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Wegener's Granulomatosis (cont.)

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What is the treatment for granulomatosis with polyangiitis (GPA)?

Granulomatosis with polyangiitis (GPA) is a serious disease and without treatment can be fatal within months. Treatment is directed toward stopping the inflammation process by suppressing the immune system.

Medications used to treat GPA include high-dose cortisone (prednisone) and the immunosuppressive drug cyclophosphamide (Cytoxan). Recent reports also suggest that trimethoprim/sulfamethoxazole (Bactrim) can also be helpful to prevent relapse of disease activity in patients with GPA.

Cytoxan that is taken by mouth with prednisone until the disease is in remission and then switched to methotrexate (Rheumatrex, Trexall) for two years and tapered off has been reported to be effective and less toxic than the traditional long-term Cytoxan treatment.

Methotrexate has recently been introduced as a drug for Cytoxan treatment failures. Moreover, it now appears that Cytoxan will not be necessary in order to maintain long-term remission and that doctors can convert to the less toxic methotrexate for maintenance. The reports also demonstrate that methotrexate can eventually be tapered off entirely after two years. Azathioprine (Imuran) has also been used as a maintenance medication after Cytoxan. Recently, intravenous immunoglobulin therapy (IVIG) has been shown to be helpful in treating relapses of granulomatosis with polyangiitis. Studies suggest that rituximab (Rituxan) may be helpful to maintain remission once the initial inflammatory disease has been controlled using medications mentioned above. These new regimens are welcome news for patients with GPA as medical researchers are searching for better treatments.

What is the prognosis for granulomatosis with polyangiitis (GPA)?

The prognosis for granulomatosis with polyangiitis (GPA) depends on which organs are involved, to what degree they are involved, the duration of the disease, and the response to treatment. Today, with an optimal response to treatment, persons with GPA can lead normal lives.

Medically reviewed by Robert J. Bryg, MD; Board Certified Internal Medicine with subspecialty in Cardiovascular Disease


Koopman, William, et al., eds. Clinical Primer of Rheumatology. Philadelphia: Lippincott Williams & Wilkins, 2003.
Kelley's Textbook of Rheumatology, W B Saunders Co, edited by Shaun Ruddy, et al., 2000.American College of Rheumatology National Meeting, November 2005, 2006, 2007.

UpToDate.Clinical manifestations and diagnosis of granulomatosis with polyangiitis and microscopic polyangiitis.

Medically Reviewed by a Doctor on 9/2/2016



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