<p>Frequently relapsing, steroid-dependent nephrotic syndrome (FRNS/SDNS) remains a therapeutic challenge. Rituximab, a B-cell-depleting agent, is effective and widely used for FRNS/SDNS, but its use can be limited by rare, serious adverse effects. Belimumab, a monoclonal antibody targeting B-cell activating factor (BAFF), is approved for treating adult systemic lupus erythematosus (SLE) and lupus nephritis (LN). We report a pediatric case of FRNS/SDNS refractory to conventional immunosuppressive regimens and intolerant of rituximab due to serious complications, who subsequently maintained complete remission for nearly one year after steroid withdrawal while receiving belimumab added to ongoing mycophenolate mofetil (MMF). This case suggests that belimumab combined with MMF is effective for refractory NS. However, as a single case report, its efficacy requires further strengthening through more cases.</p>

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Belimumab combined with mycophenolate mofetil in a child with frequently relapsing, steroid-dependent nephrotic syndrome

  • Yi Mao,
  • Jia He,
  • Weijie Kang,
  • Jialu Lu,
  • Lei Yin,
  • Youying Mao

摘要

Frequently relapsing, steroid-dependent nephrotic syndrome (FRNS/SDNS) remains a therapeutic challenge. Rituximab, a B-cell-depleting agent, is effective and widely used for FRNS/SDNS, but its use can be limited by rare, serious adverse effects. Belimumab, a monoclonal antibody targeting B-cell activating factor (BAFF), is approved for treating adult systemic lupus erythematosus (SLE) and lupus nephritis (LN). We report a pediatric case of FRNS/SDNS refractory to conventional immunosuppressive regimens and intolerant of rituximab due to serious complications, who subsequently maintained complete remission for nearly one year after steroid withdrawal while receiving belimumab added to ongoing mycophenolate mofetil (MMF). This case suggests that belimumab combined with MMF is effective for refractory NS. However, as a single case report, its efficacy requires further strengthening through more cases.