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.

  1. 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.
  2. 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.
  3. 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.
  4. Alternative treatments include:
  5. 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.
  6. Chlorambucil: a powerful immunosuppressant that has been used instead of cyclophosphamide in some cases.
  7. Mycophenolate mofetil: a drug similar to azathioprine.