What is the role of medications in the treatment of granulomatosis with polyangiitis (GPA)?

Updated: Aug 31, 2021
  • Author: Christopher L Tracy, MD; Chief Editor: Herbert S Diamond, MD  more...
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The combination of cyclophosphamide (intravenous or oral) and corticosteroids remained the recommended therapy for induction of remission in generalized granulomatosis with polyangiitis (GPA) for years. In 2011, however, the FDA approved the use of rituximab, a monoclonal antibody that targets B cells, as an alternative to cyclophosphamide for the treatment of AAV (GPA and microscopic polyangiitis).

Prophylaxis against Pneumocystisjiroveci pneumonia should be instituted while patients are taking cyclophosphamide or rituximab and corticosteroids (particularly high-dose corticosteroids) and for at least 6 months following these medications. Typically, trimethoprim-sulfamethoxazole (TMP-SMZ) at 160/800 mg 3 times weekly is used. If the patient has a sulfa allergy, dapsone 100 mg daily can be substituted.

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