Are you aware of Takayasu's Arteritis? Let's talk about it!

Have you ever heard of a disease called Takayasu's Arteritis? The name may sound a bit strange, right? But it's a rare condition that can affect our blood vessels, especially the largest blood vessels that start in the heart and the branches that branch off from it. It's also sometimes called 'pulseless disease.' Today, we'll talk about it in detail, very simply, so that you too can understand it better.

What exactly is Takayasu's Arteritis?

Simply put, Takayasu's arteritis is an inflammation of our blood vessels, which means swelling or inflammation (vasculitis). It especially affects the largest blood vessels in our body. Think about it, the main blood vessel that carries oxygen-rich blood from our heart to the entire body, which we call the 'great aorta' or 'aorta'. This disease mainly affects the aorta, and the other large blood vessels that branch off from it and carry blood to our arms, neck, and brain. Do you know what happens when this inflammation happens? The walls of our blood vessels are damaged. Then some parts of those blood vessels become weak and can bulge like a balloon. We call this an 'aneurysm'. Also, the blood vessels can swell and narrow. Then blood flow is restricted. Sometimes a blood vessel can become completely blocked, called an 'occlusion'. Rarely, this condition can also affect the arteries that supply blood to the heart, intestines, kidneys, and legs.

Who is most affected by this situation?

Takayasu's Arteritis (TAK) is a condition that most often affects young people. It is most common in women between the ages of 20 and 40. Patients are usually between the ages of 15 and 35 when diagnosed. About 80% to 90% of those with the disease are women. Doctors see this condition more often in countries like Japan and people of Mexican, Indian, and East Asian descent. Even in countries like the United States, only two to three new cases per million people are diagnosed each year. This means it is a relatively rare condition.

How does Takayasu's arteritis affect my body?

When blood vessels become narrowed, the amount of blood that reaches the organs and tissues that are supplied by those blood vessels decreases. Think of it like a clogged water pipe, which reduces the amount of water that flows through. But in Takayasu's arteritis (TAK), these changes often happen slowly and gradually. So our bodies have time to get used to carrying blood through alternative routes. These alternative routes are like the little bypasses we use when a main road is blocked. But these bypasses may not always be able to carry as much blood as the original large blood vessel. Normally, the amount of blood that flows past the blockage is enough to supply the tissues with the minimum amount of oxygen and blood they need to survive. But very rarely, if these alternate blood vessels are not working properly, the tissues can die because they are deprived of oxygen and blood.

What are the symptoms of this disease?

Some people with Takayasu arteritis (TAK) may not have any symptoms. However, about half of patients may experience general malaise and weakness. Early symptoms (stage 1): These are the symptoms that appear at the beginning of the disease:

• Fever.
• Feeling very tired ( Fatigue ).
• Muscle and joint pain .
• Food is tasteless .

Later stage symptoms (stage 2): As the disease progresses, symptoms such as:

• Chest pain .
• Difficulty breathing .
• Headache .
• Feeling dizzy.

Symptoms caused by narrowing of blood vessels and reduced blood supply:

• Tiredness, pain, or cramps in the arms and legs.
• Stomach pain due to decreased blood flow to the intestines.
• High blood pressure due to reduced blood flow to the kidneys.
• Stroke (this is very rare).
• Heart attack (this is also rare).

What caused Takayasu's arteritis?

In fact, no one knows the exact cause of Takayasu's arteritis (TAK).. But doctors and researchers think it may be an 'autoimmune condition'. That is, our body's immune system, which fights disease, mistakenly attacks its own healthy tissues. It's as if our own defenses are harming us. Some researchers think that some cases of Takayasu's arteritis may be caused by a gene inherited from both parents. Those parents may not have symptoms, because they only carry one copy of the gene. But if you inherit that particular gene from both parents, you can develop the condition and its symptoms.

How is this disease diagnosed?

Your doctor will diagnose Takayasu's arteritis by looking at several factors together, not just one.

• A complete medical history and physical examination: This is done to rule out other conditions that have similar symptoms to yours. When your doctor uses a stethoscope to examine your blood vessels, they may hear an unusual sound called a 'bruit' inside the blood vessels. When the blood vessels are too narrow, it's hard for blood to flow through them, which is when the sound is heard.
• X-rays: These show where your artery is damaged and the severity of the damage.
• Tests to detect narrowing of blood vessels or aneurysms: There are various testing methods for this.

What are the diagnostic tests?

There are several tests that can help the doctor confirm the diagnosis:

• Magnetic Resonance Imaging (MRI): This is a test that does not use X-rays. The images are created using a large magnet, electromagnetic energy waves, and a computer.
• Computed Tomography (CT) scan: X-rays and computers create images of internal organs, including large blood vessels.
• Angiography: This involves taking X-ray pictures of the inside of your blood vessels. Your doctor inserts a thin, long tube called a catheter into a large artery in your groin or arm and guides it up to the artery that needs to be examined. Then, a special dye (contrast material) is injected through the catheter into the blood vessel. When X-rays are taken, the dye makes the blood vessels show up clearly in the pictures. Sometimes, an MRI can also be used to perform an angiography.
• Positron Emission Tomography (PET scan): In this test, your doctor injects a radioactive substance through a needle into a vein in your arm. As the substance travels through your body, a scanner detects it and creates images.
• Ultrasound: This helps create images of blood vessels using sound waves.

How is Takayasu's arteritis treated?

Medications can control inflammation, or inflammation. However, some people may need surgery to bypass a blocked blood vessel and create a new path for blood to flow. Corticosteroids: Corticosteroids, such as prednisone (e.g., Rayos®, Sterapred®) or prednisolone (e.g., Flo-Pred®, Orapred®), are the most common treatments for Takayasu's arteritis (TAK).

