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

Djalila Mekahli1, Aurelia Liutkus, Bruno Ranchin

  • 1Service de Pédiatrie, Centre de référence des maladies rénales rares, Hôpital Femme Mère Enfant and Université de Lyon, Lyon, France.

Pediatric Nephrology (Berlin, Germany)
|March 13, 2009
PubMed

Insights

Long-term outcomes for pediatric idiopathic steroid-resistant nephrotic syndrome (SRNS) show improved kidney survival due to better management. Age at onset over 10 years predicts end-stage renal disease, highlighting the need for further research.

Area of Science:

  • Pediatric Nephrology
  • Immunology
  • Renal Medicine

Background:

  • Idiopathic steroid-resistant nephrotic syndrome (SRNS) presents a significant challenge in pediatric nephrology.
  • Long-term outcomes and prognostic factors for SRNS remain critical areas for investigation.

Purpose of the Study:

  • To retrospectively analyze the long-term outcomes of pediatric idiopathic SRNS.
  • To identify predictors of end-stage renal disease (ESRD) and evaluate treatment strategies.

Main Methods:

  • Retrospective study of 78 children with idiopathic SRNS across eight centers over 20 years.
  • Analysis of patient demographics, treatment strategies (e.g., ciclosporine, mycophenolate mofetil), and long-term outcomes including renal survival and patient survival.
  • Cox regression analysis was used to identify independent predictors of ESRD.

Main Results:

  • Actuarial patient survival after 15 years was 97%, with renal survival rates of 75% (5 years), 58% (10 years), and 53% (15 years).
  • Age at onset > 10 years was the sole independent predictor of ESRD (P < 0.001).
  • Twenty-six percent of patients received transplants, with a 40% recurrence rate among them.

Conclusions:

  • Kidney survival in pediatric idiopathic SRNS has improved over the past two decades due to enhanced management strategies.
  • Early identification of risk factors, such as age at onset, is crucial for predicting disease progression.
  • Further prospective trials are needed to evaluate novel immunosuppressive treatments for SRNS.

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