Pediatric C3 glomerulopathy: a 12-year single-center experience

Complement component 3 glomerulopathy (C3G) is a disease with limited data in children. We aimed to compare childhood C3G cases with adults. We also studied subgroups of pediatric C3G and predictors of poor outcome.

This is a 12-year retrospective, single-center cohort, observational study. All cases of C3G were defined based on the 2013 consensus guidelines.

C3G was diagnosed in 162 patients (119 adults, 43 pediatric) predominantly affecting males. With varied light microscopic patterns, pediatric C3G cases were categorized as follows: 23 C3 glomerulonephritis (C3GN) and 11 dense deposit disease (DDD) on electron microscopy. The pediatric DDD patients were relatively younger with more severe disease at presentation (more crescents in biopsy) but with lesser chronicity in biopsy compared with pediatric C3GN patients; however, both had a similar outcome. On comparing pediatric and adult C3G cases, adults had lower median eGFR and a higher degree of chronicity in the biopsy. The prognosis of C3G was better in pediatric patients. Predictors of kidney failure in pediatric C3G were low eGFR (HR = 0.82, p = 0.05) and severe interstitial fibrosis/tubular atrophy (HR = 1.05, p = 0.02).

Electron microscopy-based subgroups of pediatric C3G differ in clinical presentation and course of the disease but have similar prognosis and long-term outcomes. Pediatric C3G differs from adult C3G with respect to presentation, laboratory results, biopsy features, treatment, and outcome, and as such, it should be considered as a separate entity rather than a smaller version of adult C3G.