How is EGPA treated? What medicines help?

How is EGPA treated? What medicines help?

A variety of immunosuppressive drugs (drugs that suppress the immune system) are used to treat EGPA. Your healthcare provider will choose the drug based on how the EGPA is affecting you - whether it is mild (for instance, affecting the skin or joint), or potentially life-threatening.

Glucocorticoids such as prednisone or prednisolone are the most commonly used treatments. People who have a mild disease (those whose disease does not affect the nervous system, heart, kidneys, intestinal tract, or show other features of severe disease) might do extremely well on corticosteroid therapy alone. Once you have a dramatic improvement on this drug, the dosage is reduced to the lowest amount that keeps the disease under control.

People with EGPA that affects critical organs are usually treated with corticosteroids combined with another immunosuppressive drug, such as cyclophosphamide (Cytoxan®), methotrexate, Mycophenolate Mofetil or azathioprine (Imuran®).

These drugs are also used to treat other medical conditions. Azathioprine is used to prevent organ transplant rejection and is also a treatment for lupus and other autoimmune diseases. High doses of cyclophosphamide and methotrexate are used to treat certain types of cancer. In vasculitis, these drugs are given at doses that are 10 to 100 times lower than that used to treat cancer in order to suppress the immune system.

Biologic medications are also used in EGPA such as Rituximab (anti-CD20 monoclonal antibody directed against B cells) and Mepolizumab (anti-interleukin (IL)-5 monoclonal antibodies. Mepolizumab is approved by the FDA for the treatment of EGPA and is indicated in people whose disease is not controlled with oral glucocorticoids alone and who do not have severe disease. Rituximab can be used in people with kidney, skin and nerve involvements.

The goal of treatment is to stop all damage that comes from EGPA. This is known as "remission." Once the disease begins to improve, your healthcare provider may slowly reduce the corticosteroid dose, and eventually stop it completely. Stopping it completely may be difficult if a person has significant asthma, as the airways can be very sensitive to corticosteroids.

If cyclophosphamide is used for severe EGPA, it is often only given until you go into remission (usually around three to six months); you’ll then switch to another immunosuppressive agent, such as methotrexate, Mycophenolate Mofetil, azathioprine or Mepolizumab, to maintain remission.

The duration of maintenance immunosuppressive drugs depends on the person. In most instances, it is given for least one to two years before the healthcare provider considers slowly reducing the dosage and eventually stopping it.

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