Takayasu arteritis

Pulseless disease

The cause of Takayasu arteritis is unknown. 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. 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. The condition may also involve other organ systems.

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 2 arms
• High blood pressure (hypertension)

There may also be signs of inflammation (pericarditis or pleuritis).

Possible tests include:

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

Treatment of Takayasu arteritis is difficult. However, people who have the right treatment can see positive results. It is important to identify the condition early.

MEDICINES

Most people are first treated with high doses of steroids. Immunosuppressive drugs, such as azathioprine, mycophenolate, methotrexate, or leflunomide are often added.

Biologic agents including TNF inhibitors such as infliximab are recommended for people who do not improve with the previous treatment.

In difficult cases, tocilizumab or rituximab may be helpful.

SURGERY

Surgery is used to open up narrowed arteries to supply blood or open up the constriction.

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

Complications may include:

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

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