Steroid-resistant idiopathic nephrotic syndrome in children: long-term follow-up and risk factors for end-stage renal disease

J Bras Nefrol. Jul-Sep 2013;35(3):191-9. doi: 10.5935/0101-2800.20130031.
[Article in En, Portuguese]

Abstract

Introdution: Steroid resistant idiopathic nephrotic syndrome (SRINS) in children is one of the leading causes of progression to chronic kidney disease stage V (CKD V)/end stage renal disease (ESRD).

Objective: The aim of this retrospective study is to evaluate the efficacy of immunosuppressive drugs (IS) and to identify risk factors for progression to ESRD in this population.

Methods: Clinical and biochemical variables at presentation, early or late steroid resistance, histological pattern and response to cyclosporine A (CsA) and cyclophosfamide (CP) were reviewed in 136 children with SRINS. The analyzed outcome was the progression to ESRD. Univariate as well as multivariate Cox-regression analysis were performed.

Results: Median age at onset was 5.54 years (0.67-17.22) and median follow up time was 6.1 years (0.25-30.83). Early steroid-resistance was observed in 114 patients and late resistance in 22. Resistance to CP and CsA was 62.9% and 35% respectively. At last follow-up 57 patients reached ESRD. The renal survival rate was 71.5%, 58.4%, 55.3%, 35.6% and 28.5% at 5, 10, 15, 20 and 25 years respectively. Univariate analysis demonstrated that older age at onset, early steroid-resistance, hematuria, hypertension, focal segmental glomerulosclerosis (FSGS), and resistance to IS were risk factors for ESRD. The Cox proportional-hazards regression identified CsAresistance and FSGS as the only predictors for ESRD.

Conclusion: Our findings showed that CsA-resistance and FSGS were risk factors for ESRD.

MeSH terms

  • Adolescent
  • Child
  • Child, Preschool
  • Cohort Studies
  • Cyclophosphamide / therapeutic use*
  • Cyclosporine / therapeutic use*
  • Disease Progression
  • Female
  • Follow-Up Studies
  • Humans
  • Immunosuppressive Agents / therapeutic use*
  • Infant
  • Kidney Failure, Chronic / etiology*
  • Male
  • Nephrotic Syndrome / complications
  • Nephrotic Syndrome / congenital*
  • Nephrotic Syndrome / drug therapy
  • Retrospective Studies
  • Risk Factors
  • Time Factors

Substances

  • Immunosuppressive Agents
  • Cyclosporine
  • Cyclophosphamide

Supplementary concepts

  • Nephrotic syndrome, idiopathic, steroid-resistant