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Original Article

C3 glomerulopathy in children: experience at a resource-limited center

Soumya Reddy¹  , Abhishek Ghante¹, Mahesha Vankalakunti², Anil Vasudevan¹ 

¹Department of Paediatric Nephrology, St Johns Medical College Hospital, Bangalore, India
²Department of Laboratory Medicine, Manipal Hospital, Bangalore, India

Correspondence Anil Vasudevan ,Email: anilvasu@hotmail.com

Received: August 23, 2024; Revised: October 20, 2024   Accepted: October 24, 2024.

Abstract

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 (SD) age was 9 (4) years. The common presentation was acute nephritis (27 [58.6%]), while one in five (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 (C3GN) and four 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 [IQR] duration, 11.5 [6–24] months), six (19.4%) demonstrated complete kidney recovery, while the other 25 (80.7%) had kidney sequelae; of them, five (16.1%) progressed to end-stage kidney disease and two (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.

Keywords :C3 glomerulopathy, Dense deposit disease, Alternate complement pathway, Glomerulonephritis in India children