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

Treatment of Membranoproliferative Glomerulonephritis in Adolescents Aged 12–17 with C3 Glomerulopathy or Primary IC-MPGN

Clinical Scenario

This protocol addresses adolescents aged 12 to 17 years diagnosed with C3 glomerulopathy (C3G) or primary immune complex membranoproliferative glomerulonephritis (IC-MPGN) — a patient population for whom a specific, evidence-based first-line treatment approach is now established.

Treatment Goals

The primary aims are a meaningful reduction in proteinuria and stabilization of kidney function. Achieving a significant proteinuria threshold by 12 months is associated with substantially lower long-term risk of kidney failure; even stabilization of eGFR at 6 months can be considered a meaningful indicator of response.

Treatment Approach — Partial Overview

The approach involves adding a complement inhibitor — pegcetacoplan — on top of continued supportive care. The complete regimen, including dosing, administration details, and full protocol steps, is available in the structured protocol below.

Full protocol required for dosing & clinical details →
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References

DOI: 10.1016/j.ekir.2025.10.020
  1. In 2025, pegcetacoplan was also approved by the US Food and Drug Administration for the treatment of adult and pediatric patients aged $ 12 years with C3G or primary IC-MPGN to reduce proteinuria.
  2. Adolescents (12–17 yrs old), n (%)
  3. Pegcetacoplan is administered as a subcutaneous injection at a recommended dose of 1080 mg (in 20 ml) twice a week for adults using a commercially available infusion pump or on-body injector device, where available.
  4. For both C3G and primary IC-MPGN, a ≥ 50% proteinuria reduction after 12 months has consistently been associated with significantly lower risk of kidney failure, as demonstrated by both the UK RaDaR and Spanish GLOSEN registries.
  5. patients who achieve a UPCR < 0.88 g/g (< 100 mg/mmol) at 12 months after diagnosis benefit from a 90% lower risk of kidney failure than those who did not achieve this threshold.
  6. In this context, even stabilization of eGFR may be considered a success after 6 months of complement inhibitor therapy, although it must be acknowledged that changes in eGFR are dependent on factors such as disease chronicity.
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