Glomerulonefritis membranoproliferativa
ICD-10 N04.5 · ICD-11 MF8Y.4

Tratamiento de la Glomerulonefritis Membranoproliferativa con Glomerulopatía C3 en Adultos (18+)

Adultos ≥ 18 años

Este protocolo está dirigido a adultos de 18 años o más diagnosticados con glomerulopatía C3 (C3G) — un patrón específico de glomerulonefritis membranoproliferativa — que afecta a un riñón nativo o a un riñón post-trasplante.


Los objetivos clave son una reducción sustancial y sostenida de la proteinuria (evaluada a las 26 semanas y de nuevo a los 12 meses), la estabilización de la TFGe a los 6 meses y la reducción de la tinción glomerular de C3 en la biopsia renal en la semana 26.


La evidencia actual apoya la adición de un inhibidor proximal del complemento sobre el tratamiento de soporte continuo en adultos con proteinuria significativa al inicio. El protocolo completo especifica los agentes elegibles, sus vías de administración y todos los criterios relevantes.

References

DOI: 10.1016/j.ekir.2025.10.020

In 2025, iptacopan was also approved by the US Food and Drug Administration for the treatment of adults with C3G to reduce proteinuria, and by the European Medicines Agency for the treatment of adults with C3G in combination with a renin-angiotensin-aldosterone system inhibitor or as monotherapy in patients who are renin-angiotensin-aldosterone system inhibitor-intolerant.

The phase 3 trial data suggest that we should be using these agents in patients with significant proteinuria at the outset.

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.

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.

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.

Pegcetacoplan treatment resulted in 74.3% (26/35) of patients achieving a meaningful reduction in staining (≥ 2 point improvement on the 0–3 scale) by week 26 and 71.4% (25/35) had zero C3 staining in the biopsy after treatment.

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