Clinicopathological Profile and Outcome of C3 Glomerulopathy in Indian Children: Observation from a Single Center
Journal of Pediatric Nephrology,
Vol. 13 (2025),
12 May 2026
https://doi.org/10.22037/jpn.v13i1.48895
Abstract
Background: C3 Glomerulopathy (C3G) is a rare clinical entity caused by Alternate complement pathway dysregulation. Limited published literature is available regarding clinical picture and outcome in Indian children. This study aims to Retrospectively analyze the clinicopathological spectrum and response to Mycophenolate Sodium (MPA) in the treatment of C3G.
Methods: Records of all patients ≤ 18 years diagnosed as C3G between Jan 2017 and Dec 2020 were retrieved after ethical approval. C3 GN and dense deposit disease (DDD) were diagnosed based on EM. Clinical features at presentation and outcomes in terms of response to MPA and death/kidney failure were evaluated. Improvement in (eGFR) by 50% and spot urine protein creatinine ratio (UPCR) < 0.5g/g defined complete remission (CR). Remission was partial (PR) if eGFR improved by 50-25% and spot UPCR was 0.5-3.5g/g. No response was defined as improvement in eGFR by ≤25% and spot UPCR>3.5g/g.
Results: 22 children (9 boys) were diagnosed as C3 Glomerulopathy: C3 GN n=15 (68%) and DDD) n=7 (32%). Age at presentation (median, range) was 7.66,10 years and 9.28,7 years in C3GNand DDD respectively (P = 0.24). Acute nephritic syndrome was the commonest presentation in C3GN (8/15, 53%). Whereas rapidly progressive renal failure was more common in DDD (4/7, 57%). Nephrotic range proteinuria was more common in DDD (85%). All children received prednisolone. Eighteen children received MPA, 3 cyclophosphamide and 1 received cyclosporine. On MPA Ten (55%) had CR and 4 (22.3%) had PR. Four (22.2%) had no response. Out of 4 with no response 3 progressed to kidney failure, 1 expired. Response to treatment was poorer (NR 3, 75%) in children who did not receive MPA(p<0.05).
Conclusion:Children with DDD had more severe presentation and poorer outcome. MMF was useful in treating C3G patients with a higher probability of remission and lower probability of kidney failure.
- C3 glomerulopathy
- Dense deposit disease
- Children
- C3G
- End stage renal Disease
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References
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