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Treatments and drugs
By Mayo Clinic staffWith early diagnosis and appropriate treatment, you may recover from Wegener's granulomatosis within a few months. Maintenance therapy often needs to be continued for 18 to 24 months. In some cases, longer treatment may be necessary. Because the disease can recur, your doctor will continue monitoring your condition closely after treatment.
Medications
Your doctor may prescribe:
- Corticosteroids. Medications such as prednisone help suppress the immune system and treat the early signs and symptoms of Wegener's granulomatosis.
- Immune suppressant medications. Most people require another immunosuppressive drug, such as cyclophosphamide (Cytoxan), azathioprine (Azasan, Imuran) or methotrexate (Rheumatrex, Trexall), to counteract the body's immune reaction.
- Biologic therapy. Rituximab (Rituxan) is approved by the Food and Drug Administration for Wegener's. It reduces the number of a type of cell in your body (B cell) involved with inflammation. Rituximab may be as effective as cyclophosphamide in treating severe cases of Wegener's, and may be even better for treating Wegener's that recurs.
When standard treatments aren't effective, some doctors who are experienced in treating Wegener's use experimental drugs. Drugs currently under investigation include mycophenolate mofetil (CellCept), infliximab (Remicade), intravenous immunoglobulin, deoxyspergualin and antithymocyte globulin.
Side effect treatments
Because of potential side effects of the drugs used to treat Wegener's, such as lowering your body's ability to fight off infection, your doctor will monitor your condition while you're taking them. Drugs your doctor may prescribe to help prevent drug-related side effects include:
- Sulfamethoxazole-Trimethoprim (Bactrim, Septra) to prevent lung infection
- Medicine such as bisphosphonates (Fosamax) to prevent bone loss (osteoporosis) associated with prednisone use
- Folic acid, a synthetic form of the B vitamin folate, to prevent sores and other signs and symptoms associated with the depletion of folate in your body from methotrexate use
Other treatments
- Plasmapheresis. Also known as plasma exchange, this treatment removes the liquid portion of your blood (plasma) and separates it from the blood cells. The blood cells are then put back into your body, and your body produces new plasma to make up for what was removed. In people who have very serious Wegener's granulomatosis, plasmapheresis can help the kidneys recover.
- Surgery. If you've had kidney failure as a result of Wegener's granulomatosis, you may need a kidney transplant to restore normal kidney function. Talk to your doctor to determine whether a kidney transplant might be an option for you. Your eligibility to receive a donated kidney will depend, in part, on how your other organs have been affected by the disease.
- Longo DL, et al. Harrison's Online. 18th ed. New York, N.Y.: The McGraw-Hill Companies; 2012. http://www.accessmedicine.com/resourceTOC.aspx?resourceID=4. Accessed Oct. 12, 2012.
- Granulomatosis with polyangiitis (Wegener's). American College of Rheumatology. http://www.rheumatology.org/practice/clinical/patients/diseases_and_conditions/wegeners.asp. Accessed Oct. 12, 2012.
- Holle JU, et al. Clinical manifestations and treatment of Wegener's granulomatosis. Rheumatologic Disease Clinics of North America. 2010;36:507.
- Papadakis MA, et al. Current Medical Diagnosis & Treatment 2013. 52nd ed. New York, N.Y.: The McGraw-Hill Companies; 2013. http://www.accessmedicine.com/resourceTOC.aspx?resourceID=1. Accessed Oct. 12, 2012.
- Falk RJ, et al. Clinical manifestations and diagnosis of Wegener's granulomatosis and microscopic polyangiitis. http://www.uptodate.com/index. Accessed Sept. 21, 2012.
- FDA approves Rituxan to treat two rare disorders. U.S. Food and Drug Administration. http://www.fda.gov/NewsEvents/Newsroom/PressAnnouncements/ucm251946.htm. Accessed Aug. 22, 2012.
- Langford CA. Update on the treatment of granulomatosis with polyangiitis (Wegener's). Current Treatment Options in Cardiovascular Medicine. 2012;14:164.
- Turnball J, et al. Adverse effects of therapy for ANCA-associated vasculitis. Best Practice and Research Clinical Rheumatology. 2009;23:391.
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