Work

A Decade of C3 Glomerulopathy—A Nationwide Cohort Study

Year: 2025

Type: article

Abstract: Introduction C3 glomerulopathy (C3G) is a rare but devastating disease affecting children and adults. It frequently leads to end stage kidney failure and currently no specific treatment exists. C3G is used as a collective term for dense deposit disease (DDD) and C3 glomerulonephritis (C3GN) and is thought to sometimes occur in post-infectious settings (C3-PIGN). Currently little is known about the incidence and distribution of subtypes throughout the population. We analyzed a large cohort of C3G patients in the Netherlands regarding incidence, disease subtype distribution in relation to geographical factors and histopathological findings. Methods A search in the Dutch Nationwide Pathology Databank (Palga) was performed to identify patients diagnosed with C3G from January 2014 until December 2023, subsequently ascertained by two independent observers. We assessed the correlation of disease subtypes with glomerular patterns, and the geographical distribution was charted. Results The selection resulted in a cohort of 280 patients consisting of C3GN (n=101), DDD (n=39), unspecified C3G (n=106), C3-PIGN (n=29) and others (n=5). The median age at biopsy diagnosis was 19 (range 4-75) for DDD and 54 (range 2-86) for C3GN, showing age distribution depends on C3G subtype (p<0.001). DDD and C3G were associated with membranoproliferative pattern and C3-PIGN with endocapillary or exudative pattern. Conclusion Our results show consistent assessment of kidney biopsies across the country and absence of geographical factors influencing disease development .

Source: Kidney International Reports

DOI: 10.1016/j.ekir.2025.11.007

Authors: Rick H. Overwijk, Fiona R. Kolbinger, Mark Eijgelsheim, Olaf M. Dekkers, Andreas Kronbichler, Ingeborg M. Bajema