A Narrative Review of C3 Glomerulopathy: From Pathogenesis to Targeted Therapy - PubMed
2 hours ago
- #complement system
- #targeted therapy
- #C3 glomerulopathy
- C3 glomerulopathy (C3G) is an ultra-rare, progressive kidney disease driven by dysregulation of the alternative complement pathway.
- The disease involves deposition of complement proteins in kidney glomeruli, leading to inflammation, tissue damage, and often proliferative glomerulonephritis.
- Nearly half of C3G patients develop kidney failure within 10 years, with high recurrence rates after kidney transplant.
- Severity of proteinuria and reduced eGFR are key clinical predictors of kidney failure, while biomarkers need further study for prognostic utility.
- There is no established standard of care or approved therapies for C3G in Canada, highlighting significant unmet needs.
- Novel complement-directed therapies like iptacopan and pegcetacoplan show promise in reducing proteinuria and stabilizing kidney function, including post-transplant.
- This review synthesizes current knowledge for Canadian nephrologists, incorporating patient perspectives and expert clinical insights, despite limitations like lack of systematic review and potential bias.