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. 2021 Nov 8;16(11):e0258319.
doi: 10.1371/journal.pone.0258319. eCollection 2021.

Thrombotic microangiopathy after kidney transplantation: Analysis of the Brazilian Atypical Hemolytic Uremic Syndrome cohort

Hong Si Nga  1 , Lilian Monteiro Pereira Palma  2 , Miguel Ernandes Neto  1 , Ida Maria Maximina Fernandes-Charpiot  3 , Valter Duro Garcia  4 , Roger Kist  4 , Silvana Maria Carvalho Miranda  5 , Pedro Augusto Macedo de Souza  5 , Gerson Marques Pereira Jr  5 , Luis Gustavo Modelli de Andrade  1
Affiliations

Thrombotic microangiopathy after kidney transplantation: Analysis of the Brazilian Atypical Hemolytic Uremic Syndrome cohort

Hong Si Nga et al. PLoS One. .

Abstract

Background: Atypical Hemolytic Uremic Syndrome (aHUS) is an ultra-rare disease that potentially leads to kidney graft failure due to ongoing Thrombotic Microangiopathy (TMA). The aim was evaluating the frequency of TMA after kidney transplantation in patients with aHUS in a Brazilian cohort stratified by the use of the specific complement-inhibitor eculizumab.

Methods: This was a multicenter retrospective cohort study including kidney transplant patients diagnosed with aHUS. We collected data from 118 transplant centers in Brazil concerning aHUS transplanted patients between 01/01/2007 and 12/31/2019. Patients were stratified into three groups: no use of eculizumab (No Eculizumab Group), use of eculizumab for treatment of after transplantation TMA (Therapeutic Group), and use of eculizumab for prophylaxis of aHUS recurrence (Prophylactic Group).

Results: Thirty-eight patients with aHUS who received kidney transplantation were enrolled in the study. Patients' mean age was 30 years (24-40), and the majority of participants was women (63% of cases). In the No Eculizumab Group (n = 11), there was a 91% graft loss due to the TMA. The hazard ratio of TMA graft loss was 0.07 [0.01-0.55], p = 0.012 in the eculizumab Prophylactic Group and 0.04 [0.00-0.28], p = 0.002 in the eculizumab Therapeutic Group.

Conclusion: The TMA graft loss in the absence of a specific complement-inhibitor was higher among the Brazilian cohort of kidney transplant patients. This finding reinforces the need of eculizumab use for treatment of aHUS kidney transplant patients. Cost optimization analysis and the early access to C5 inhibitors are suggested, especially in low-medium income countries.

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Conflict of interest statement

I declared that the authors Modelli de Andrade LG, Palma LM, and Miranda SMC received fees from Alexion pharmaceutical to Travel grants and honoraria for speaking and participation at meetings. This does not alter our adherence to PLOS ONE policies on sharing data and materials.

Figures

Fig 1
Fig 1. Flowchart study.
Fig 2
Fig 2. Cumulative incidence of TMA graft loss (days) in the Brazilian aHUS cohort patients divided by groups: Not received eculizumab (red), prophylactic eculizumab (green), and eculizumab treatment (blue).
Fig 3
Fig 3. Cumulative survival (days) in the Brazilian aHUS cohort patients divided by groups: Not received eculizumab (red), prophylactic eculizumab (green), and eculizumab treatment (blue).
Fig 4
Fig 4. Outcomes of the Brazilian aHUS cohort patients divided by year of the aHUS diagnosis.
Colors: not received eculizumab (red), prophylactic eculizumab (green) and eculizumab treatment (blue).
See this image and copyright information in PMC

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