Takayasus Arteritis

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Takayasus Arteritis is an uncommon form of vasculitis, a spectrum of conditions characterized by inflammation of your blood vessels. Takayasu's arteritis damages the large arteries in your body, particularly the aorta — your body's principal blood vessel, which carries blood from your heart to the rest of your body — and its major branches.

Inflammation of a blood vessel, as occurs in Takayasus arteritis, can cause layers of the blood vessel to thicken. This narrows the blood vessel, reducing the amount of blood — and thus, the oxygen and nutrients — that reaches your body's tissues. Reduced blood flow can lead to conditions such as high blood pressure, heart failure and other circulatory problems. In some cases, a blood clot may form in an affected artery, obstructing blood flow and causing a stroke or heart attack. Sometimes instead of becoming narrower, a blood vessel may weaken and form a bulge. If the aneurysm bursts, it can be life-threatening.

Treatment usually consists of relieving the inflammation in the arteries and preventing potential complications. With early detection and appropriate treatment, the signs and symptoms of Takayasus arteritis can be managed successfully for many years.

General symptoms associated with Takayasu's arteritis include:

  • Fever
  • Fatigue
  • Night sweats
  • Weight loss
  • Muscle or joint pain, or both

  • Narrowing of your arteries due to inflammation may lead to reduced blood flow throughout your body, resulting in signs and symptoms such as:
  • Arm weakness or pain with use
  • Lightheadedness, headaches
  • Visual disturbances
  • High blood pressure
  • Difference in blood pressure between two arms
  • Diminished or absent pulse at the wrist
  • Mild anemia, which may make you feel tired or a little weak
  • Chest pain

  • In the beginning, you may experience only the more general signs and symptoms, such as fever, fatigue, weight loss and pain. But as your arteries become narrowed and blood flow to other organs and tissues is reduced, other more specific signs and symptoms, such as arm weakness and visual disturbances, may become apparent.

    What causes Takayasu's arteritis remains unknown. Some experts believe infection plays a role in the disease, particularly because people who have Takayasus arteritis often have or have had tuberculosis, but the association between these two illnesses isn't clear. In the United States, for example, most people with Takayasus arteritis have no history of tuberculosis.

    Another theory is that Takayasu's arteritis is an autoimmune disease, in which your immune system malfunctions and attacks your own arteries as if they were foreign substances.

    The goal of treatment is to control inflammation and prevent further damage to your blood vessels, with minimal long-term side effects. Takayasus arteritis can sometimes be difficult to treat because even if you appear to be in remission, disease activity may still continue, albeit "silently." In addition, by the time some people are diagnosed, it's possible that irreversible damage may already have been done.

    Still, with early detection and appropriate treatment, the signs and symptoms of Takayasu's arteritis can be managed successfully for many years. If your condition is relatively stable and uncomplicated, you may not need treatment.

    Treatment usually consists of medications and, in some cases, surgery.

    . . . Medications
    Many of these medications have long-term side effects, so your doctor will try to balance their benefits against their potential risks by controlling dosing of medications and the length of time you take them.

      Corticosteroids The first line of treatment is usually with a corticosteroid drug such as prednisone or methylprednisolone (Medrol). About half the people treated with corticosteroids respond well. You often start feeling better in just a few days, but you may need to continue taking medication for an extended period of time. After the first month, your doctor may gradually begin to lower the dose until you reach the lowest dose of corticosteroids you need to control inflammation. Some of your signs and symptoms may return during this tapering period. Long-term side effects of corticosteroids include cataracts, high blood sugar, increased risk of infections, loss of calcium from bones, menstrual irregularities, suppressed adrenal gland hormone production, thin skin, obesity, weakness in shoulders and thighs, easy bruising, and slower wound healing.
      Cytotoxic drugs If your condition doesn't respond well to corticosteroids or you have trouble tapering off the medication, you may need treatment with cytotoxic drugs, such as methotrexate (Rheumatrex), azathioprine (Imuran) or cyclophosphamide (Cytoxan). These drugs suppress the inflammation in your blood vessels. Mycophenolate mofetil (CellCept), another immunosuppressant used to prevent transplant rejection, also may be helpful for Takayasus arteritis, though the Food and Drug Administration hasn't approved it for this purpose. Immunosuppressants have risks. They can increase your susceptibility to infection, as well as your risk of developing lymph node tumors and skin cancer.
      TNF-alpha inhibitors In a small study, a group of medications called tumor necrosis factor-alpha inhibitors were able to help some people who had difficult-to-treat Takayasu's arteritis. This class of medications helps to reduce inflammation by inhibiting the action of TNF-alpha protein, which plays an active role in initiating your body's inflammatory response. Given further confirmation of the drug's effectiveness, doctors hope it will be a useful addition to the treatment options for Takayasu's.
    . . . Surgery
    If your arteries become severely narrowed or blocked, surgery may be necessary to open or bypass these arteries to allow an uninterrupted flow of blood. Often, this helps to improve signs and symptoms, such as high blood pressure and chest pain. In some cases, though, narrowing or blockage may recur, requiring a second procedure. Procedures used include:

      Bypass surgery In this procedure, an artery or a vein is removed from a different part of your body and attached to the blocked artery, providing a bypass for blood to flow through.
      Percutaneous angioplasty During this procedure, a tiny balloon is threaded through a blood vessel and into the affected artery. Once in place the balloon is expanded to widen the blocked area.
      Stenting Tiny wire mesh coils called stents may be inserted into the area widened by angioplasty. The stents help to prop open the artery to prevent the blood vessel from narrowing again.


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