TAKAYASU TREATMENT

In an autoimmune disease like Takayasu Arteritis, the immune system attacks and harms the bodies' own tissues. The exact cause of Takayasu's disease is unknown. Most symptoms of the disease are caused by vasculitis (an inflammation of the blood vessels). Inflammation is a characteristic reaction of the body to injury or disease and is marked by 2 phases: 1) a systemic phase (inflamed stage, fever, fatigue, weight loss, etc.); and 2) an occlusive phase (narrowing of the affected arteries).

Doctors think that an autoimmune reaction may cause the blood vessels to become inflamed, but they do not know what triggers this reaction. Under normal conditions, the immune system protects the body from diseases and infections by killing harmful "foreign" substances, such as germs, that enter the body. In an autoimmune reaction, the immune system mistakenly attacks and harms the body's own tissues. In this case, Takayasu arteritis is a chronic inflammatory condition that affects the largest blood vessel in the body (the aorta) and its branches.

Doctors usually begin treatment for Takayasu's arteritis with glucocorticoid medications, such as prednisone. If these medications alone do not work well, then stronger immunosuppressive drugs may be added, including methotrexate (Folex, Methotrexate, Rheumatrex), azathioprine (Imuran) or cyclophosphamide (Cytoxan, Neosar). Recent studies suggest that injectable etanercept (Enbrel) or infliximab (Remicade) may be helpful to control the vascular inflammation of Takayasu's arteritis when other medications fail. Blood pressure medications also may be necessary to control high blood pressure.

If Takayasu's arteritis has caused significant narrowing of an artery, the narrowed segment may need to be widened or repaired. Depending on the artery affected, this can be done in one of three ways:

- Traditional (open) surgery
- Percutaneous transluminal balloon angioplasty, in which a small balloon is inserted into the blood vessel, then inflated to dilate it
- Percutaneous placement of a stent, in which a small mesh device is placed in the artery to keep the blood vessel open

Inflamed segments of arteries may become weakened, leading to the formation of an aneurysm, or outpouching of the vessel. These may expand over time and rupture, a potentially life-threatening event; therefore, tests for aneurysm (including MRA) may be recommended for people with Takayasu's arteritis, and if an aneurysm is large or expanding, surgical repair may be recommended.

Prognosis

Symptoms usually go away in about 60% of people with Takayasu's arteritis when they are treated with glucocorticoids alone. However, symptoms return in about half of these patients. When people who have symptoms return are treated with a combination of glucocorticoids and other immunosuppressive drugs, 40% to 81% have symptoms go away again, but it's common for them to return later. Overall, about 77% of people with Takayasu's arteritis respond to some form of medical therapy.

In general, about 83% of people survive for at least 15 years after diagnosis. This figure drops to 66% for those with severe high blood pressure or significant damage to the aorta.

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