Related Experiment Video
Updated: Mar 23, 2026
Analyses of Proteinuria, Renal Infiltration of Leukocytes, and Renal Deposition of Proteins in Lupus-prone MRL/lpr Mice
Published on: June 8, 2022
C3 Glomerulopathy and post-infectious glomerulonephritis define a disease spectrum
Badria Al-Ghaithi1, Rahul Chanchlani1,2,3, Magdalena Riedl4
1Division of Nephrology, The Hospital for Sick Children, 555 University Avenue, Toronto, ON, M5G 1X8, Canada.
Insights
Post-infectious glomerulonephritis (PIGN) and C3 glomerulopathy (C3G) may represent a disease spectrum. While PIGN typically resolves, C3G has a poorer prognosis, necessitating distinct management strategies.
Area of Science:
- Nephrology
- Pediatric Nephrology
- Immunology
Background:
- Post-infectious glomerulonephritis (PIGN) typically has a benign course, but severe presentations involve complement alternative pathway (CAP) dysregulation.
- C3 glomerulopathy (C3G), also linked to CAP dysregulation, shares histopathological features with PIGN but has a worse outcome.
- A potential disease spectrum exists between PIGN and C3G, with historical misclassification of C3G as PIGN.
Purpose of the Study:
- To investigate the hypothesis that PIGN and C3G represent a disease spectrum.
- To compare the clinical courses and outcomes of children with PIGN and those reclassified as C3G.
Main Methods:
- Retrospective review of 33 children diagnosed with PIGN between 1985-2010 who had renal biopsies.
- Reclassification of 8 children into C3G based on current criteria.
- Outcome assessment based on proteinuria, C3 levels, and renal function.
Main Results:
- Sixteen (72.7%) children with typical PIGN achieved complete recovery.
- Only 2 (25%) children reclassified as C3G had a favorable outcome.
- Despite more severe initial presentation in typical PIGN, long-term outcomes were better compared to C3G.
Conclusions:
- Findings support PIGN and C3G as part of a disease spectrum.
- Distinguishing between PIGN and C3G is crucial for determining long-term clinical implications.
- Different management strategies are indicated for PIGN and C3G based on their distinct outcomes.
Background:
Post-infectious glomerulonephritis (PIGN) usually follows a benign course, but few children have an atypical, severe presentation, and these exceptional cases have been linked to the dysregulation of the complement alternative pathway (CAP). There is a considerable overlap in the histopathological features of PIGN and C3 glomerulopathy (C3G), which is also associated with CAP dysregulation but has a poorer outcome. We hypothesized that PIGN and C3G define a disease spectrum, and that in the past there may be some children with C3G who were misclassified with PIGN before C3G was described as a separate disease entity.
Methods:
Children with PIGN (n = 33) diagnosed between 1985 and 2010 who underwent a renal biopsy due to their unusual course were reviewed and of them, 8 were reclassified into C3G based on the current classification criteria. Outcome was based on the degree of proteinuria, C3 level, and renal function at follow-up.
Results:
Sixteen (72.7%) children with typical PIGN recovered completely as compared to only 2 (25%) with C3G. Of note, children with "typical" PIGN had a more severe disease course at onset; however, the outcome at last follow up was favorable.
Conclusions:
Our results support the hypothesis that PIGN and C3G form a disease spectrum and have different long-term clinical implications and management strategies.
Related Concept Videos
01:24Nephrotic Syndrome I : Introduction
01:20Renal Corpuscle
Glomerulus: Structure and Function
The glomerulus is a tiny, intricate network of capillaries located at the beginning of the nephron. It's enveloped by the Bowman's capsule and receives its blood supply from an afferent arteriole, which divides into numerous...
01:10Nephrons
01:29Acute Kidney Injury II: Pathophysiology
01:28Chronic Kidney Disease III: Interprofessional Care
01:25Glaucoma: Overview