How is granulomatosis with polyangiitis (GPA) treated?

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

The mainstay of treatment for granulomatosis with polyangiitis (GPA) is a combination of corticosteroids and cytotoxic agents. Treatment should be tailored to appropriately treat GPA manifestations while minimizing long-term toxicities to the patient. Untreated generalized or severe GPA typically carries a dismal prognosis, with up to 90% of patients dying within 2 years, usually of respiratory or renal failure. Even non-renal GPA carries a mortality rate of up to 40%. [42]

Outcomes for this previously fatal disease improved dramatically with the introduction in the 1970s of cyclophosphamide, which is administered in combination with corticosteroids. [4] Approximately 90% of patients with GPA respond to cyclophosphamide, with approximately 75% experiencing complete remission. However, 30%-50% of those who initially respond experience at least one relapse, requiring another course of therapy.

In general, limited disease does not require as aggressive therapy as does severe disease. Additionally, treatment recommendations are different for induction of remission and maintenance of remission.


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