Kidney Week

Abstract: FR-PO968

10 Years of C3 Glomerulopathy in the Netherlands: A Large Cohort of a Rare Kidney Disease

Session Information

• Pathology and Lab Medicine - 1
  October 25, 2024 | Location: Exhibit Hall, Convention Center
  Abstract Time: 10:00 AM - 12:00 PM

Category: Pathology and Lab Medicine

• 1800 Pathology and Lab Medicine

Authors

• Overwijk, Rick H., Universitair Medisch Centrum Groningen, Groningen, Groningen, Netherlands

Rick H. Overwijk, MD

• Kolbinger, Fiona R., Purdue University, West Lafayette, Indiana, United States

Fiona R. Kolbinger, MD, DrMed

• Eijgelsheim, Mark, Universitair Medisch Centrum Groningen, Groningen, Groningen, Netherlands

Mark Eijgelsheim, MD, MSc, PhD

• Kronbichler, Andreas, Medical University Innsbruck, Innsbruck, Austria

Andreas Kronbichler, MD, PhD

• Bajema, Ingeborg M., Universitair Medisch Centrum Groningen, Groningen, Groningen, Netherlands

Ingeborg M. Bajema, MD, PhD

Background

C3 glomerulopathy (C3G) is a rare but devastating disease affecting both children and adults. It frequently leads to end stage kidney disease within ten years after diagnosis and 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 occurrence and distribution throughout the population. In this study we analyzed a large cohort of C3G patients in the Netherlands regarding disease distribution in relation to geographical factors and histopathological findings.

Methods

A search in the Dutch national pathology database (PALGA) was performed. All patients diagnosed with C3G from January 2014 until December 2023 were identified by two independent observers. Cross tabulations were used to determine the correlation of disease subtypes and glomerular patterns, and their geographical distribution. Non-parametric analysis was used to evaluate the age distribution at diagnosis.

Results

The selection resulted in a cohort of 280 patients consisting of: C3GN (101), DDD (39), unspecified C3G (106), C3-PIGN (29) and other (5). The median age of diagnosis was 19 for DDD and 58 for C3GN, showing that age distribution depends on C3G subtype (p<0.001). C3G subtypes were also associated with glomerulonephritis patterns (p<0.001), with DDD and C3G associated with mesangiocapillary pattern and C3-PIGN with endocapillary or exudative pattern.

Conclusion

This large cohort is a stepping stone for better characterizing contributing factors in the etiology of C3G and can potentially demystify this rare and devastating disease.