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Late Onset Thrombotic Microangiopathy in Kidney Transplants; Poor Outcome Despite Eculizumab Treatment

Lookup NU author(s): Emily Glover, Dr Emma Montgomery, Dr Edwin Wong, Dr Michal Malina, Professor Kevin MarchbankORCiD, Professor David KavanaghORCiD, Professor Neil SheerinORCiD

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This work is licensed under a Creative Commons Attribution 4.0 International License (CC BY 4.0).


Abstract

Atypical hemolytic uremic syndrome (aHUS) is a rare cause of end stage kidney disease (ESKD) associated with a high rate of recurrence in kidney transplants causing a post-transplant thrombotic microangiopathy (TMA). Prophylactic eculizumab can prevent disease recurrence in select patients. Treating at the time of post-transplant TMA occurrence is the only option if the diagnosis of aHUS is not established pre-transplant. We report our experience of using eculizumab at the point of post-transplant TMA in those with a diagnosis or suspicion of aHUS. We conducted a case note review of 26 patients treated with eculizumab for post-transplant TMA. Screening for complement pathway defects included testing for variants in genes of the complement pathway and anti-factor H autoantibodies. 34.6% of recipients had an identified complement pathway defect. Median time to presentation with post-transplant TMA was 8.4 months. Death-censored graft survival 12 months after starting eculizumab was 68% for the cohort and was worse in those presenting >12 months post-transplant where this figure was 42.9%. The outcome is poor despite eculizumab treatment for those presenting >12 months after transplantation with TMA.


Publication metadata

Author(s): Glover EK, Montgomery EK, Wong EKS, Johnson S, Malina M, Marchbank KJ, Kavanagh D, Sheerin NS

Publication type: Article

Publication status: Published

Journal: Transplant International

Year: 2025

Volume: 38

Online publication date: 24/11/2025

Acceptance date: 11/11/2025

Date deposited: 11/11/2025

ISSN (print): 0934-0874

ISSN (electronic): 1432-2277

Publisher: Frontiers

URL: https://doi.org/10.3389/ti.2025.15404

DOI: 10.3389/ti.2025.15404

Data Access Statement: The original contributions presented in the study are included in the article/Supplementary Material, further inquiries can be directed to the corresponding author.


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Funding

Funder referenceFunder name
Wellcome Trust (203914/Z/16/Z)

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