Long-term outcome of idiopathic steroid-resistant nephrotic syndrome: a multicenter study

Pediatr Nephrol. 2009 Aug;24(8):1525-32. doi: 10.1007/s00467-009-1138-5. Epub 2009 Mar 12.

Abstract

Long-term outcome of idiopathic steroid-resistant nephrotic syndrome was retrospectively studied in 78 children in eight centers for the past 20 years. Median age at onset was 4.4 years (1.1-15.0 years) and the gender ratio was 1.4. Median follow-up period was 7.7 years (1.0-19.7 years). The disease in 45 patients (58%) was initially not steroid-responsive and in 33 (42%) it was later non-responsive. The main therapeutic strategies included administration of ciclosporine (CsA) alone (n = 29; 37%) and CsA + mycophenolate mofetil (n = 18; 23%). Actuarial patient survival rate after 15 years was 97%. Renal survival rate after 5 years, 10 years and 15 years was 75%, 58% and 53%, respectively. An age at onset of nephrotic syndrome (NS) > 10 years was the only independent predictor of end-stage renal disease (ESRD) in a multivariate analysis using a Cox regression model (P < 0.001). Twenty patients (26%) received transplants; ten showed recurrence of the NS: seven within 2 days, one within 2 weeks, and two within 3-5 months. Seven patients lost their grafts, four from recurrence. Owing to better management, kidney survival in idiopathic steroid-resistant nephrotic syndrome (SRNS) has improved during the past 20 years. Further prospective controlled trials will delineate the potential benefit of new immunosuppressive treatment.

Publication types

  • Multicenter Study

MeSH terms

  • Adolescent
  • Child
  • Child, Preschool
  • Cyclophosphamide / therapeutic use*
  • Cyclosporine / therapeutic use*
  • Drug Resistance
  • Female
  • Humans
  • Immunosuppressive Agents / therapeutic use*
  • Infant
  • Male
  • Mycophenolic Acid / analogs & derivatives*
  • Mycophenolic Acid / therapeutic use
  • Nephrotic Syndrome / drug therapy*
  • Retrospective Studies
  • Steroids / therapeutic use
  • Time Factors
  • Treatment Outcome

Substances

  • Immunosuppressive Agents
  • Steroids
  • Cyclosporine
  • Cyclophosphamide
  • Mycophenolic Acid