Takayasu Arteritis - A Rare Form of Inflammatory Vasculitis

What Is Takayasu Arteritis?

Takayasu arteritis is an inflammatory vascular disorder of the largest blood vessels of the body. It is an uncommon form of systemic inflammatory vascular disorder. It affects the aorta (the largest blood vessel supplying the tissues of the body from the heart) and the arterial branches of the aorta that bring purified blood from the heart to the body tissues. The arteries of the head, neck, upper arms, and shoulders are mainly affected by the disorder. Arteries that supply blood to the heart, gastrointestinal tract, kidneys, and lower extremities are less commonly involved. The inflammation damages the walls of the blood vessels making them weak and bulging out which may result in an aneurysm. Sometimes the blood vessels may become completely occluded resulting in an interrupted blood supply to the tissues of the body.

What Are the Synonyms of Takayasu Arteritis?

• Takayasu disease.

• Pulseless disease.

What Is the Epidemiology of Takayasu Arteritis?

• It mainly affects children.

• It is more common in females than males.

• Mainly the people falling in the age group of 20 to 40 years are affected by the disease.

• It is more commonly seen in the people of Japan and in the people of Mexican, Indian, and East Asian origin.

What Is the Etiology of Takayasu Arteritis?

The etiology of Takayasu arteritis is unknown.

• Autoimmune Reaction - Some researchers believed it to be an autoimmune condition (where the immune cells of the body start attacking the normal healthy cells of the body).

• Hereditary Factors - It is also believed to have a genetic background in some cases. It is believed to be an autosomal recessive disorder.

What Is the Pathophysiology of Takayasu Arteritis?

It is characterized by the granulomatous inflammation of all three layers of the arteries resulting in fibrosis of the blood vessels with the narrowing of the lumen. These changes will lead to the weakening and stretching of the arterial walls sometimes resulting in a balloon-like bulge in the large and medium-sized arteries. This condition is called arterial aneurysm which will decrease the blood supply to the body organs supplied by the artery.

Sometimes the blocking of the blood vessels also takes place resulting in reduced blood supply. The changes that are occurring are gradual, eventually leading to increased blood pressure, cardiac failure, or even stroke. In rare cases, the tissue that is not receiving enough blood and oxygen may also die.

What Are the Symptoms of Takayasu Arteritis?

The symptoms of the disease usually occur in two stages:

Stage 1: The early symptoms of Takayasu’s arteritis include the following:

• Fever.

• Generalized illness.

• Tiredness.

• Muscle or joint pain.

• Less appetite.

Stage 2: The symptoms that develop at a later stage of Takayasu’s arteritis include the following:

• Pain in the chest.

• Breathlessness.

• Headache.

• Dizziness or fainting.

Narrowing of the arteries and decreased blood supply to the body tissues can cause:

• Extreme pain, fatigue, and weakness in the upper and lower extremities.

• Abdominal pain due to reduced blood flow to the intestines.

• High blood pressure.

• Stroke in rare cases.

• Heart failure in rare cases.

What Are the Diagnostic Tests for Takayasu Arteritis?

The diagnosis of the disease will require the following:

• Complete Medical History- The healthcare professional should thoroughly examine the patient to rule out another disease with similar clinical symptoms.

• Physical Examination- The doctor might hear an unusual sound called a bruit while examining the patient through their stethoscope. The sound is created because of the blood flow, which is reduced due to the narrowing of the blood vessels.

• X-Rays- They are used to check where the artery is damaged and the amount of damage done.

• Magnetic Resonance Imaging- It is a test that uses electromagnetic energy waves to produce images of the pathology on the computer.

• Computed Tomography- It is also used to view the images of internal organs like the blood vessels.

• Angiography- It is the procedure to examine the X-ray images of the internal part of the blood vessels. A long, slender catheter tube is inserted into a large artery in the arm or the groin region by the doctor and attached to the artery. The catheter is then used to inject the contrast dye into the blood vessel to take the X-ray images. This contrasting material helps to differentiate the pathological blood vessel from other blood vessels in the X-ray images.

• Positron Emission Tomography- A radioactive substance is injected through a needle into the arm of the patient. This substance is detected by a scanner while traveling through the body to form the images.

• Ultrasound- Here, the sound waves are used to produce images of the blood vessels.

What Is the Treatment of Takayasu Arteritis?

The healthcare professional should use drug and surgical therapy to treat the patient.

Drug Therapy:

• Corticosteroids - They are most commonly used for the management of the inflammatory symptoms of the disease. The dose of the steroids can be reduced gradually. They will show their effect within one hour of administration.

• Immunosuppressive Drugs - These have also been prescribed by the doctor to modulate the immunity of the host.

• Tumor Necrosis Factor Inhibitors - They may also be used by some patients to suppress inflammation.

Surgical Therapy:

• Angioplasty - It is a surgical procedure used to broaden the blocked or narrowed arteries.

• Bypass Surgery - The blood is redirected around a section of a blocked or narrowed artery in the heart. A healthy blood vessel is taken from the legs, arms, or chest and connected below and above the blocked arteries in the heart.

• Aortic Valve Replacement - People suffering from Takayasu arteritis may sometimes require an aortic valve replacement.

How to Take Care of Oneself?

• People suffering from Takayasu arteritis usually develop high blood pressure, which is crucial to manage and control.

• If the blood pressure is not maintained, it may lead to serious complications such as stroke, heart and kidney failure, and diseases of the heart.

• If one is taking immunosuppressants, chances are there that they may contract the infections very easily. So it is important to get vaccinated.

Conclusion:

Takayasu arteritis is a chronic inflammatory disease of the blood vessels. Therefore, regular health checkups are needed with the doctor to manage the disease. The symptoms will come and go, so it is important to check one’s health. Long-term treatment is needed to manage the disease well.