These medications work wonders in eliminating active inflammation in the blood vessels and putting the disease into remission.

Your doctor will gradually reduce the dose of prednisone to the lowest effective dose to minimize side effects. Some people can gradually stop taking the medication without symptoms returning. However, these corticosteroids are not completely effective for some people. Also, more than half of people who take these medications may experience a return of symptoms or a worsening of their condition. Immunosuppressive medications: Doctors also commonly prescribe medications that suppress the immune system, such as:

• Methotrexate (e.g. Rheumatrex®, Trexall®)
• Azathioprine (e.g. Imuran®, Azasan®)
• Mycophenolate (e.g. Cellcept®, Myfortic®)
• Leflunomide
• Cyclophosphamide

When these immunosuppressants are used with prednisone, about 50% of people who previously had flares can have their symptoms resolved and can gradually stop taking prednisone. Tumor-Necrosis Factor (TNF) inhibitors: Some people may also be given medications called TNF inhibitors to help fight inflammation. Examples of these include:

• Etanercept
• Infliximab
• Tocilizumab

Overall, about 25% of people with Takayasu's arteritis cannot fully control the disease without continued medication.

What are the side effects of the treatment?

The medications used to treat Takayasu's arteritis can have some serious side effects. However, other medications can help control these side effects. Side effects can include:

• Decreased resistance to infections.
• Decreased bone mass (osteoporosis).
• Difficulty seeing.
• Seizures.

Corticosteroids start working within a few hours of starting treatment, so you may feel relief quickly.

What other treatments are used?

In some cases, high blood pressure (hypertension) can be caused by narrowing of the arteries to the kidneys. In this case, the doctor can use something like a balloon to widen the narrowed blood vessel, which is called an angioplasty. Or, a bypass operation can be performed to allow blood to flow normally to the kidneys. This may help to normalize blood pressure without taking high blood pressure medication. Some people may have severe problems with narrowed blood vessels in their arms or legs, making it difficult to walk or do other activities. Bypass surgery can also correct these problems. If there is an aneurysm, it can be repaired with surgery or a bypass. Some people with Takayasu's arteritis may also need to have an aortic valve replacement.

How do I take care of myself?

Many people with Takayasu's arteritis also have high blood pressure (hypertension). It's important to control this. If you don't treat high blood pressure, things like this can happen:

• Stroke.
• Heart disease.
• Kidney failure.

If you take medications that suppress your immune system, consider getting these vaccines to protect against infections:

• Flu vaccine.
• Pneumonia vaccine.
• Herpes zoster (Shingles) vaccine.

What can someone with Takayasu's arteritis expect?

Although there is no cure for Takayasu's arteritis, it is a treatable disease. Most people with the disease recover with treatment. However, many people can become disabled to some extent, or rarely completely, due to the disease. The effects of the disease can have a significant impact on daily activities. About half of people with Takayasu's arteritis have to make changes to their jobs. However, about 25% of people with the disease lead completely normal lives. Another 25% have had to make some changes to their activities. People living with this chronic disease need to have regular medical check-ups and, if necessary, changes in their medications. Doctors also need to monitor the side effects of the medications and do blood tests. The following can be complications of Takayasu's arteritis:

• Heart failure.
• Stroke.
• Blood clots.
• A heart attack.

Takayasu arteritis is a long-term disease that develops slowly. You will need long-term treatment, often with medication. In countries like the United States and Japan, about 3% of people with Takayasu arteritis die within an average of five years of the disease. This may be because doctors are able to diagnose the disease early and treat it correctly. In other parts of the world, the outcome can be worse because of delays in diagnosis and treatment or poor access to health services. If left untreated, Takayasu arteritis can be fatal.

How do I take care of my health?

It's important to keep track of how you're feeling and stay in regular contact with your doctor so you can catch any new or worsening symptoms early.

When should I see a doctor?

If your Takayasu arteritis symptoms come back during or after treatment, see your doctor right away. If you are planning to become pregnant, talk to your doctor about it. It is possible to have a successful pregnancy with Takayasu arteritis, but it is important to know how to manage it.

When should I go to an Emergency Treatment Unit (ETU) ?

If you have any of these symptoms, call 911 immediately (in Sri Lanka, call the 1990 Suwaseriya Ambulance Service or go to the nearest hospital):

• Chest pain.
• Difficulty breathing.
• Weak pulse.
• Symptoms of a heart attack (e.g., severe chest pain that radiates down the arm, sweating, nausea).
• Signs of a stroke (e.g., drooping on one side of the face, numbness in an arm, difficulty speaking).

What questions should I ask my doctor?

• How long will I have to take medication for Takayasu's arteritis?
• How often do I need to come for follow-up appointments?
• Can my condition be controlled without surgery?
• How often do I need to have imaging tests?

Finally, what to remember (Take-Home Message)

Because Takayasu's Arteritis is a long-term, or 'chronic', condition, you will need to see a doctor regularly. Over time, your symptoms may go from being under control to being under control. That's why it's important to stay in touch with your doctor regularly. Don't be afraid to tell your doctor if you notice any changes in your health. Your doctor will want to know about it, because that's when he or she can help you.

Remember, even with this disease, with proper treatment and following medical advice, you too can live a good life. Stay strong!

Takayasu's arteritis, Takayasu's arteritis, pulseless disease, vasculitis, aorta, arteritis, blood vessel disease, high blood pressure, autoimmune disease