Takayasu Disease

Takayasu’s arteritis, named after Dr. Mikoto Takayasu, who first described it in 1908, is a rare condition characterized by inflammation that damages large and medium-sized arteries. The most commonly affected arteries are the branches of the aorta, which supplies blood to the arms and travels through the neck to reach the brain. The aorta itself is frequently involved as well. Although less common, arteries supplying blood to other vital organs like the heart, intestines, kidneys, and legs may also be affected.

Arteritis refers to the inflammation of arteries, the blood vessels responsible for carrying blood from the heart to the rest of the body. It is closely related to vasculitis, which involves inflammation of blood vessels in general, including both veins and arteries

Who Gets Takayasu’s Arteritis?

• Most often, young women (20–40 years old)
• People of Asian descent
• More common in Japan and Mexico

What happens during Takayasu’s arteritis?

The inflammation damages your arteries, weakening and stretching them. This can lead to aneurysms (bulges) or blockages that reduce blood flow. Think of a kinked hose; the water flow weakens or stops completely. The lack of blood flow can cause various problems, depending on which arteries are affected.

Symptoms of Takayasu’s Arteritis

While some individuals with Takayasu’s arteritis may not experience any symptoms, approximately half of those affected will exhibit a general sense of illness.

Early-stage symptoms of Takayasu’s arthritis include:

• Fever
• Fatigue
• Muscle or joint pain
• Loss of appetite

Later-stage symptoms may include:

• Chest pain
• Shortness of breath
• Headaches
• Dizziness

Narrowing of blood vessels and reduced blood supply can lead to:

• Fatigue, pain, or aching in the arms and legs
• Abdominal pain due to reduced blood flow to the intestines
• High blood pressure resulting from decreased blood flow to the kidneys
• Stroke (rare occurrence)
• Heart attack (rare occurrence)

Causes of Takayasu’s Arteritis

The exact cause of TAK remains unknown. It’s believed to be related to an autoimmune reaction, where the immune system mistakenly attacks healthy tissues it should leave alone.

Researchers suspect that in some instances, Takayasu’s arteritis may be linked to a specific gene inherited from both parents. Individuals who carry only one copy of this gene may not exhibit symptoms themselves. However, inheriting this gene from both parents can lead to the development of the condition and its associated symptoms.

Diagnosis and Tests

How is Takayasu’s arteritis diagnosed?

Your healthcare provider’s diagnosis of Takayasu’s arteritis relies on various factors, including:

• Complete medical history and physical examination to rule out other conditions with similar symptoms. Your provider may detect an unusual sound known as a bruit using a stethoscope. This sound can occur when there’s significant narrowing in the blood vessels, making it difficult for blood to flow smoothly.
• X-rays to visualize areas of artery damage and assess its severity.
• Procedures to identify blood vessel narrowing or the presence of aneurysms.

Tests to diagnose Takayasu’s arthritis

Your healthcare provider will employ several diagnostic tests, including:

• Magnetic resonance imaging (MRI): This imaging test uses a powerful magnet, electromagnetic energy waves, and a computer to create detailed images without the use of X-rays.
• Computed tomography (CT scan): Doctors will utilize X-rays and computer technology to produce images of internal organs, including the large blood vessels.
• Angiography: X-ray imaging of the interior of blood vessels. A catheter is inserted into a large artery in your groin or arm, then guided to the targeted artery. Contrast material is injected through the catheter to highlight the blood vessels, allowing for visualization on X-ray images. Alternatively, an MRI may be used for angiography.
• Positron emission tomography (PET): Doctors insert a radioactive substance into your body, and a scanner detects its movement to create images.
• Ultrasound: This imaging technique uses sound waves to generate images of your blood vessels.

Treatment

Steroids for Initial Relief:

• Doctors first use steroid medications, like prednisone, to quickly reduce inflammation.
• These medications often work within hours and can significantly improve symptoms.
• However, they might not completely control the disease for everyone.

Balancing Improvement with Side Effects:

• Once the disease seems under control, doctors slowly lower the steroid dosage.
• This aims to maintain the positive effects while minimizing side effects from long-term steroid use.
• In some cases, patients can eventually stop taking steroids altogether without a relapse.

Additional Treatments for Long-Term Control:

• Unfortunately, about half of patients experience relapses as the steroid dose is lowered.
• To prevent this, doctors may prescribe other medications along with steroids.
• These medications, called immunosuppressants, help control the overactive immune system that causes inflammation.
• With this combined approach, around 50% of patients who previously relapsed can achieve remission (disease under control) and eventually stop taking steroids altogether.

Blood pressure management is crucial.

• Many Takayasu’s arteritis patients also have high blood pressure.
• Carefully managing blood pressure is vital to prevent complications like stroke, heart disease, and kidney problems.

Restoring Blood Flow and Addressing Blockages:

• In some cases, narrowed arteries can affect kidney function.
• Procedures like angioplasty (opening blocked arteries) or bypass surgery (creating a detour for blood flow) might be needed.
• This can restore normal blood flow and potentially eliminate the need for blood pressure medications.
• For patients with significant narrowing in the arteries supplying limbs, bypass surgery can improve function and mobility.
• Additionally, surgery can repair weak spots in arteries called aneurysms.

Takayasu’s arteritis and Pregnancy

While women with Takayasu’s arteritis can have successful pregnancies, the condition and its treatment may impact fertility and pregnancy outcomes. Prior to conception, it’s important for women with Takayasu’s arteritis to work with their doctors to develop a plan to minimize pregnancy complications. Regular prenatal check-ups are essential to monitor and manage the condition throughout pregnancy.

Complications

Takayasu’s arteritis involves cycles of inflammation and healing within the arteries, which can lead to various complications, including:

• Hardening and narrowing of blood vessels, resulting in reduced blood flow to organs and tissues.
• High blood pressure is often due to decreased blood flow to the kidneys.
• Inflammation of the heart, affecting the heart muscle or valves.
• Heart failure is caused by high blood pressure, heart inflammation, leakage of blood back into the heart through the aortic valve, or a combination of these factors.
• A stroke stems from decreased or blocked blood flow in the arteries supplying the brain.
• A transient ischemic attack (TIA), also known as a ministroke, acts as a warning sign for a potential stroke without causing permanent damage.
• Aneurysm formation in the aorta occurs when weakened vessel walls bulge, posing a risk of rupture.
• Heart attack, resulting from decreased blood flow to the heart.
• Aortic aneurysm and dissection: A weak spot in the aorta may bulge (aneurysm), increasing the risk of aortic dissection—a tear in the aortic lining.

Living with Takayasu’s arteritis

• While there’s no cure, most people improve with treatment.
• Regular checkups and medication adjustments are crucial.
• High blood pressure management is important to prevent complications.
• A healthy pregnancy is possible, but close monitoring is needed.

Source:

• Takayasu’s Arteritis Research Association
• Mayo Clinic
• Cleveland Clinic