Treatment of Frequently Relapsing or Steroid-Dependent Nephrotic Syndrome in Children

Children with nephrotic syndrome who relapse frequently, or who cannot be weaned from glucocorticoids without relapsing, present a distinct clinical challenge — particularly when ongoing steroid exposure leads to significant adverse effects. Continuing glucocorticoid monotherapy is not appropriate for this group.


This protocol is indicated for children with frequently relapsing nephrotic syndrome or steroid-dependent nephrotic syndrome — especially those who have developed serious glucocorticoid-related adverse effects. A strategy that spares ongoing high-dose glucocorticoid exposure is required.

A glucocorticoid-sparing agent is prescribed to prevent relapses — with the patient ideally in remission on glucocorticoids before initiation — and the full protocol details the available agent choices, sequencing, and co-administration requirements.

References

For children with frequently relapsing nephrotic syndrome who develop serious glucocorticoid-related adverse effects and for all children with steroid-dependent nephrotic syndrome, we recommend that glucocorticoid-sparing agents be prescribed to prevent relapses, rather than no treatment or continuation with glucocorticoid treatment alone.

Patients should ideally be in remission with glucocorticoids prior to the initiation of glucocorticoid-sparing agents such as oral calcineurin inhibitors (CNIs), cyclophosphamide, levamisole, mycophenolate mofetil (MMF), and rituximab. Coadministration of glucocorticoids is recommended for ≥2 weeks following the initiation of glucocorticoid-sparing treatment.

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