Membranoproliferative glomerulonephritis
ICD-10 N04.5 · ICD-11 MF8Y.4

Treatment of Membranoproliferative Glomerulonephritis in Children Younger Than 12 Years with C3 Glomerulopathy or IC-MPGN

Children younger than 12 years with C3 glomerulopathy or primary immune complex membranoproliferative glomerulonephritis (IC-MPGN) represent a distinct and underserved subgroup — one that falls outside the age range of most randomised controlled trials yet frequently presents at precisely this age.

Evidence gap & unmet need Randomised controlled trials have excluded patients aged under 12 years. The available evidence for complement inhibitor therapy in this group derives almost entirely from small cohorts or individual cases of compassionate use. Because disease onset in children often occurs before this age threshold, this gap is clinically urgent.
Treatment approach (partial) Where inflammatory changes are present on biopsy, the current standard of care centres on immunosuppression; consideration of access to a complement inhibitor via clinical trial enrolment or compassionate use / expanded access is part of the decision framework where available.

References

DOI: 10.1016/j.ekir.2025.10.020

Finally, it is important to acknowledge that randomized controlled trials have excluded patients aged < 12 years and as such, the only experience of complement inhibitor therapy in this group comes from small cohorts or individual cases of compassionate use.

This represents an urgent unmet need as the disease onset in children often occurs before 12 years of age.

Where there are inflammatory changes within biopsies, immunosuppression has been used, most commonly glucocorticoid therapy and mycophenolate mofetil, with escalation to B-cell depletion with rituximab considered in IC-MPGN patients with no improvement.

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