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

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 stratifi...

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Autores principales: Nga, Hong Si, Palma, Lilian Monteiro Pereira, Ernandes Neto, Miguel, Fernandes-Charpiot, Ida Maria Maximina, Garcia, Valter Duro, Kist, Roger, Miranda, Silvana Maria Carvalho, Macedo de Souza, Pedro Augusto, Pereira, Gerson Marques, de Andrade, Luis Gustavo Modelli
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Public Library of Science 2021
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8575299/
https://www.ncbi.nlm.nih.gov/pubmed/34748552
http://dx.doi.org/10.1371/journal.pone.0258319
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author Nga, Hong Si
Palma, Lilian Monteiro Pereira
Ernandes Neto, Miguel
Fernandes-Charpiot, Ida Maria Maximina
Garcia, Valter Duro
Kist, Roger
Miranda, Silvana Maria Carvalho
Macedo de Souza, Pedro Augusto
Pereira, Gerson Marques
de Andrade, Luis Gustavo Modelli
author_facet Nga, Hong Si
Palma, Lilian Monteiro Pereira
Ernandes Neto, Miguel
Fernandes-Charpiot, Ida Maria Maximina
Garcia, Valter Duro
Kist, Roger
Miranda, Silvana Maria Carvalho
Macedo de Souza, Pedro Augusto
Pereira, Gerson Marques
de Andrade, Luis Gustavo Modelli
author_sort Nga, Hong Si
collection PubMed
description 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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spelling pubmed-85752992021-11-09 Thrombotic microangiopathy after kidney transplantation: Analysis of the Brazilian Atypical Hemolytic Uremic Syndrome cohort Nga, Hong Si Palma, Lilian Monteiro Pereira Ernandes Neto, Miguel Fernandes-Charpiot, Ida Maria Maximina Garcia, Valter Duro Kist, Roger Miranda, Silvana Maria Carvalho Macedo de Souza, Pedro Augusto Pereira, Gerson Marques de Andrade, Luis Gustavo Modelli PLoS One Research Article 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. Public Library of Science 2021-11-08 /pmc/articles/PMC8575299/ /pubmed/34748552 http://dx.doi.org/10.1371/journal.pone.0258319 Text en © 2021 Nga et al https://creativecommons.org/licenses/by/4.0/This is an open access article distributed under the terms of the Creative Commons Attribution License (https://creativecommons.org/licenses/by/4.0/) , which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
spellingShingle Research Article
Nga, Hong Si
Palma, Lilian Monteiro Pereira
Ernandes Neto, Miguel
Fernandes-Charpiot, Ida Maria Maximina
Garcia, Valter Duro
Kist, Roger
Miranda, Silvana Maria Carvalho
Macedo de Souza, Pedro Augusto
Pereira, Gerson Marques
de Andrade, Luis Gustavo Modelli
Thrombotic microangiopathy after kidney transplantation: Analysis of the Brazilian Atypical Hemolytic Uremic Syndrome cohort
title Thrombotic microangiopathy after kidney transplantation: Analysis of the Brazilian Atypical Hemolytic Uremic Syndrome cohort
title_full Thrombotic microangiopathy after kidney transplantation: Analysis of the Brazilian Atypical Hemolytic Uremic Syndrome cohort
title_fullStr Thrombotic microangiopathy after kidney transplantation: Analysis of the Brazilian Atypical Hemolytic Uremic Syndrome cohort
title_full_unstemmed Thrombotic microangiopathy after kidney transplantation: Analysis of the Brazilian Atypical Hemolytic Uremic Syndrome cohort
title_short Thrombotic microangiopathy after kidney transplantation: Analysis of the Brazilian Atypical Hemolytic Uremic Syndrome cohort
title_sort thrombotic microangiopathy after kidney transplantation: analysis of the brazilian atypical hemolytic uremic syndrome cohort
topic Research Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8575299/
https://www.ncbi.nlm.nih.gov/pubmed/34748552
http://dx.doi.org/10.1371/journal.pone.0258319
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