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Lookup NU author(s): Emily Glover, Dr Kate Smith-Jackson, Dr Vicky Brocklebank, Dr Valerie Wilson, Dr Patrick Walsh, Dr Emma Montgomery, Dr Edwin Wong, Dr Sally Johnson, Dr Michal Malina, Professor David KavanaghORCiD, Professor Neil SheerinORCiD
This work is licensed under a Creative Commons Attribution 4.0 International License (CC BY 4.0).
© 2023 Lippincott Williams and Wilkins. All rights reserved.Background Atypical hemolytic uremic syndrome (aHUS) is a rare cause of end-stage kidney disease and associated with poor outcomes after kidney transplantation from early disease recurrence. Prophylactic eculizumab treatment at the time of transplantation is used in selected patients with aHUS. We report a retrospective case note review describing transplant outcomes in patients with aHUS transplanted between 1978 and 2017, including those patients treated with eculizumab. Methods The National Renal Complement Therapeutics Centre database identified 118 kidney transplants in 86 recipients who had a confirmed diagnosis of aHUS. Thirty-eight kidney transplants were performed in 38 recipients who received prophylactic eculizumab. The cohort not treated with eculizumab comprised 80 transplants in 60 recipients and was refined to produce a comparable cohort of 33 transplants in 32 medium and high-risk recipients implanted since 2002. Complement pathway genetic screening was performed. Graft survival was censored for graft function at last follow-up or patient death. Graft survival without eculizumab treatment is described by complement defect status and by Kidney Disease: Improving Global Outcomes risk stratification. Results Prophylactic eculizumab treatment improved renal allograft survival (P = 0.006) in medium and high-risk recipients with 1-y survival of 97% versus 64% in untreated patients. Our data supports the risk stratification advised by Kidney Disease: Improving Global Outcomes. Conclusions. Prophylactic eculizumab treatment dramatically improves graft survival making transplantation a viable therapeutic option in aHUS.
Author(s): Glover EK, Smith-Jackson K, Brocklebank V, Wilson V, Walsh PR, Montgomery EK, Wong EKS, Johnson S, Malina M, Kavanagh D, Sheerin NS, Ahmed A, Ashby D, Awan A, Baker R, Bhandari S, Bingham C, Bommayya G, Border D, Breen C, Brown H, Brown A, Carmichael P, Chowdhury P, Curran S, Donne R, Dudley C, Dutt T, Eardley K, Eblamo-Abad E, Gale D, Griffin S, Harty J, Hewins P, Holt R, Ingham V, Kilbride H, Kingdon E, Lewis R, Mansfield N, Marks SD, Mason P, Mead P, Morton M, Muniraju T, Nagaraja P, Padmanabhan N, Plant N, Raftery T, Rowe P, Salama A, Shenbagaraman P, Taylor A, Tomlinson K, Torpey N, Uniacke M, Walbaum D, Webb M, Williams M, Woodman A, Shah S, Saif I
Publication type: Article
Publication status: Published
Journal: Transplantation
Year: 2023
Volume: 107
Issue: 4
Pages: 994-1003
Print publication date: 01/04/2023
Online publication date: 31/03/2023
Acceptance date: 07/08/2022
Date deposited: 17/08/2022
ISSN (print): 0041-1337
ISSN (electronic): 1534-6080
Publisher: Lippincott Williams and Wilkins
URL: https://doi.org/10.1097/TP.0000000000004355
DOI: 10.1097/TP.0000000000004355
PubMed id: 36413152
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