A pediatric case of C3 glomerulonephritis initially misclassified as IgA nephropathy with a favorable response to C3-targeted therapy - PubMed
4 hours ago
- #C3 glomerulonephritis
- #Pegcetacoplan
- #Complement pathway
- A 13-year-old girl initially diagnosed with IgA nephropathy was later correctly identified as having C3 glomerulonephritis (C3GN).
- Despite standard immunosuppressive treatment, the patient only achieved partial remission and relapsed, showing persistent C3 consumption and C5b9 activation.
- A repeat biopsy confirmed C3GN with a membranoproliferative pattern, and genetic testing found no pathological variants.
- The patient showed no response to immunosuppressive therapy for nearly two years but achieved complete remission within three months of starting the C3 inhibitor pegcetacoplan.
- This case underscores the diagnostic challenges of C3GN and highlights the potential effectiveness of C3-targeted therapy.