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C3 Glomerulopathy: recent advances and an update on management.
Lilian Monteiro Pereira Palma1, Maria Izabel Neves de Holanda Barbosa2, Sanjeev Sethi3
1Universidade Estadual de Campinas, Faculdade de Ciências Médicas, Nefrologia Pediátrica, Campinas, SP, Brazil.
C3 glomerulopathy (C3G) is a kidney disease causing inflammation and often leading to kidney failure. New complement inhibitor treatments show promise in improving patient outcomes and slowing disease progression.
Area of Science:
- Nephrology
- Immunology
- Pathology
Background:
- C3 glomerulopathy (C3G) is defined by C3 deposition in glomeruli, encompassing C3 glomerulonephritis (C3GN) and dense deposit disease (DDD).
- C3G is a progressive condition with high rates of end-stage kidney disease (ESKD) and post-transplant recurrence.
- Understanding C3G pathophysiology involves alternative complement pathway dysregulation, histopathology, and proteomic analyses.
Purpose of the Study:
- To review the evolving understanding of C3 glomerulopathy pathophysiology.
- To highlight recent advancements in treatment strategies for C3G.
- To emphasize the role of complement pathway inhibition in managing C3G.
Main Methods:
- Literature review of C3G pathophysiology, histopathology, and clinical trials.
- Analysis of diagnostic criteria including immunofluorescence and electron microscopy.
- Evaluation of treatment outcomes for complement inhibitors.
Main Results:
- Dysregulation of the alternative complement pathway is central to C3G pathogenesis.
- Newer treatments targeting complement pathways show significant improvements in proteinuria and GFR.
- Individualized treatment approaches are becoming feasible based on pathophysiological profiles.
Conclusions:
- Targeting the alternative complement pathway offers a promising therapeutic strategy for C3G.
- Complement inhibitors represent a significant advancement in C3G management.
- Further research into C3G subtypes and treatment responses is warranted.
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