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Therapy and outcomes of C3 glomerulopathy and immune-complex membranoproliferative glomerulonephritis
Priyanka Khandelwal1, Swati Bhardwaj1, Geetika Singh2
1Division of Nephrology, ICMR Center for Advanced Research in Nephrology, Department of Pediatrics, All India Institute of Medical Sciences, Ansari Nagar, New Delhi, 110029, India.
Insights
Outcomes for pediatric dense deposit disease (DDD), C3 glomerulonephritis (C3GN), and immune-complex MPGN (IC-MPGN) are often poor, with limited remission rates and significant risk of kidney failure, especially for DDD patients.
Area of Science:
- Nephrology
- Pediatric Nephrology
- Glomerular Diseases
Background:
- Limited data exists on the treatment and outcomes of dense deposit disease (DDD), C3 glomerulonephritis (C3GN), and immune-complex MPGN (IC-MPGN) in pediatric populations.
- These conditions represent significant causes of kidney disease in children, necessitating a better understanding of their clinical course and therapeutic responses.
Purpose of the Study:
- To evaluate the clinicopathological features, treatment responses, and adverse outcomes in children diagnosed with C3 glomerulopathy and IC-MPGN.
- To identify factors associated with remission and long-term kidney survival in this cohort.
Main Methods:
- Retrospective analysis of kidney biopsies from pediatric patients (<18 years) with C3 glomerulopathy and IC-MPGN between 2007 and 2019.
- Patients were classified using immunohistochemistry and electron microscopy; treatment regimens included prednisolone, mycophenolate mofetil, tacrolimus, and/or IV cyclophosphamide.
- Clinicopathological data, treatment response, and adverse outcomes (eGFR < 15 mL/min/1.73 m², or death) were assessed.
Main Results:
- Out of 92 patients, 48 had DDD, 26 had C3GN, and 18 had IC-MPGN. Complete or partial remission was achieved in only 28.5% (DDD), 36.1% (C3GN), and 16.7% (IC-MPGN) of patients.
- Low serum albumin (<2.5 g/dL) and persistently low serum C3 levels were associated with a lack of remission.
- Five-year kidney survival was 62.6% for DDD, 85.5% for C3GN, and 88.5% for IC-MPGN. Rapidly progressive GN, age > 10 years at onset, and DDD were independent predictors of adverse outcomes.
Conclusions:
- The outcomes for pediatric C3 glomerulopathy and IC-MPGN are generally unsatisfactory, with a low proportion achieving remission.
- Dense deposit disease (DDD) presents a higher risk for rapidly progressive GN and adverse kidney outcomes, including failure.
- Achieving remission is a significant protective factor against adverse outcomes in these pediatric kidney diseases.
Background:
Data on therapy and outcome of dense deposit disease (DDD), C3 glomerulonephritis (C3GN), and immune-complex MPGN (IC-MPGN) in children are limited.
Methods:
In this retrospective single-center study from 2007 to 2019, kidney biopsies were reviewed to include patients aged <18-years with C3 glomerulopathy and IC-MPGN. Initial immunosuppression comprised prednisolone, mycophenolate mofetil (n = 51), tacrolimus (n = 11), and/or IV cyclophosphamide (n = 20). Clinicopathological features, response to therapy, and adverse outcome (eGFRcr < 15 mL/min/1.73 m2 or death) were evaluated.
Results:
A total of 92 patients were classified as DDD (n = 48, 52.2%), C3GN (n = 26, 28.3%), and IC-MPGN (n = 18, 19.6%) by immunohistochemistry and electron microscopy; 8 patients with DDD were misclassified as IC-MPGN on immunofluorescence. At last follow-up (median 4.3 years), complete or partial remission occurred in 28.5, 36.1, and 16.7% patients with DDD, C3GN, and IC-MPGN, respectively. Serum albumin at onset < 2.5 g/dL (HR = 0.29, P = 0.005) and persistently low serum C3 (HR = 0.34, P = 0.02) were associated with lack of remission. The 5-year kidney survival was 62.6, 85.5, and 88.5% in patients with DDD, C3GN, and IC-MPGN, respectively (log-rank, P = 0.006). Presentation as rapidly progressive GN (HR = 11.2, P < 0.001), age > 10 years at onset (HR = 4.0, P = 0.004), and DDD (HR = 4.2, P = 0.02) were independently associated with adverse outcome; achieving remission was protective (HR = 0.04; P < 0.001).
Conclusion:
Outcome in patients with C3 glomerulopathy and IC-MPGN was unsatisfactory, and only a small proportion of patients achieved complete or partial remission. Patients with DDD were more likely to present with rapidly progressive GN and were at higher risk of adverse outcomes, including kidney failure.
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