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Takayasu arteritis

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

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. However, it is now being seen more often in other parts of the world. 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. Takayasu arteritis has many features that are similar to giant cell arteritis or temporal arteritis in older people.

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 two arms
  • High blood pressure (hypertension)

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

Exams and Tests

There is no blood test available to make a definite diagnosis. The diagnosis is made when a person has symptoms and imaging showing blood vessel narrowing suggesting inflammation.

Possible tests include:

Treatment

Treatment of Takayasu arteritis is difficult. However, people who have the right treatment can see improvement. 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.

Other biologic agents that show promising benefits for treating Takayasu arteritis include tocilizumab, and rituximab.

SURGERY

Surgery or angioplasty may be used to open up narrowed arteries to supply blood or open up the constriction.

Aortic valve replacement may be needed in some cases.

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
  • Aortic valve insufficiency
  • Pleuritis
  • Stroke
  • Gastrointestinal bleeding or pain from blockage of bowel blood vessels

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.

Alternative Names

Pulseless disease

References

Carmona FD, Coit P, Saruhan-Direskeneli G, et al. Corrigendum: analysis of the common genetic component of large vessel vasculitides through a meta-immunochip strategy. Sci Rep. 2017;7:46012. PMID: 28378796 www.ncbi.nlm.nih.gov/pubmed/28378796.

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.

Keser G, Aksu K. What is new in management of Takayasu arteritis? Presse Med. 2017;46(7-8 Pt 2):e229-e235. PMID: 28774475 www.ncbi.nlm.nih.gov/pubmed/28774475.

Mekinian A, Comarmond C, Resche-Rigon M, et al. Efficacy of biological-targeted treatments in Takayasu arteritis: multicenter, retrospective study of 49 patients. Circulation. 2015;132(18):1693-1700. PMID: 26354797 www.ncbi.nlm.nih.gov/pubmed/26354797.

Stone JH, Klearman M, Collinson N.Trial of tocilizumab in giant-cell arteritis. N Engl J Med. 2017;377(15):1494-1495. PMID: 29020600 www.ncbi.nlm.nih.gov/pubmed/29020600.

Review Date 10/1/2017

Updated 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, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team.