Initial treatment for Granulomatosis with polyangiitis (Wegener's) usually includes the anti-inflammatory corticosteroid drug prednisone. For milder or more limited disease, your doctor may also recommend treatment with the drug methotrexate (Folex, Rheumatrex).
For active or severe disease, your doctor may recommend treatment with a combination of two drugs: prednisone along with cyclophosphamide (Cytoxan, Neosar) or rituximab (Rituxan). Cyclophosphamide and rituximab are powerful immune-suppressing medications.
As your symptoms subside, your doctor will try to reduce the dosage of each drug gradually.
Once your condition improves and you've taken cyclophosphamide or rituximab for three to six months, your doctor may suggest switching medications. He or she may recommend methotrexate or azathioprine (Imuran, Azasan) for a total of one year of treatment. However, longer courses of therapy may be necessary if disease control is interrupted by periods of active disease.
Cyclophosphamide is a very strong drug. It can cause serious side effects such as bladder damage (especially bleeding) and an increased risk of bladder cancer. Cyclophosphamide and rituximab increase a person's risk of serious infections.
If you develop serious side effects related to these medications, your doctor may prescribe an alternative medication.
A procedure called plasmapheresis may help some people. In this procedure, blood is filtered and returned to the body. This procedure may help because it removes harmful antibodies but details about why it may work are unknown.