Takayasu’s Arteritis

Takayasu’s Arteritis Definition

Takayasu’s Arteritis (TA) is a very rare form of Vasculitis, a condition which causes severe blood vessel inflammation. In this condition, the aorta and its main branches suffer from major damage and inflammation. The aorta is the large artery responsible for carrying blood from the heart to the other parts of human body. In some rare instances, the coronary artery may also be involved.

The disorder initially presents itself through sensorineural hearing loss and bilateral cataracts. The disease can cause narrowing or blockage of the arteries and lead to a disorder named stenoses. It may also cause aneurysms by dilating the arteries. Some recent researches suggest an association between Takayasu’s Arteritis and tuberculosis.

It is also known by the following names:

Picture 1 – Takayasu’s Arteritis

• Aortic Arch Syndrome
• Pulseless Disease
• Takayasu’s Disease
• Nonspecific Aortoarteritis

Takayasu’s Arteritis Causes

The exact factors responsible for this condition are still not known. Scientists believe TA to be an autoimmune disorder. In other words, the disease is supposed to result from a malfunctioning immune system which attacks the arteries, mistaking them for some foreign substance.

Takayasu’s Arteritis Symptoms

Depending on the severity of TA, the symptoms of the disorder can be divided into two stages:

First stage symptoms

These include:

• Rapid weight loss without any obvious reasons
• Fatigue
• Low-grade fever
• Muscle and joint pain

Many patients do not develop these initial symptoms. The symptoms might occur only after the arteries are damaged by the inflammation.

Second Stage Symptoms

These symptoms do not occur until the arteries are significantly narrowed by the inflammation which obstructs the blood, nutrients and oxygen flow to certain tissues and organs. The signs of the second stage comprise of:

• Claudication (pain and weakness in arm or leg with use)
• Dizziness
• Fainting
• Headache
• Memory loss
• High blood pressure
• Visual disturbances
• Difficulty in concentrating
• Different blood pressure in two arms
• Low or absent pulse rate in wrists as the disorder makes it difficult to detect normal pulse due to narrowing of arteries (this arteritis is also referred to as “pulseless disease” due to this symptom)
• Chest pain
• Neck pain
• Anemia
• Shortness of breath (rare), due to high blood pressure in lungs and arteries

Takayasu’s Arteritis Diagnosis

The diagnosis of TA is quite difficult as patients may not display any symptoms during the early stage of the disease. The narrowing of arteries, which may occur months or even years after the onset of the condition, may be its first indication. The other earliest signs of TA, that help to make its diagnosis, include:

• Unexplained increase in blood pressure
• Difficulty in measuring blood pressure on one side of the body
• Varying blood pressure reading in two arms
• Signs of poor blood circulation

Doctors suspect the presence of this arteritis if a patient experiences at least three of the following six diagnostic criteria:

• The onset of the symptoms occurring at the age of 40 or less
• Muscular pain and weakness in arms and legs with movement
• Weak pulse in brachial artery
• Hearing an abnormal sound (referred to as bruit), indicating turbulent blood flow, when a doctor listens to the subclavian artery or aorta with a stethoscope
• Evident signs of aorta or other artery damage on an angiogram
• At least 10 mm Hg difference between the systolic blood pressure readings in the left and right arm of patients

Doctors perform a thorough physical examination and also ask patients detailed questions about the symptoms present. The following diagnostic procedures are used for confirming its presence:

• Blood test
• Angiography
• Magnetic resonance angiography or MRA
• Magnetic resonance imaging or MRI
• CT (computerized tomography) angiography
• Ultrasonography

Unlike other forms of Vasculitis, tissue biopsy is not very useful for making this diagnosis.

