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

Updated: Oct 09, 2019
  • Author: Christopher L Tracy, MD; Chief Editor: Herbert S Diamond, MD  more...
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Answer

Historically, glucocorticoid monotherapy prolonged median survival in GPA by only 7.5 months. There have been no clinical trials evaluating the role or dosing of glucocorticoids in AAV, but every clinical trial has used glucocorticoids in combination with other immunosuppressants. Thus, corticosteroids remain the cornerstone of treatment for AAV, especially for the induction of remission. [4]

One meta-analysis sought to answer the question of comparing glucocorticoid regimens in AAV. The authors found that studies with a longer course of glucocorticoids (ie, nonzero target dose) were associated with fewer relapses. Another retrospective study by McGregor et al found that once remission is reached for at least 1 month, glucocorticoid therapy beyond 6 months was associated with a greater risk of infection without a significantly reduced risk of relapse. [69] Further studies are still needed to define the best regimen with respect to glucocorticoid dose and duration of therapy. [38]

Glucocorticoids are usually given orally. If a rapid response is needed, however, such as in the case of rapidly progressive glomerulonephritis and/or alveolar hemorrhage, intravenous pulse methylprednisolone (0.5-1 g/day for 3 consecutive days) can be used and then followed by oral prednisone.

Initial high-dose glucocorticoids (1 mg/kg/day) should be continued for at least 1 month. Doses should not be reduced to less than 15 mg/day within the first 3 months. The dose should then be slowly tapered to a maintenance dose of 10 mg/day or less during remission. [4] Methods to prevent glucocorticoid-induced osteoporosis should be followed.


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