What is the role of trimethoprim-sulfamethoxazole in the treatment of granulomatosis with polyangiitis (GPA)?

Updated: Aug 31, 2021
  • Author: Christopher L Tracy, MD; Chief Editor: Herbert S Diamond, MD  more...
  • Print

Pneumocystis pneumonia has an annual incidence of 1% but is a potentially deadly complication of immunosuppressive therapy in patients with GPA, especially with prolonged lymphocytopenia.

Prophylaxis against Pneumocystisjiroveci pneumonia should be instituted while patients are taking cyclophosphamide and corticosteroids (particularly high-dose corticosteroids). Typically, trimethopim-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. Pneumocystis prophylaxis has also been recommended during rituximab treatment and for at least 6 months following the last rituximab infusion.

Reports have examined TMP-SMZ use in isolation without other immunosuppressive medications in the induction phase of treatment in patients with very limited disease; however, prospective trials of TMP-SMZ as monotherapy have been disappointing.

In adults, TMP-SMZ has been shown to prevent relapses of GPA in remission. [18] This effect of TMP-SMZ may be due to anti-inflammatory action or decrease in infections, particularly respiratory tract infections.

Did this answer your question?
Additional feedback? (Optional)
Thank you for your feedback!