What is Takayasu Arteritis?

Takayasu arteritis, also known as pulseless disease, is a disease that causes inflammation throughout the body, primarily damaging the medium and large-sized arteries and their branches. This condition tends to mainly affect young Asian women. The disease often impacts the aorta (the main blood vessel in the body) and its major branches like the renal arteries (leading to kidneys), carotid arteries (leading to the brain), and subclavian arteries (going to the upper limbs), resulting in narrowing, blockages, or ballooning (aneurysms) of these large blood vessels.

There are signs that the disease may be related to an abnormal response from the body’s immune system, specifically its cell-mediated immunity. However, the root cause of Takayasu arteritis remains mostly unknown. Diagnosing this disease mainly relies on suspecting its presence based on signs and symptoms and confirming it through specific vascular imaging tests (arteriograms).

Typically, the initial treatment involves medications such as corticosteroids to reduce inflammation. However, lately, more people are undergoing surgical procedures to manage this disease. This shift to surgery is due to observations that medication alone often doesn’t completely stop the disease progression and that the disease tends to recur at quite high rates.

What Causes Takayasu Arteritis?

It’s unclear what exactly causes a condition known as Takayasu arteritis. This condition is a type of inflammation disease that affects your arteries, the vessels that carry blood from your heart to the rest of your body. The general belief is that this condition might be triggered by an issue with our body’s immune system, which is the body’s defense mechanism against illnesses.

Eventually, this inflammation results in a thickening of the walls of your arteries, making it tougher for blood to flow through. This can lead to reduced blood flow to certain parts of the body, a condition we call ischemia. It can also cause the formation of pseudoaneurysms, which are false bulges in the artery walls that can potentially be dangerous if they rupture.

Risk Factors and Frequency for Takayasu Arteritis

Takayasu arteritis is a rare disease, mostly found in young adults, especially females. Its cases worldwide are quite low, about 1 to 2 individuals out of a million. Even though it is more typical in the 40 to 50 age bracket, it can occur in very young kids and older adults as well. This disease is more commonly seen in people from Asian or Mexican backgrounds and is not very common in North America.

Takayasu arteritis is a rare condition.
The global rate of occurrence is just 1 to 2 cases per million people.
It affects females much more than males, at a ratio of 9:1.
It’s usually seen in people aged 40 to 50, but it can also affect young children and the elderly.
The disease is mostly found in individuals of Asian or Mexican descent.
Incidence is rare in North America.

Signs and Symptoms of Takayasu Arteritis

Takayasu arteritis, often referred to as “pulseless disease”, starts with very general symptoms such as fever, fatigue, muscle pain, weight loss, and loss of appetite. These symptoms are common in many illnesses, which is why they’re not specific to this disease. Unfortunately, most people don’t realize they have Takayasu arteritis until more severe symptoms show up. These are usually due to impaired blood flow and can include high blood pressure due to narrowed kidney arteries, neurological changes from blockage of the carotid artery in the neck, or arm discomfort due to reduced blood flow to the upper body. Because of these late-stage symptoms, diagnosis is often delayed. As the disease progresses, it can result in extremely poor blood circulation in the upper or lower body, leading to weak or absent pulses, or possibly a vascular sound called a bruit. Other noteworthy late findings may involve accelerated hardening of the arteries or heart failure.

Fever
Fatigue
Muscle pain
Weight loss
Loss of appetite
High blood pressure (from narrowed kidney arteries)
Neurological changes (from blockage of the carotid artery)
Arm discomfort (from reduced blood flow to the upper body)
Weak or absent pulses
A vascular sound called a bruit
Possible accelerated hardening of the arteries
Heart failure

Testing for Takayasu Arteritis

To diagnose a condition called Takayasu arteritis, your doctor needs to be on high alert. They’ll collect a detailed history of your symptoms and conduct a thorough physical checkup. Imaging tests are also very important in the diagnostic process.

CT angiography (CTA) is now generally the first step used to assess how widespread the disease might be. Previously, doctors used conventional angiography. These standards come from the 1994 Tokyo International Conference Classification. CTA allows doctors to see if the walls of your vessels are thickening and if the space inside your vessels is narrowing.

Magnetic resonance imaging (MRI) and MR angiography (MRA) might also be used for further identification and provide detailed images from multiple angles without the use of ionizing radiation. However, these techniques sometimes overestimate the degree of the disease, and it can be harder to access an MRI or MRA.

Treatment Options for Takayasu Arteritis

If you have Takayasu arteritis, which is a form of inflammation in your body’s large arteries, the first line of treatment is usually a type of anti-inflammatory medication known as corticosteroids. Doctors might also use other drugs that help to suppress the immune system either alongside or instead of corticosteroids. However, there’s no clear evidence that these drugs are better than corticosteroids alone.

Revascularization, a procedure to restore blood flow to the arteries, has always been known to be beneficial for treating arterial diseases, but because the course of Takayasu arteritis was so uncertain, doctors used to be hesitant about using this method as the primary treatment. Traditionally, surgeries were reserved for cases where arterial blockages – an effect of the disease – persisted even after treating with corticosteroids or immunosuppressants. The thinking on this is changing though, as some have started questioning the effectiveness of medication in stopping the disease.

Recent research has shown that half of the people with Takayasu arteritis will have the disease come back and cause complications related to blood vessels within ten years of being diagnosed. You would be most likely to see the disease return if you are a male or if you have a high level of C-reactive protein which is a marker of inflammation in the body.

Therefore, surgical procedures to restore blood flow are becoming a more common treatment choice. In the past, doctors have tried a procedure called transluminal angioplasty where a small balloon is used to open up narrowed or blocked arteries. However, the effects of this procedure don’t last in the long run because of the fibrous nature of the blocked arteries in Takayasu arteritis patients. Most patients treated with this method had their arteries narrow again within around three years and almost all needed another procedure at some point. Therefore, this process is mainly used as a quick fix to relieve symptoms when dealing with other medical issues.

Another surgical option is to rebuild the affected arteries. The technique used for this depends on where the disease has affected your arteries and your body’s specific structure. Doctors will typically use either Dacron or a material called polytetrafluoroethylene for major reconstructions of the aorta, the largest artery in your body, and a vein from your leg (saphenous vein) for rebuilding arteries in your extremities or to your kidney or intestines. It’s important to note that using your own leg vein could lead to an abnormal bulge (aneurysm) over time. Also, the procedure risks early failure when the graft, the material used to rebuild the artery, is attached to an artery affected by the disease.

Surgical treatment is generally well accepted because most people diagnosed with Takayasu arteritis are young. The surgery often leads to dramatic improvements in symptoms, like reducing or even curing high blood pressure or resolving difficulties with blood flow to the upper limbs. Given these results, it is expected that surgical correction of symptoms caused by Takayasu arteritis will become even more common.

When it comes to medical conditions that may affect our body’s arteries, there’s quite a considerable list, which includes:

Aortic Coarctation (a narrowing of the large blood vessel that leads from the heart)
Atherosclerosis (hardening and narrowing of the arteries)
Behcet’s Disease (causes blood vessel inflammation throughout your body)
Giant Cell Arteritis (inflammation of the lining of the arteries)
Ig-G4 Related Disease (inflammation that can affect various parts of the body)
Kawasaki Disease (a disease that causes inflammation in the walls of some blood vessels in the body)
Rheumatoid Arthritis (an autoimmune disease that can cause joint pain and damage throughout your body)
Sarcoidosis (collection of inflammatory cells that form lumps known as granulomata)