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Updated: Jun 22, 2025

TBase - an Integrated Electronic Health Record and Research Database for Kidney Transplant Recipients
Published on: April 13, 2021
Updates on C3 Glomerulopathy in Kidney Transplantation: Pathogenesis and Treatment Options
Giulia Bartoli1, Andrea Dello Strologo1, Giuseppe Grandaliano1,2
1Department of Translational Medicine and Surgery, Università Cattolica dl Sacro Cuore, 00168 Rome, Italy.
Insights
C3 glomerulopathy, a rare kidney disease, often recurs after kidney transplants, impacting graft survival. New anti-complement therapies show promise for managing this condition in transplant recipients.
Area of Science:
- Nephrology
- Immunology
- Transplantation
Background:
- C3 glomerulopathy involves abnormal complement alternative pathway activation, leading to C3 deposition in kidneys.
- Disease recurrence affects over 50% of kidney transplant recipients, significantly reducing graft survival.
- Recurrence is a major cause of kidney graft loss, second only to organ rejection.
Purpose of the Study:
- To review the impact of C3 glomerulopathy on kidney grafts.
- To present current and emerging treatment options for C3 glomerulopathy in transplant patients.
Main Methods:
- Literature review summarizing existing data on C3 glomerulopathy recurrence and treatment.
- Analysis of ongoing clinical studies on novel therapeutic agents.
Main Results:
- Identified risk factors for recurrence include delayed graft function, infection, and monoclonal gammopathy.
- Standard treatments like corticosteroids and mycophenolate mofetil have limitations.
- Emerging anti-complement drugs (eculizumab, Ravalizumab, avacopan) show encouraging preliminary results.
Conclusions:
- C3 glomerulopathy poses a significant threat to kidney graft survival post-transplantation.
- Novel anti-complement therapies are needed and demonstrate promising outcomes for managing C3 glomerulopathy recurrence.
Abstract:
C3 glomerulopathy is a rare disease, characterized by an abnormal activation of the complement's alternative pathway that leads to the accumulation of the C3 component in the kidney. The disease recurs in more than half of kidney transplant recipients, with a significant impact on graft survival. Recurrence of the primary disease represents the second cause of graft loss after organ rejection. In C3 glomerulopathy, there are several risk factors which can promote a recurrence during transplantation, such as delayed graft function, infection and monoclonal gammopathy. All these events can trigger the alternative complement pathway. In this review, we summarize the impact of C3 glomerulopathy on kidney grafts and present the latest treatment options. The most widely used treatments for the disease include corticosteroids and mycophenolate mofetil, which are already used chronically by kidney transplant recipients; thus, additional treatments for C3 glomerulopathy are required. Currently, several studies using anti-complement drugs (i.e., eculizumab, Ravalizumab, avacopan) for C3 glomerulopathy in kidney transplant patients are ongoing with encouraging results.
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