What is the prognosis of pediatric nephrotic syndrome resistant to steroids?

Updated: Mar 04, 2020
  • Author: Jerome C Lane, MD; Chief Editor: Craig B Langman, MD  more...
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Answer

Approximately 10% of patients overall with INS do not respond to an initial trial of steroids (2% of patients with MCNS do not respond to steroids). Additionally, about 1-3% of patients who initially do respond to steroids later become resistant to treatment ("late non-responders"). [1]

Most patients who do not achieve remission of proteinuria with steroids have kidney biopsy findings other than MCNS. The most common diagnosis in these patients is FSGS.

More than 60% of patients with nephrotic syndrome and FSGS who fail to achieve remission with any treatment progress to end-stage kidney disease (ESKD). In contrast, only 15% of patients with FSGS who achieve remission by any treatment progress to ESKD. [53] Gipson et al reported a 90% lower risk of progression to ESKD in patients with INS who achieved remission. [54]

Thus, patients with steroid-resistant INS have a good prognosis if remission of proteinuria can be achieved by medications other than corticosteroids. Failure to respond to treatment (ie, failure to achieve remission) and kidney insufficiency at presentation are predictors of poor outcome and progression to ESKD. [55]


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