Background <p>Rituximab (RTX) is widely used to prevent relapses in steroid-dependent or frequently-relapsing nephrotic syndrome (SD/FRNS). However, the impact of B-cell depletion duration on long-term outcomes remains uncertain. We compared short-term B-cell depletion (limited RTX course) with prolonged depletion maintained by repeated RTX infusions and assessed long-term disease activity.</p> Methods <p>In this retrospective multicenter study, we included children with SD/FRNS who received RTX before the age of 18&#xa0;years between 2007 and 2017. Short-depletion corresponded to one or two RTX infusions, whereas prolonged-depletion reflected repeated infusions over time to maintain B-cell depletion. Prolonged remission was defined as the absence of relapse and immunosuppressive therapy for at least 2&#xa0;years after the last anti-CD20 infusion.</p> Results <p>A total of 117 patients were included (median age at first RTX infusion: 11.6&#xa0;years; 65% were boys), of whom 48 (41%) had short-depletion and 69 (59%) had prolonged-depletion. Baseline characteristics were comparable between groups. At 2&#xa0;years after the last RTX infusion, 69% of patients in the short-depletion group and 65% in the prolonged-depletion group were in prolonged remission. Overall, 89 patients (75%) relapsed during follow-up. Median time to first relapse was significantly longer in the long-depletion group (36 vs. 19&#xa0;months, <i>p</i> = 0.04). Prolonged hypogammaglobulinemia was more frequent with prolonged-depletion, whereas infection rates were similar between groups.</p> Conclusion <p>RTX exerts a suspensive rather than curative effect in SD/FRNS. Prolonged B-cell depletion extends relapse-free survival but is associated with more frequent hypogammaglobulinemia, without an increase in severe infections.</p> Graphical abstract <p></p>

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B cell depletion with rituximab in children with steroid-sensitive nephrotic syndrome: does depletion duration change outcome?

  • Gael Cals,
  • Claire Dossier,
  • Olivia Boyer,
  • Jean Daniel Delbet,
  • Julien Hogan,
  • Antoine Mouche,
  • Tim Ulinski,
  • Cyrielle Parmentier

摘要

Background

Rituximab (RTX) is widely used to prevent relapses in steroid-dependent or frequently-relapsing nephrotic syndrome (SD/FRNS). However, the impact of B-cell depletion duration on long-term outcomes remains uncertain. We compared short-term B-cell depletion (limited RTX course) with prolonged depletion maintained by repeated RTX infusions and assessed long-term disease activity.

Methods

In this retrospective multicenter study, we included children with SD/FRNS who received RTX before the age of 18 years between 2007 and 2017. Short-depletion corresponded to one or two RTX infusions, whereas prolonged-depletion reflected repeated infusions over time to maintain B-cell depletion. Prolonged remission was defined as the absence of relapse and immunosuppressive therapy for at least 2 years after the last anti-CD20 infusion.

Results

A total of 117 patients were included (median age at first RTX infusion: 11.6 years; 65% were boys), of whom 48 (41%) had short-depletion and 69 (59%) had prolonged-depletion. Baseline characteristics were comparable between groups. At 2 years after the last RTX infusion, 69% of patients in the short-depletion group and 65% in the prolonged-depletion group were in prolonged remission. Overall, 89 patients (75%) relapsed during follow-up. Median time to first relapse was significantly longer in the long-depletion group (36 vs. 19 months, p = 0.04). Prolonged hypogammaglobulinemia was more frequent with prolonged-depletion, whereas infection rates were similar between groups.

Conclusion

RTX exerts a suspensive rather than curative effect in SD/FRNS. Prolonged B-cell depletion extends relapse-free survival but is associated with more frequent hypogammaglobulinemia, without an increase in severe infections.

Graphical abstract