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Takayasu arteritis

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

Alternative Names

Pulseless disease

Causes

The cause of Takayasu arteritis is unknown. The disease occurs mainly in children and women younger than 40. It is more common in people from of Asian or African descent.

Takayasu arteritis appears to be an autoimmune condition, which means the body's immune system mistakenly attacks healthy tissue. The condition may also involve other organ 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

Exams 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:

Treatment

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 reserved for complications caused by narrowed arteries.

Surgery to open up narrowed arteries may be needed to supply blood or open up the constriction

Outlook (Prognosis)

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 at survival than children.

Possible Complications

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

When to Contact a Medical Professional

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

References

Maksimowicz-McKinnon K, Hoffman GS. Takayasu arteritis: what is the long-term prognosis? Rheum Dis Clin North Am. 2007;33:777-786.

Clifford A, Hoffman GS. Recent advances in the medical management of Takayasu arteritis: an update on use of biologic therapies. Curr Opin Rheumatol. 2014;26(1):7-15.

Updated: 1/22/2014

Gordon A. Starkebaum, MD, Professor of Medicine, Division of Rheumatology, University of Washington School of Medicine. Also reviewed by David Zieve, MD, MHA, Bethanne Black, and the A.D.A.M. Editorial team.


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