Definition of Takayasu arteritis:

Takayasu arteritis is an inflammation of the aorta -- the artery that carries blood from the heart to the rest of the body -- and its major branches.

See also: Aortic arch syndrome

Causes, incidence, and risk factors:

The cause of Takayasu arteritis is unknown. The disease occurs mainly in children and women younger than 30, especially those who are of Asian or African descent.

It appears to be an autoimmune condition, where immune cells are wrongly targeted against the body's own tissues, and it may involve other systems.

Symptoms:

• Arm weakness or pain with use
• Chest pain
• Dizziness
• Fatigue
• Fever
• Light-headedness
• Muscle or joint pain
• Skin rash
• Night sweats
• Vision changes
• Weight loss

Signs and tests:

• 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).

Tests:

• Arteriogram
• Angiogram
• Complete blood count (CBC)
• C-reactive protein (CRP)
• Electrocardiogram (ECG)
• Erythrocyte sedimentation rate (ESR)
• Magnetic resonance angiography (MRA)
• Magnetic resonance imaging (MRI)
• Ultrasound
• X-ray of the chest

Treatment:

Treatment of Takayasu arteritis is difficult, but patients who do have the right treatment can see positive results. Early detection is important.

Most patients are treated with steroids and immunosuppressive drugs. Surgery is reserved for complications caused by narrowed arteries.

Surgery to bypass narrowed arteries -- angioplasty or stent placement -- may be needed to supply blood or open up the constriction.

Expectations (prognosis):

This disease can be fatal. However, with a combination of aggressive medical and surgical treatment, death rates have dropped dramatically.

In well-managed cases of Takayasu arteritis, the long-term survival rate is greater than 90%. The survival rate is better for adults than for children.

Complications:

• Blood clot
• Heart attack
• Heart failure
• Pericarditis
• Pleuritis
• Stroke

Calling your health care provider:

Call your health care provider if you have symptoms of this condition. Weak pulse, chest pain, and breathing difficulty require immediate care.