Cyclophosphamide (Cytoxan) which is an anti-cancer drug, and corticosteroids, such as prednisone, are used to treat Wegeners disease. These are powerful drugs that suppress the immune system.
- Initial treatment is generally with corticosteroids and oral cyclophosphamide (CYC), 1 mg/kg/day and 2 mg/kg/day respectively. Occasionally CYC is given in monthly IV doses.
- Monitoring of the white blood count is essential during CYC therapy. Once remission is attained (normally 3 to 6 months), treatment is frequently changed to azathioprine or methotrexate, which are less toxic drugs.
- Total duration of therapy should be at least 1 year, or longer in high risk patients. Corticosteroids are tapered to a low maintenance dose, 5-10 mg/day. Plasmapheresis may be beneficial in severe disease or pulmonary hemorrhage.
- Alternative treatments include:
- Rituximab: a very promising drug currently in clinical trials. It has been successful when used several times as a compassionate treatment for those who cannot tolerate CYC.
- Chlorambucil: a powerful immunosuppressant that has been used instead of cyclophosphamide in some cases.
- Mycophenolate mofetil: a drug similar to azathioprine.