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Clinical Services at Rush Types of Vasculitis

Takayasu’s disease (TKD)

What is the cause of Takayasu’s disease?
What are the symptoms of Takayasu’s disease?
How is the diagnosis of Takayasu’s disease made?
What is the treatment for Takayasu’s disease?

What is the prognosis for Takayasu’s disease?

Takayasu’s disease (Takayasu’s 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, including the pulmonary artery that serves the lungs. This large-vessel vasculitis was first described in 1908 by Japanese ophthalmologist Mikito Takayasu, who had observed disturbed blood vessels in the retina of the eye.

Takayasu’s disease is occasionally called “pulseless disease” because the narrowing of blood vessels to the arms can make it difficult to detect peripheral pulses.

Women, generally under 40 years in age, are eight to nine times more likely to be diagnosed with Takayasu’s disease than men are. Women of Asian descent seem to be most susceptible.

What is the cause of Takayasu’s disease?

The cause of Takayasu’s disease is unknown. It appears to be an autoimmune condition, where immune cells wrongly target the body's own tissues, and it may involve other systems.

What are the symptoms of Takayasu’s disease?

In some patients, there may be an initial phase with symptoms of an inflammatory illness, such as malaise, fever, night sweats, weight loss, joint pain and fatigue. Often there is a secondary phase characterized by pain in the arms and legs during repetitive activities. The affected blood vessels may be so constricted that a normal pulse cannot be felt in the neck, elbow, wrist or lower extremities. Also during this phase, there may be narrowing of the artery to the kidneys, which can lead to water and salt retention, resulting in high blood pressure.

Neurological problems caused by restricted blood flow to the brain can range from lightheadedness to seizures in severe cases. In rare instances, there may be serious complications involving the eyes. Due to the decrease in blood circulation to the arms, Raynaud's phenomenon (change in color of the extremities) frequently accompanies Takayasu’s disease.

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

How is the diagnosis of Takayasu’s disease made?

Diagnosing Takayasu’s disease can be extremely difficult, partly because there may be no symptoms or only nonspecific symptoms for years, until complications such as dilation of the aorta, critically reduced blood flow to an arm or leg, or a stroke brought on by high blood pressure in the brain result.

If Takayasu’s disease is suspected, an angiogram or a magnetic resonance imaging (MRI) study can be performed to see if large artery disease is present in a manner consistent with the initial diagnosis.

Prominent narrowing due to inflammation, granuloma and fibrosis is often seen in arterial studies such as magnetic resonance angiography(MRA), computed tomography angiography (CTA) or arterial angiography (DSA)

Tests:

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

What is the treatment for Takayasu’s disease?

Most patients with Takayasu’s disease respond to steroids such as prednisone. High doses of such medications over the long term can have significant side effects, so monitoring of the dose level is critical. If a patient does not respond to steroids, surgery may be required to open up the affected blood vessels.

For long-term treatment, immunosuppressives such as methotrexate, azathioprine (Imuran), cyclophosphamide (Cytoxan) or infliximab (Remicade) may be used with the prednisone, but there is no consensus as to their efficacy in treating this disease.

What is the prognosis for Takayasu’s disease?

This disease can have severe complications, and early intervention is critical. In well-managed cases of Takayasu’s disease that combine aggressive medical and surgical treatment as needed, the long-term survival rate is greater than 90 percent.

Medication toxicity remains a major concern in Takayasu’s disease, so it is very important that patients get regular check-ups, be vaccinated and be constantly monitored for any infection or side effects of therapy. All patients affected by Takayasu’s disease should be under the supervision of a skilled health team that is familiar with these conditions, their treatment and the side effects of immunosuppressive medications.
 





Contact Name
Rush Vasculitis Clinic
Contact Phone
(312) 563-2800
Contact E-mail
contact_rush@rush.edu



Location
Orthopedic Building
1611 W. Harrison St., Suite 510
Chicago, IL 60612



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