Takayasu’s Arteritis

Takayasu’s arteritis (TAK) is a rare disease in which the walls of large arteries (the aorta and its major branches) become inflamed. This often results in narrowing of the arteries, with decrease in blood flow to the places they are going. Less commonly, the artery enlarges to form an aneurysm, which does not cause symptoms directly but has a risk of rupturing if it gets too large. About 80% of patients with TAK are female, and the disease is usually diagnosed between the ages of 20 and 40. It is more common in patients of East Asian and South Asian ancestry, but it is a rare disease throughout the world. 

Causes and Risk Factors

Although the cause of TAK is unknown, it is thought to be an autoimmune disease. Genetics plays a small role. There is no known association with infection or another environmental exposure.

Symptoms

Symptoms vary depending on which arteries have become narrow. As with many other inflammatory diseases, patients with TAK often feel ill with “flu-like” symptoms before the disease is treated.  Symptoms include: 

  • Pain in the hand or lower leg with use (claudication), often with absent pulse or abnormally reduced blood pressure
  • Abdominal pain especially after eating
  • Chest pain with activity
  • Dizziness
  • Headache due to very high blood pressure (caused by narrowing of an artery supplying a kidney)
  • Muscle and joint pain, fatigue, fever 

Diagnosis

Because biopsy of large arteries is dangerous and most patients with TAK do not require surgical bypass, TAK is rarely diagnosed by examining tissue. Rather, imaging techniques such as MR angiography (MRA), CT angiography (CTA), PET scanning, and occasionally angiography are used to look for typical patterns of vessel involvement in patients with symptoms suspicious for TAK.

Treatment

TAK is highly treatable but requires strong immune-suppressive medications. The disease is chronic and therefore requires long-term treatment in about 80% of patients. Treatment prevents narrowing from worsening but does not usually improve symptoms that are due to permanent damage that has already occurred.

  • Corticosteroids ae usually used at high doses at the beginning of treatment and when the disease relapses. It is often continued at very low doses to maintain remission.
  • Immunosuppressive drugs such as methotrexate and azathioprine are may be used to reduce the need for corticosteroids long-term.
  • Biologic immunosuppressive drugs such as infliximab, adalimumab, and tocilizumab are often used if methotrexate and azathioprine are ineffective
  • Some patients require procedures such as surgery or angioplasty and stent placement in order to reduce symptoms that are due to permanent damage.

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