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Childhood onset C3 glomerulopathy: recurrence after kidney transplantation-a case series
Yael Borovitz1,2, Daniel Landau1,2, Amit Dagan1,2
1Nephrology Institute, Schneider Children's Medical Center, Petah Tikva, Israel.
Insights
Childhood C3 Glomerulopathy recurrence after kidney transplant is high. Eculizumab shows some benefit, but new treatments are needed for better post-transplant outcomes in C3G patients.
Area of Science:
- Nephrology
- Immunology
- Transplantation
Background:
- C3 Glomerulopathy (C3G) is a complement-mediated kidney disease.
- It involves dysregulation of the alternative complement pathway.
- High recurrence rates and graft failure occur after kidney transplantation (KTx).
Purpose of the Study:
- To investigate C3G recurrence post-KTx in pediatric patients.
- To evaluate treatment outcomes in this population.
Main Methods:
- A case study of pediatric C3G patients undergoing KTx (2015-2023).
- Data included complement analysis, treatments, and outcomes.
- Follow-up assessed graft function and recurrence.
Main Results:
- All five pediatric C3G patients who received KTx experienced recurrence.
- Eculizumab improved graft function in three patients.
- One patient experienced graft failure despite eculizumab, but a second transplant was successful with pre-emptive eculizumab.
Conclusions:
- Pediatric C3G recurrence post-KTx appears common.
- Eculizumab offers potential benefits but is not universally effective.
- Novel therapeutic strategies are crucial for improving outcomes in C3G patients post-transplant.
Background:
C3 Glomerulopathy (C3G) is a complement-mediated disease, with predominant C3 deposits, where pathogenic genetic variants in complement system components and circulating autoantibodies result in loss of control of the alternative pathway, have been described. A high incidence of disease recurrence including graft failure has been reported after kidney transplantation (KTx). Currently treatment modalities for preventing and treating post KTx C3G recurrence (plasma exchange, rituximab and eculizumab) in adults have yielded inconsistent results. Data on post KTx C3G recurrence in childhood-onset C3G is still unknown.
Methods:
A comprehensive case study of patients diagnosed with C3G as children or adolescents, who underwent KTx between the years 2015-2023. Data collected included complement workup, treatment modalities, and outcomes.
Results:
19 patients with C3G were identified during the study period. Five patients developed ESRD and received a kidney transplant. C3G recurrence was diagnosed post KTx in 100% of patients. Graft function improved in 3 of these patients (two with anti-factor H antibodies) after eculizumab treatment, one patient reached graft failure 9 months after transplantation despite eculizumab, recieved a second successful transplantation with pre-emptive eculizumab treatment and one patient showed histologic signs of disease recurrence without clinical signs.
Conclusions:
C3G recurrence after KTx in patients diagnosed as children or adolescents may be higher than previously described. Treatment with eculizumab is beneficial in some patients. New treatments are needed for improving post-transplant outcome in patients with C3G.
