WHAT IS TAKAYASU’S ARTERITIS?

Takayasu's arteritis is a condition in which the walls of the biggest arteries of the body become inflamed. This condition is a form of vasculitis that is also called occlusive thromboaortopathy and it always affects the aorta and vessels branching off the aorta. The aorta is the main artery of the body that carries blood from the heart. It is an important part of the cardiovascular system and branches to supply blood to the head, arms, thorax, and abdomen. Takayasu's arteritis is an uncommon illness that can have serious consequences because it can lead to aneurysms, heart attacks, and strokes.

What is the cause of Takayasu's arteritis?

The cause of this arteritis is thought to be an autoimmune reaction but some believe a viral infection can also act as a trigger. Interestingly, some patients with Takayasu's arteritis also have ulcerative colitis. This is an inflammatory condition of the colon. The colon is the large intestine and part of the digestive system).

Takayasu's arteritis occurs in families suggesting that a genetic component exists. Recent work suggests that mutations of certain genes are involved in the development of Takayasu's arteritis. These include the following:

• A mutation of the human leukocyte antigen (HLA) gene, which is involved in the development of the arteritis.
• There are also certain alterations of genes that code for immunoglobulin receptors that have been implicated.

Immunoglobulins are proteins made by white blood cells, which are an important part of the immune system response. Genetic mutations can result in a flawed immune system that results in excess inflammation. This appears to be the case with Takayasu’s arteritis.

The condition is most common among females under 40 years of age and is more often diagnosed in people from the Far East. The illness is not common but can occur in males as well as females, just at a lower rate.

Takayasu's arteritis symptoms

There are several symptoms that a person can have that can indicate they have Takayasu's arteritis.  Many of these symptoms are also typical of other illnesses so a visit to the doctor is recommended.

The most common symptoms of Takayasu's arteritis are described below.

• Hypertension: High blood pressure is when there is increased systolic over diastolic pressures, indicating the greater force of the blood moving through the blood vessels.
• Pulmonary hypertension: This is when blood pressure within the lungs is elevated.
• Angina: chest pain
• Headaches
• Weight loss
• Fever
• Night sweats
• Dizziness and fainting
• Pain in the intestines and stomach
• Painful arms and legs: This is most noticeable when the person walks or lifts their limbs.

Takayasu's arteritis complications

There are several complications that occur in people who have Takayasu's arteritis. These can be disabling or fatal. Complications include the following:

• Heart attack: Coronary arteries, which supply the heart muscle can become blocked which can result in myocardial infarction (heart attack).
• Transient Ischemic Attack:  This is a stroke that is temporary and it may mean a real stroke is imminent.
• Stroke: A stroke (cerebrovascular accident) can happen with this arteritis because it can lead to blood clots that block carotid arteries that supply the brain with blood.
• Aneurysms; Aortic aneurysm is likely; this is when the wall of the aorta bulges outwards. If this bursts the person is likely to die.
• Kidney damage and failure: The elevated blood pressure of patients with Takayasu's arteritis can damage the kidney eventually causing the organ to fail.

Takayasu's arteritis diagnosis

A physical exam needs to be done and blood pressure recorded. There are also some imaging tests that can help with a diagnosis. The methods used to diagnose Takayasu's arteritis are given below:

• Blood pressure readings of all the limbs. The values will differ among limbs if arteritis is a problem because blood flow is often compromised due to narrowed areas.
• Magnetic resonance angiography: This is when a contrast dye is injected into the blood vessels and an MRI is done to better visualize the blood vessels to check for structural abnormalities.
• Computer tomography angiography: This also involves using contrast but the imaging machine is CT, not MRI.
• Ultrasonography with contrast dye. This can indicate the blood vessel structure and any narrowed or blocked areas.

Imaging techniques in people with Takayasu's arteritis often show narrowed regions in the arteries as well as blocked regions and bulging areas (indicative of aneurysms).

The occurrence of a cardiac event like a heart attack or a stroke without any risk factors may make a doctor suspect this condition.

Takayasu's arteritis treatment

The illness is a chronic condition that cannot be cured but can be managed. Treating the inflammation is an important part of managing this condition. It is the inflammatory response of the immune system that causes much of the structural damage to the blood vessels and increases the likelihood of an aneurysm or stroke.

Some of the treatments become necessary after complications such as heart attack and stroke have occurred. Treatment options are listed and described below.

• Corticosteroid medications: Examples that are used include prednisone; this helps control the inflammation that causes a lot of the damage to the blood vessels.
• Immunosuppressant medicines: The most commonly used immunosuppressants for this condition include the use of methotrexate or azathioprine.
• Anti-tumor necrosis factor medications: Rituximab and tocilizumab can be used because they help reduce the inflammatory response typical of this disease.
• Stent grafts and angioplasty: This is needed when vessels have become too narrow resulting in compromised blood flow.
• Coronary artery bypass: Sometimes surgery is needed to bypass coronary arteries that are blocked and where a stent or angioplasty will not work.
• Anti-platelet medication: This may be needed for patients who begin to develop blood clots.

Conclusion

Takayasu's arteritis is an uncommon form of vasculitis in which large blood vessels are inflamed. The illness is thought to be an autoimmune condition that has a genetic component. The illness, although quite rare, needs to be diagnosed quickly before severe complications such as heart attacks or aneurysms occur. Treatment usually begins by decreasing the immune response and controlling the inflammation that leads to blood vessel damage.

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Sources

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