Takayasu arteritis

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Definition

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.

Alternative Names

Pulseless disease, Large-vessel vasculitis

Causes

The cause of Takayasu arteritis is not known. 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 in the blood vessel wall. The condition may also involve other organ systems.

This condition 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 tests show blood vessel abnormalities suggesting inflammation.

Possible tests include:

Treatment

Treatment of Takayasu arteritis is difficult. However, people who have the right treatment can improve. It is important to identify the condition early. The disease tends to be chronic, requiring long-term use of anti-inflammatory medicines.

MEDICINES

Most people are first treated with high doses of corticosteroids such as prednisone. As the disease is controlled the dose of prednisone is decreased.

In almost all cases, immunosuppressive drugs are added to reduce the need for long-term use of prednisone and yet maintain control of the disease.

Conventional immunosuppressive agents such as methotrexate, azathioprine, mycophenolate, cyclophosphamide, or leflunomide are often added.

Biologic agents may also be effective. These include TNF inhibitors such as infliximab, etanercept, and tocilizumab.

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 reduced 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. Immediate care is needed if you have:

  • Weak pulse
  • Chest pain
  • Breathing difficulty

Images

Heart - section through the middle
Heart valves - anterior view
Heart valves - superior view

References

Alomari I, Patel PM. Takayasu arteritis. In: Ferri FF, ed. Ferri's Clinical Advisor 2020. Philadelphia, PA: Elsevier; 2020:1342.e4-1342.e7.

Barra L, Yang G, Pagnoux C; Canadian Vasculitis Network (CanVasc). Non-glucocorticoid drugs for the treatment of Takayasu's arteritis: A systematic review and meta-analysis. Autoimmun Rev. 2018;17(7):683-693. PMID: 29729444 pubmed.ncbi.nlm.nih.gov/29729444/.

Dejaco C, Ramiro S, Duftner C, et al. EULAR recommendations for the use of imaging in large vessel vasculitis in clinical practice. Ann Rheum Dis. 2018;77(5):636-643. PMID: 29358285 pubmed.ncbi.nlm.nih.gov/29358285/.

Ehlert BA, Abularrage CJ. Takayasu disease. In: Sidawy AN, Perler BA, eds. Rutherford's Vascular Surgery and Endovascular Therapy. 9th ed. Philadelphia, PA: Elsevier; 2019:chap 139.

Serra R, Butrico L, Fugetto F, et al. Updates in pathophysiology, diagnosis and management of Takayasu arteritis. Ann Vasc Surg. 2016;35:210-225. PMID: 27238990 pubmed.ncbi.nlm.nih.gov/27238990/.

Review Date: 
10/18/2019
Reviewed By: 
Gordon A. Starkebaum, MD, MACR, ABIM Board Certified in Rheumatology, Seattle, WA. Also reviewed by David Zieve, MD, MHA, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team.

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