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Second-line options for refractory steroid-sensitive and -resistant nephrotic syndrome
1Pediatric Nephrology, University Medical Center Hamburg-Eppendorf, Martinistr. 52, D-20246 Hamburg, Germany.
Expert Review of Clinical Pharmacology
|November 25, 2011
Summary
Idiopathic nephrotic syndrome in children often requires alternative treatments beyond steroids due to toxicity and relapses. Emerging therapies like mycophenolic acid and rituximab show promise, but refractory cases still need further research.
Area of Science:
- Pediatric Nephrology
- Immunology
- Pharmacology
Background:
- Idiopathic nephrotic syndrome (INS) in children frequently relapses or becomes steroid-dependent, necessitating alternative therapies.
- Steroid toxicity and treatment dilemmas arise in frequent relapsers and steroid-dependent cases.
- Current treatment options have limitations, including side effects and lack of efficacy in refractory cases.
Purpose of the Study:
- To review current and emerging treatment options for pediatric idiopathic nephrotic syndrome.
- To highlight challenges in managing steroid-sensitive and steroid-resistant nephrotic syndrome.
- To identify unmet needs in the treatment of refractory cases.
Main Methods:
- Literature review of current therapeutic strategies for pediatric INS.
- Analysis of data on alternative immunosuppressants and biologics.
- Discussion of diagnostic approaches for steroid-resistant cases, including genetic testing and biopsy.
Main Results:
- Steroid-sparing agents like cyclophosphamide and levamisole have been used, but mycophenolic acid is emerging as an alternative.
- Calcineurin inhibitors (cyclosporine A, tacrolimus) are effective but require long-term use with potential side effects.
- Rituximab shows promise for refractory cases and may be useful in less complicated scenarios.
- Focal segmental glomerulosclerosis (FSGS) requires genetic testing and biopsy, with treatments like pulse methylprednisolone and calcineurin inhibitors.
Conclusions:
- Despite advances, some pediatric INS patients exhibit treatment-refractory courses, posing a medical dilemma.
- Mycophenolic acid and rituximab offer potential therapeutic avenues, though their efficacy in FSGS may be less than in steroid-sensitive INS.
- Standardization of treatment protocols and further prospective studies are crucial for managing complicated INS cases.
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