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C3 glomerulopathy in children: experience at a resource-limited center
Soumya Reddy1, Abhishek Ghante1, Mahesha Vankalakunti2
1Department of Pediatric Nephrology, St. John's Medical College Hospital, St. John's National Academy of Health Sciences, Bengaluru, India.
Insights
Pediatric C3 glomerulopathy (C3G) presents diversely, often with acute nephritis. Limited diagnostic tools in resource-limited settings hinder comprehensive management, leading to significant kidney morbidity and progression to end-stage kidney disease.
Area of Science:
- Nephrology
- Pediatric Nephrology
- Glomerular Diseases
Background:
- C3 glomerulopathy (C3G) in children exhibits varied clinical and pathological features.
- Diagnostic challenges in resource-limited settings include lack of complement assays and advanced testing.
- This heterogeneity impacts patient outcomes and management strategies.
Purpose of the Study:
- To characterize the clinicopathological spectrum of pediatric C3G in a resource-limited environment.
- To assess the effectiveness of immunosuppressive therapy in these patients.
- To evaluate the renal outcomes and long-term prognosis of children with C3G.
Main Methods:
- Retrospective cohort study of 46 children diagnosed with C3G via kidney biopsy (2013-2021).
- Analysis of clinical, laboratory, treatment, and outcome data at presentation and during follow-up.
- Limited use of electron microscopy; no complement assays or genetic testing performed.
Main Results:
- Common presentations included acute nephritis (58.6%) and rapidly progressive glomerulonephritis (19.5%).
- Focal crescentic glomerulonephritis was the predominant histological pattern (30.4%).
- Only 19.4% achieved complete kidney recovery; 16.1% progressed to end-stage kidney disease, and 4.3% died.
Conclusions:
- Pediatric C3G shows a broad clinicopathological range, with dense deposit disease (DDD) being less frequent.
- Most children experience significant morbidity, underscoring the need for better diagnostic capabilities.
- Absence of genetic and C3Nephritic factor testing impedes precise phenotyping and optimal management in resource-limited settings.
Background:
In children, C3 glomerulopathy (C3G) is a heterogeneous disease characterized by diverse clinicopathological profiles and kidney outcomes. However, diagnostic work-up in resource-limited settings is challenging because of the unavailability of complement assays and limited access to electron microscopy or genetic testing.
Purpose:
This study aimed to describe the clinicopathological features and response to immunosuppression and evaluate renal outcomes among children with C3G in a resource-limited setting.
Methods:
This retrospective cohort study involved a review of the hospital records of 46 children (2013-2021) diagnosed with C3G on kidney biopsy. Their clinical, laboratory, treatment, and outcome details at onset and follow-up were noted.
Results:
The mean (standard deviation) age was 9 (4) years. The common presentation was acute nephritis (27 [58.6%]), while 1 in 5 (19.5%) presented with rapidly progressive glomerulonephritis. Focal crescentic glomerulonephritis (14 [30.4%]) was the common histological pattern. Electron microscopy was performed in 22 (47.8%), of which 17 were C3 glomerulonephritis and 4 were dense deposit disease (DDD). None of the patients underwent complement assay or genetic testing. Almost two-thirds (63%) received empirical immunosuppressive therapy, most commonly steroids. Of the 31/46 who completed follow-up (median [interquartile range] duration, 11.5 [6-24] months), 6 (19.4%) demonstrated complete kidney recovery, while the other 25 (80.7%) had kidney sequelae; of them, 5 (16.1%) progressed to end-stage kidney disease and 2 (4.3%) died by the last follow-up.
Conclusion:
Pediatric C3G has a variable clinicopathological spectrum, while DDD is less common. Most patients present with glomerulonephritis and significant morbidities. The lack of genetic and C3Nephritic factor testing is a barrier to the comprehensive phenotyping and management of C3G in resource-limited settings.
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