[8].

How Is It Diagnosed?

Diagnosing Takayasu’s arteritis can be challenging because early symptoms are vague and similar to many other conditions^[1]. A doctor should suspect this disease in a young person, especially a woman, who has neck pain along the path of the carotid arteries, absent or weak pulses in the arms or legs, unusual sounds (bruits) when listening to arteries, or unexplained high blood pressure^[13].

Physical Examination and Medical History

The doctor will check for reduced or absent pulses in the arms and legs, measure blood pressure in multiple limbs (which may show significant differences between arms), and listen to major arteries with a stethoscope for bruits^[9].

Blood Tests

Blood tests can reveal signs of inflammation in the body. Common tests include measuring the erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP), which are markers of inflammation. Many patients also have anemia, meaning too few red blood cells^[9]. These blood tests will later be used to monitor disease activity during treatment^[13].

Imaging Studies

Several types of imaging tests help doctors see the damaged blood vessels and confirm the diagnosis:

• Angiography: A doctor inserts a thin tube called a catheter into a large blood vessel and injects a special dye. X-ray images show how blood flows through the arteries and reveal any narrowing, blockages, or abnormal areas^[9]
• MRI (Magnetic Resonance Imaging) or MRA (Magnetic Resonance Angiography): These tests create detailed pictures of blood vessels without using catheters or X-rays. They show the size and structure of arteries and can detect inflammation in the vessel walls^[9]
• CT (Computed Tomography) Angiography: This combines computer-processed X-ray images with contrast dye to visualize the aorta and nearby arteries^[9]
• Doppler Ultrasound: This test uses sound waves to evaluate blood flow and detect narrowing in arteries^[9]

Imaging studies can show characteristic features of Takayasu’s arteritis, including circumferential thickening of artery walls, areas of narrowing (stenosis), and sometimes ballooning out of the vessel wall (aneurysmal dilation), often affecting multiple sites^[13].

Diagnostic Criteria

Recent diagnostic criteria require that patients be age 60 or younger at diagnosis and have evidence of blood vessel inflammation in the aorta or its branches confirmed by imaging. Additional features such as being female, having limb claudication, arterial bruits, reduced pulses, and involvement of multiple arterial territories help confirm the diagnosis^[8].

Treatment Options

While there is no cure for Takayasu’s arteritis, treatment focuses on controlling inflammation, preventing further damage to arteries, and managing complications. People who have no symptoms may not need treatment, but most patients require medications^[1].

Medications

Corticosteroids are the primary treatment for active Takayasu’s arteritis. Doctors typically start with oral prednisone at a dose of 0.5 to 1 milligram per kilogram of body weight per day for adults. The goal is to gradually reduce this dose over time: below 20 milligrams per day by the end of the third month, and below 0.1 milligrams per kilogram per day by the end of the sixth month. Some patients may be able to stop corticosteroids completely after 24 months if the disease remains controlled^[13].

However, many patients need additional medications to help control the disease and reduce the amount of corticosteroids needed, since long-term steroid use causes significant side effects. These additional medications include^[5][11]:

• Methotrexate
• Azathioprine
• Mycophenolate
• Cyclophosphamide
• Biologic drugs such as tocilizumab (which blocks a substance called interleukin-6 that drives inflammation) or TNF inhibitors like adalimumab or infliximab

Research shows that tocilizumab can help patients achieve clinical remission and reduce their need for corticosteroids, though symptoms may return if the medication is stopped^[11].

Low-dose aspirin (75 to 300 milligrams per day for adults) may be prescribed to prevent blood clots, particularly in patients with narrowed arteries to the brain^[5][13]. If high blood pressure develops, medications to control blood pressure are essential and should be started promptly^[13].

Surgical Treatments

Some patients need surgical procedures to restore blood flow through severely narrowed or blocked arteries. Options include^[9]:

• Angioplasty and stenting: A doctor threads a catheter with a balloon to the narrowed area, inflates the balloon to widen the artery, and may place a small mesh tube (stent) to keep it open
• Bypass surgery: Surgeons create a new route for blood to flow around blocked arteries using a blood vessel taken from another part of the body

Surgery is most successful when performed after inflammation has been controlled with medication. Surgery during active inflammation carries a higher risk of complications and repeat procedures^[3].

Managing Corticosteroid Side Effects

Because corticosteroids cause bone loss, patients should take vitamin D and calcium supplements to help prevent osteoporosis. Doctors also monitor for other side effects including weight gain, high blood sugar, and increased infection risk. Seasonal flu and pneumococcal vaccines are recommended^[13].

Living With Takayasu’s Arteritis

Takayasu’s arteritis is a chronic disease that requires long-term management. Many patients experience periods when the disease is quiet (remission) alternating with periods when symptoms return (relapse), even with treatment^[1].

Regular Medical Care

Ongoing monitoring is essential. Patients should keep regular appointments with their doctor to check disease activity through blood tests and imaging studies, assess treatment effectiveness, and watch for complications. Blood pressure should be measured at each visit. Because blocked arteries in the arms can cause falsely low readings, doctors may need to measure blood pressure in the legs instead^[5].

Lifestyle Considerations

Maintaining overall health helps manage Takayasu’s arteritis and reduce the risk of complications. Important steps include^[5]:

• Following a balanced, healthy diet
• Engaging in regular physical activity as approved by the doctor
• Not smoking, as smoking damages blood vessels
• Managing blood pressure and cholesterol levels
• Maintaining a healthy weight

Physical exercise may help improve cardiovascular fitness and overall health in patients with Takayasu’s arteritis, though patients should discuss appropriate activity levels with their healthcare team^[21].

Emotional and Social Support

Living with a chronic disease can be emotionally challenging. Support from family, friends, or support groups can help patients cope with the impact of the disease on daily life. Organizations such as the Vasculitis Foundation provide educational resources and connect patients with others who have similar conditions^[5].

Outlook

The outlook for Takayasu’s arteritis varies widely. Some patients have only mild symptoms and live relatively normal lives with medication. Others may experience severe complications requiring surgery or causing disability. With early diagnosis and appropriate treatment, many people can control the inflammation and prevent serious complications^[2]. The disease can go into remission, though careful monitoring remains necessary throughout life^[1].