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

Tratamento de Glomerulonefrite Membranoproliferativa com Glomerulopatia C3 em Adultos (18+)

Adultos ≥ 18 anos

Este protocolo destina-se a adultos com 18 anos ou mais diagnosticados com glomerulopatia C3 (C3G) — um padrão específico de glomerulonefrite membranoproliferativa — afetando rim nativo ou pós-transplante.


Os principais objetivos são uma redução substancial e sustentada da proteinúria (avaliada às 26 semanas e novamente aos 12 meses), estabilização da TFGe em 6 meses, e redução da coloração glomerular de C3 na biópsia renal até a semana 26.


As evidências atuais apoiam a adição de um inibidor proximal do complemento além dos cuidados de suporte contínuos em adultos com proteinúria significativa no início do tratamento. O protocolo completo especifica os agentes elegíveis, suas vias de administração e todos os critérios 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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