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Takayasu arteritis

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Definition

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 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

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).

Exams and Tests

Possible tests include:

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 used to open up narrowed arteries 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 of survival than children.

Possible Complications

Complications may include:

  • 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.

Images

Heart, section through the middle

References

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. PMID: 24225487 www.ncbi.nlm.nih.gov/pubmed/24225487.

Glebova NO, Abularrage CJ. Takayasu's disease. In: Cronenwett JL, Johnston W, eds. Rutherford's Vascular Surgery. 8th ed. Philadelphia, PA: Elsevier Saunders; 2014:chap 80.

Renauer PA, Saruhan-Direskeneli G, Coit P, et al. Identification of susceptibility loci in IL6, RPS9/LILRB3, and an intergenic locus on chromosome 21q22 in Takayasu Arteritis in a genome-wide association study. Arthritis Rheumatol. 2015;67:1361-1368. PMID: 25604533 www.ncbi.nlm.nih.gov/pubmed/25604533.

Review Date: 
10/18/2015
Reviewed By: 
Gordon A. Starkebaum, MD, Professor of Medicine, Division of Rheumatology, University of Washington School of Medicine, Seattle, WA. Also reviewed by David Zieve, MD, MHA, Isla Ogilvie, PhD, and the A.D.A.M. Editorial team.

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