Takayasu’s Arteritis Differential Diagnosis

A wide range of disorders are characterized by symptoms similar to those of TA. To confirm the diagnosis, it is important to rule out the possibility of the presence of these conditions – which include:

• Aortic Coarctation
• Giant Cell Arteritis
• Buerger Disease
• Atherosclerosis
• Rheumatoid Arthritis
• Behcet Disease
• Systemic Lupus Erythematosus
• Wegener Granulomatosis
• Sarcoidosis
• Kawasaki Disease

Takayasu’s Arteritis Treatment and Management

Controlling arterial inflammation and preventing any further damage to blood vessels are the main objects of the treatment of TA. Another important goal is to avoid as many long term side effects as possible. The treatment may be difficult as the artery damage may sometimes continue silently even after a patient seems to be recovering. Treatment generally involves certain medications and may require surgical intervention in some cases.

Medications

Some of the medications used for this purpose may have serious side effects. Naturally, a doctor tries to balance the benefits and the potential side effects by adjusting their dosages. The following drugs are used for this treatment:

Corticosteroids

The treatment usually begins with corticosteroids like methylprednisolone (Medrol) and prednisone. In around 50% of the total cases, the patients respond well to these medications.

Cytotoxic drugs

Cytotoxic drugs such as azathioprine (Azasan, Imuran) and methotrexate (Rheumatrex, Trexall), may be necessary if the patient is not responding wells to the corticosteroids. These medicines are beneficial for reducing the blood vessel inflammation.

Transplant medications

In some cases, medicines generally used by people after organ transplantation may work well for treating Takayasu’s Disease. The drugs can help to stop the artery inflammation by suppressing the activity of the immune system.

Surgery

Surgery may be necessary for treating certain cases of the disease where the arteries are severely blocked. The procedures involve opening or bypassing the arteries to allow better circulation.

Bypass surgery

In this technique, the surgeon removes a healthy vein or artery for some part of the patient’s body and attaches it to the damaged artery to restore normal blood flow.

Percutaneous angioplasty

It is performed by threading a tiny balloon into the blocked artery through one blood vessel. The balloon is then expanded for widening the blocked area.

Stenting

Stents are small wire mesh coils that are inserted into the affected area after it is widened by angioplasty. These help in opening the blocked artery and also prevent further narrowing of the blood vessel.

Takayasu’s Arteritis Prognosis

Patients generally get well after receiving proper treatment. Approximately 60% of the individuals get well after being treated with glucocorticoids. However, the risk of recurrence is very high in these cases. The success rate of the immunosuppressive drugs ranges between 40% and 80%.

Takayasu’s Arteritis Complications

Severe cases of TA are associated with a number of complications including:

• Narrowing and hardening of blood vessels
• High blood pressure
• Ischemia of eyes, brain, arms and face
• Ischemic stroke
• Heart attack
• Heart failure
• Inflammation of heart
• Aneurysm
• Transient ischemic attack
• Lung disorders

The medications used for the treatment can cause various side effects, which include:

Picture 2 – Takayasu’s Arteritis Image

• Cataracts
• Obesity
• Abdominal pain
• Slower rate of wound healing
• Thin skin

Patients are not recommended to take these medications during pregnancy as they may lead to serious complications.

Takayasu’s Arteritis Prevention

It is not possible to prevent the condition as its exact etiology is unknown. However, maintaining a healthy diet and performing regular exercises is recommended as they help to prevent various complications that may arise from TA. Proper management of hypertension caused by the syndrome is very important for preventing any further vascular damage.

Takayasu’s Arteritis Life Expectancy

In around 85% of cases, patients survive for at least fifteen years after being diagnosed with the disorder. However, this figure is reduced by 70% in sufferers having serious aorta damage or extremely high blood pressure.

Takayasu’s Arteritis Prevalence

According to the incidence statistics for TA, 2 to 3 among every one million individuals are affected by this type of Vasculitis every year. It can occur in both children and adults (up to 40 years old), but is more common in young people 15 years to 20 years old. Women are more likely to develop the disease compared to men. Aortic Arch Syndrome can affect people from all around the world but has a higher prevalence in Asia.

Takayasu’s Arteritis is a curable but potentially fatal disorder which requires early diagnosis and prompt treatment. Many forums and support groups including the following provide treatment guidelines to help the patients survive with the condition.