Treatment outcome of late steroid-resistant nephrotic syndrome: A study by the Midwest Pediatric Nephrology Consortium

Caroline Straatmann, Rose Ayoob, Rasheed Gbadegesin, Keisha Gibson, Michelle N. Rheault, Tarak Srivastava, Cheryl L. Tran, Debbie S. Gipson, Larry A. Greenbaum, William E. Smoyer, V. Matti Vehaskari

Research output: Contribution to journalArticlepeer-review

16 Scopus citations

Abstract

Background: Idiopathic nephrotic syndrome (NS) in children is classified as steroid sensitive or steroid resistant. Steroid sensitivity typically portends a low risk of permanent renal failure. However, some initially steroid-sensitive patients later develop steroid resistance. These patients with late steroid resistance (LSR) are often treated with immunosuppressant medications, but the effect of these additional drugs on the long-term prognosis of LSR is still unknown. Methods: A retrospective chart review was performed on patients diagnosed with idiopathic NS and subsequent LSR during the 8-year study period from 2002 up to and including 2009, with a minimum of 2 years of follow-up. Primary outcome measures were proteinuria and renal function. Results: A total of 29 patients were classified as having LSRNS. The majority of patients received treatment with calcineurin inhibitors and/or mycophenolate mofetil. Seven patients received three or more non-steroid immunosuppressant medications. Sustained complete or partial remission was achieved in 69 % of patients. Three developed end-stage renal disease, and all others maintained normal renal function. There were 13 episodes of serious adverse events, none of which were fatal or irreversible. Conclusion: Most patients with LSRNS responded to immunosuppressive therapy by reduction or resolution of proteinuria and preservation of renal function. The results suggest that immunosuppressive treatment is a viable option in NS patients who develop LSR.

Original languageEnglish (US)
Pages (from-to)1235-1241
Number of pages7
JournalPediatric Nephrology
Volume28
Issue number8
DOIs
StatePublished - Aug 1 2013

Keywords

  • Adverse events of immunosuppression
  • Immunosuppressant therapy
  • Late steroid-resistant nephrotic syndrome
  • Outcome of nephrotic syndrome
  • Steroid-resistant nephrotic syndrome

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