Definition

Takayasu arteritis is a type of inflammation that affects large arteries, primarily targeting the major arteries, particularly the aorta (the body's largest artery that comes directly from the heart) and its main branches. This condition can lead to complications in blood vessels and often requires medication to control its symptoms.

Causes

The exact cause of Takayasu Arteritis is unknown. It is classified as a type of vasculitis (inflammation of blood vessels), which is suspected to involve an autoimmune process where the immune system attacks the body’s own cells. The disease may be triggered by viral infections or other infections.

Inflammation in the arteries causes thickening, narrowing, and scarring of the blood vessels. These changes can lead to blockages, making it harder for blood to flow smoothly. Consequently, tissues in the body may receive insufficient oxygen to function properly.

Takayasu Arteritis also causes damage to the elastic fibers in blood vessels. This can cause the walls of blood vessels to thin and weaken, allowing them to form pockets (aneurysms). These aneurysms are easily ruptured and, because they are located in large arteries, can result in significant blood loss.

Risk Factor

Takayasu arteritis is extremely rare, affecting 1-2 people per million. It most often occurs in women under the age of 40. While it occurs globally, it is most commonly seen in Asia. Some cases may run in families, and studies suggest that certain genes increase the risk of developing this disease.

Symptoms

Takayasu arteritis symptoms typically occur in two stages. In the first stage, you may experience the following:

• Fatigue
• Unexplained weight loss
• Joint and muscle pain
• Mild fever, often accompanied by night sweats

However, not everyone experiences these symptoms. In some cases, the inflammation can damage the arteries over several years before the disease is even noticed.

In the second stage, inflammation narrows the arteries, drastically reducing blood flow. This can lead to organs not receiving enough oxygen and nutrients to function properly. Symptoms during this stage may include:

• Weakness or pain in the arms and legs during use
• Weak pulse, difficulty measuring blood pressure, or significant differences in blood pressure between the arms
• Lightheadedness, dizziness, or fainting
• Headache or vision problems
• Memory issues or difficulty thinking
• Chest pain or shortness of breath
• High blood pressure
• Diarrhea or blood in stool
• Anemia (low red blood cell count)

Diagnosis

Diagnosing Takayasu arteritis is challenging. The doctor will begin with a detailed history and physical examination. If Takayasu Arteritis is suspected, further laboratory and imaging tests may be required.

Laboratory tests may include a complete blood count, immune tests, protein analysis, and urinalysis. A complete blood count may show nonspecific results such as an elevated erythrocyte sedimentation rate (ESR), increased white blood cell count, and reduced red blood cells. However, these results can be seen in other diseases too. Immune tests may include the detection of autoantibodies such as rheumatoid factor, antinuclear antibodies, anticardiolipin antibodies, and antineutrophil cytoplasmic antibodies (ANCAs). Yet, these tests are also not specific to Takayasu Arteritis. Protein tests like albumin and globulin may show abnormalities due to kidney damage. Urinalysis may resemble nephrotic syndrome, a condition where the kidneys lose excessive protein.

Imaging is crucial in diagnosing Takayasu Arteritis. One common method is CT (computed tomography) angiography, which uses contrast dye to highlight blood vessels on X-rays and show thickening and narrowing of the vessel walls. MRI or MRA (magnetic resonance imaging/angiography) is another option, which uses magnetic waves instead of radiation, though it may not be as accessible as CT angiography.

Management

Treatment for Takayasu Arteritis typically begins with medications to reduce inflammation. These medications may include corticosteroids or other immunosuppressive medications.

Other treatment options may involve surgery to reopen narrowed blood vessels, though this is generally reserved for cases that do not respond to medication. Surgical options may also involve reconstructing damaged arteries. Depending on the location of the affected arteries, synthetic materials or other blood vessels may be used. Surgery is often considered to improve blood flow to organs such as the intestines or kidneys. Another common procedure is bypass surgery, which reroutes blood around a blocked artery, often done to restore blood flow to the brain and arms.

Surgery typically yields favorable outcomes in patients with Takayasu arteritis due to their relatively young average age.

Complications

Takayasu Arteritis may recur in cycles of inflammation. Long-term complications include:

• Stiff, narrowed arteries, reducing blood flow to organs
• High blood pressure due to reduced blood flow to the kidneys
• Heart inflammation, affecting the muscles and valves
• Heart failure caused by a combination of high blood pressure, heart inflammation, and valve damage
• Stroke from blocked or reduced blood flow to the brain
• Transient ischemic attack (TIA), also known as a mini-stroke, which usually resolves within 24 hours but signals reduced blood flow to the brain
• Aortic aneurysm, where the vessel wall weakens and bulges, risking rupture
• Heart attack due to reduced blood flow to the heart muscles

Prevention

Since Takayasu Arteritis is an extremely uncommon condition and its exact cause is still unknown, prevention is challenging. Regular checkups in accordance with doctor's orders are the most likely way to prevent complications from Takayasu Arteritis. You can consult your doctor more easily if you have Takayasu Arteritis and receive routine checkups. This way, the doctor can treat complications before they worsen.

When to See a Doctor?

You should seek medical attention immediately if you experience shortness of breath, chest pain radiating to the arm, neck, back, or jaw, or stroke symptoms such as weakness on one side, slurred speech, or facial drooping. If you have symptoms similar to those listed in the Symptoms section, consult a doctor, as early detection can improve the chances of effective treatment for Takayasu Arteritis.

If you have already been diagnosed with Takayasu Arteritis, keep in mind that symptoms can come and go even after treatment. Be alert for any recurring or new symptoms and notify your doctor promptly.

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