The European Rare Kidney Disease Reference Network


Thematic Area:  Steroid Resistant Nephrotic Syndrome

No guidelines specifically applying to hereditary forms of SRNS are currently available. For the time being, the Workgroup therefore endorses the sections of the KFIGO Glomerulonephritis guideline that refer to pediatric SRSNS, and the qualifying commentary of the Canadian Society of Nephrology. 

1) KDIGO Guideline - Treatment of Steroid-Resistant Nephrotic Syndrome in Children:
     New Guidelines from KDIGO. 

    Pediatr Nephrol 2013; 28: 409-14

2) Canadian Society of Nephrology Commentary on the 2012 KDIGO Clinical Practice Guideline
     for Glomerulonephritis.   
      Am J Kidney Dis 2014; 63:354-62

Core Recommendations:
  1. Use of a calcineurin inhibitor as initial therapy for children with SRNS is recommended.

  2. Mycophenolate mofetil, high-dose corticosteroids, or a combination of these agents to be considered in children who fail to achieve complete or partial remission on CNI.

  3. Cyclophosphamide not to be given to children with SRNS. 

  4. Treatment with ACE-I or ARBs recommended for children with SRNS.

Comments by evaluators:
  • Guideline is largely outdated since role of genetic diagnostics in SRNS is not covered.