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Kidney Week

Abstract: PO1656

Outcome of Kidney Transplantation in Atypical Hemolytic Uremic Syndrome Without Eculizumab Prophylaxis: A Single-Center Experience

Session Information

Category: Glomerular Diseases

  • 1203 Glomerular Diseases: Clinical, Outcomes, and Trials

Authors

  • Duineveld, Caroline, Radboudumc, Nijmegen, Gelderland, Netherlands
  • Bouwmeester, Romy N., Radboudumc, Nijmegen, Gelderland, Netherlands
  • Wijnsma, Kioa L., Radboudumc, Nijmegen, Gelderland, Netherlands
  • Van De Kar, Nicole, Radboudumc, Nijmegen, Gelderland, Netherlands
  • Wetzels, Jack F., Radboudumc, Nijmegen, Gelderland, Netherlands
Background

A high risk of aHUS recurrence (60-80%) is reported after kidney transplantation. Therefore, it is suggested to perform kidney transplantation in aHUS patients with eculizumab prophylaxis. In 2017 we reported a favorable outcome after kidney transplantation in aHUS patients without eculizumab prophylaxis, using kidneys from living donors and a transplantation protocol aimed at reduction of endothelial injury.(1) Here, we present the results of our treatment protocol with prolonged follow-up.

Methods

All patients with a previous history of aHUS who received a living or deceased donor kidney transplantation in the Radboud University medical center between 2011 and 2020 were evaluated.

Results

We included 26 aHUS patients (M 9; F 17, median age at transplantation 47y, range 22-69). In 22 patients (85%) 24 genetic variants were found: C3 (N=14), CFH (N=8), CFB (N=2). Recurrence risk was considered high in 18 patients and moderate in 8 patients. Nineteen patients received a graft from a living donor (LD) and 7 patients a graft from a brain-death deceased donor (DBD). All patients were treated with low-dose tacrolimus. Six patients (23%) developed aHUS recurrence (4/19 LD, 2/7 DBD) and were treated with eculizumab. Of note, recurrence occurred >12 months after transplantation in two patients. No patient lost the graft due to aHUS recurrence. One patient lost the graft due to rejection and BK nephropathy, one patient died with a functioning graft due to infections. After a median follow-up of 63.6 months (range 12-116) median eGFR was 53.5 ml/min/1.73m2, and proteinuria was negligible (median urine protein-creatinine ratio 0.12 g/10mmol, range 0.04-0.4).

Conclusion

Kidney transplantation without eculizumab prophylaxis is feasible and safe with a relatively low recurrence rate. Atypical HUS recurrence may present late after kidney transplantation and may not be prevented by short course eculizumab prophylaxis.

References: (1) Duineveld, C., et al., Living Donor Kidney Transplantation in Atypical Hemolytic Uremic Syndrome: A Case Series. Am J Kidney Dis, 2017. 70(6): p. 770-777.