Takayasu arteritis

Other Names: TA; Young female arteritis; Takayasu disease; Pulseless disease; Aortic arch syndrome Takayasu arteritis is a condition that causes inflammation of the main blood vessel that carries blood from the heart to the rest of the body (aorta) and its associated branched blood vessels. As a result of the inflammation, the blood vessel walls become thick and make it difficult for blood to flow. Over time, impaired blood flow causes damage to the heart and various other organs of the body.

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Causes

The cause of Takayasu arteritis is not known. The disease occurs mainly in children and women between the ages of 20 to 40. It is more common in people of East Asian, Indian or Mexican descent. However, it is now being seen more often in other parts of the world. Several genes that increase the chance of having this problem were recently found. Takayasu arteritis appears to be an autoimmune condition. This means the body's immune system mistakenly attacks healthy tissue in the blood vessel wall. The condition may also involve other organ systems. This condition has many features that are similar to giant cell arteritis or temporal arteritis in older people.

Signs and symptoms

Symptoms may include:

• Arm weakness or pain with use
• Chest pain
• Dizziness
• Fatigue
• Fever
• Lightheadedness
• Muscle or joint pain
• Skin rash
• Night sweats
• Vision changes
• Weight loss
• Decreased radial pulses (at the wrist)
• Difference in blood pressure between the two arms
• High blood pressure (hypertension)
• There may also be signs of inflammation (pericarditis or pleuritis).

Diagnosis

There is no blood test available to make a definite diagnosis. The diagnosis is made when a person has symptoms and imaging tests show blood vessel abnormalities suggesting inflammation. Possible tests include:

• Angiogram, including coronary angiography
• Complete blood count(CBC)
• C-reactive protein (CRP)
• Electrocardiogram (ECG)
• Erythrocyte sedimentation rate (ESR)
• Magnetic resonance angiography (MRA)
• Magnetic resonance imaging (MRI)
• Computed tomography angiography (CTA)
• Positron emission tomography (PET)
• Ultrasound
• X-ray of the chest

Treatment

Treatment of Takayasu arteritis is difficult. However, people who have the right treatment can improve. It is important to identify the condition early. The disease tends to be chronic, requiring long-term use of anti-inflammatory medicines.

MEDICINES Most people are first treated with high doses of corticosteroids such as prednisone. As the disease is controlled the dose of prednisone is decreased. In almost all cases, immunosuppressive drugs are added to reduce the need for long-term use of prednisone and yet maintain control of the disease. Conventional immunosuppressive agents such as methotrexate, azathioprine, mycophenolate, cyclophosphamide, or leflunomide are often added. Biologic agents may also be effective. These include TNF inhibitors such as infliximab, etanercept, and tocilizumab.

SURGERY Surgery or angioplasty may be used to open up narrowed arteries to supply blood or open up the constriction. Aortic valve replacement may be needed in some cases.

Prognosis

This disease can be fatal without treatment. However, a combined treatment approach using medicines and surgery has reduced death rates. Adults have a better chance of survival than children.

Possible Complications Complications may include:

• Blood clot
• Heart attack
• Heart failure
• Pericarditis
• Aortic valve insufficiency
• Pleuritis
• Stroke
• Gastrointestinal bleeding or pain from blockage of bowel blood vessels

NIH genetic and rare disease info

• NIH info on Takayasu arteritis

Takayasu arteritis is a rare disease.

Takayasu arteritis Resources
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