Types of Treatments
- The main treatment for patients with EGPA is the use of glucocorticoids, which is sometimes combined with a type of immunosuppressant in more serious cases. The treatment of EGPA is very similar to the treatment of another ANCA-associated vasculitis called Granulomatosis with polyangiitis (GPA). Other features of EGPA like asthma and hypertension (high blood pressure) are treated normally as if they were seen separate from EGPA.
- Glucocorticoids (ex. prednisone) are usually used in the treatment of EGPA in order to suppress the systemic symptoms and arrest the progression of EGPA. More specifically, these medications suppress the immune system and help reduce the amount of inflammation of the blood vessels. Also, if there is involvement of the heart, kidneys, digestive tract or the central nervous system (brain or spinal cord), then a higher dose of corticosteroids are usually given in combination with immunosuppressants.
- Cyclophosphamide (CYC)
- This drug is usually used in combination with steroids (corticosteroids) in mostly severe cases of EPGA involving many organs.
- Azathioprine (AZA) and Methotrexate (MTX)
- In milder cases, corticosteroids can be used in combination with either MTX or AZA. However, AZA is often preferred because it is easier to monitor for drug side effects.
- Mycophenolate mofetil (MMF)
- This drug has been used in the past for the treatment of patients with GPA. Though less commonly, there have also been reports of MMF being used for patients with EGPA as well.
It is important to note that different combinations of medications are used at different phases in the treatment of EGPA. For example, initially, corticosteroids and cyclophosphamide are used together to begin treatment (induction therapy). Later on, maintenance therapy (see below) consists of drugs such as azathioprine (AZA). Also note that AZA and MTX are often used in milder cases of EGPA, in conjunction with corticosteroids instead of cyclophosphamide.
Refractory diseases, which do not respond to medications such as steroids and immunosupressants, can be treated using biological therapies. This drug has been used in the treatment of the other ANCA-associated vasculitides such as GPA and MPA. However, there is little research on the use of this drug in children with EGPA.
- A technique that removes the liquid portion of the patient’s blood (called plasma) which may contain disease-related substances. After this, the body is given an infusion of fresh protein or plasma which allows the body to produce new plasma. This has been done in patients with EGPA and kidney involvement, however the results are not very reliable and there may be no real benefit of this treatment.
- Refractory disease describes cases in which patients do not respond optimally to the initial treatment. In these cases, there have been some studies which have used intravenous immune globulin (IVIG) in combination with glucocorticoids.