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Complement-Amplifying Conditions in Atypical Hemolytic Uremic Syndrome: A Canadian Case Series

RATIONALE: Thrombotic microangiopathies (TMAs) are systemic disorders that often affect the kidneys and encompass a heterogeneous group of conditions, including atypical hemolytic uremic syndrome (aHUS). The complement pathway is thought to play a crucial role in the pathogenesis of aHUS, and a favo...

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Autores principales: Patriquin, Christopher J., Pavenski, Katerina, Garland, Jocelyn, Girard, Louis-Philippe, Isenring, Paul
Formato: Online Artículo Texto
Lenguaje:English
Publicado: SAGE Publications 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9125052/
https://www.ncbi.nlm.nih.gov/pubmed/35615072
http://dx.doi.org/10.1177/20543581221100288
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author Patriquin, Christopher J.
Pavenski, Katerina
Garland, Jocelyn
Girard, Louis-Philippe
Isenring, Paul
author_facet Patriquin, Christopher J.
Pavenski, Katerina
Garland, Jocelyn
Girard, Louis-Philippe
Isenring, Paul
author_sort Patriquin, Christopher J.
collection PubMed
description RATIONALE: Thrombotic microangiopathies (TMAs) are systemic disorders that often affect the kidneys and encompass a heterogeneous group of conditions, including atypical hemolytic uremic syndrome (aHUS). The complement pathway is thought to play a crucial role in the pathogenesis of aHUS, and a favorable response can be obtained through complement C5 inhibition. There is emerging evidence to suggest that the same is also true for several other forms of TMA. OBJECTIVE: The purpose of this series is to report cases of aHUS in which both an innate defect of the alternative complement pathway and a complement-amplifying condition were suspected. METHODS: This case series describes 8 patients who were managed in Canadian tertiary centers for aHUS and who presented initially with complement-amplifying conditions. RESULTS: In all cases, aHUS was associated with organ dysfunction and in some, with an innate defect of the alternative complement pathway. The complement-amplifying conditions identified were diverse including immune disorders, pregnancy, and a Shiga toxin infection. Patients improved rapidly when treated with eculizumab or plasma exchange. CONCLUSIONS: These observations illustrate the seriousness of secondary aHUS. They also add to existing lines of evidence that the complement pathway is potentially involved in this condition and that it should be considered as a therapeutic target of interest under such circumstances.
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spelling pubmed-91250522022-05-24 Complement-Amplifying Conditions in Atypical Hemolytic Uremic Syndrome: A Canadian Case Series Patriquin, Christopher J. Pavenski, Katerina Garland, Jocelyn Girard, Louis-Philippe Isenring, Paul Can J Kidney Health Dis Original Basic Research RATIONALE: Thrombotic microangiopathies (TMAs) are systemic disorders that often affect the kidneys and encompass a heterogeneous group of conditions, including atypical hemolytic uremic syndrome (aHUS). The complement pathway is thought to play a crucial role in the pathogenesis of aHUS, and a favorable response can be obtained through complement C5 inhibition. There is emerging evidence to suggest that the same is also true for several other forms of TMA. OBJECTIVE: The purpose of this series is to report cases of aHUS in which both an innate defect of the alternative complement pathway and a complement-amplifying condition were suspected. METHODS: This case series describes 8 patients who were managed in Canadian tertiary centers for aHUS and who presented initially with complement-amplifying conditions. RESULTS: In all cases, aHUS was associated with organ dysfunction and in some, with an innate defect of the alternative complement pathway. The complement-amplifying conditions identified were diverse including immune disorders, pregnancy, and a Shiga toxin infection. Patients improved rapidly when treated with eculizumab or plasma exchange. CONCLUSIONS: These observations illustrate the seriousness of secondary aHUS. They also add to existing lines of evidence that the complement pathway is potentially involved in this condition and that it should be considered as a therapeutic target of interest under such circumstances. SAGE Publications 2022-05-19 /pmc/articles/PMC9125052/ /pubmed/35615072 http://dx.doi.org/10.1177/20543581221100288 Text en © The Author(s) 2022 https://creativecommons.org/licenses/by/4.0/This article is distributed under the terms of the Creative Commons Attribution 4.0 License (https://creativecommons.org/licenses/by/4.0/) which permits any use, reproduction and distribution of the work without further permission provided the original work is attributed as specified on the SAGE and Open Access pages (https://us.sagepub.com/en-us/nam/open-access-at-sage).
spellingShingle Original Basic Research
Patriquin, Christopher J.
Pavenski, Katerina
Garland, Jocelyn
Girard, Louis-Philippe
Isenring, Paul
Complement-Amplifying Conditions in Atypical Hemolytic Uremic Syndrome: A Canadian Case Series
title Complement-Amplifying Conditions in Atypical Hemolytic Uremic Syndrome: A Canadian Case Series
title_full Complement-Amplifying Conditions in Atypical Hemolytic Uremic Syndrome: A Canadian Case Series
title_fullStr Complement-Amplifying Conditions in Atypical Hemolytic Uremic Syndrome: A Canadian Case Series
title_full_unstemmed Complement-Amplifying Conditions in Atypical Hemolytic Uremic Syndrome: A Canadian Case Series
title_short Complement-Amplifying Conditions in Atypical Hemolytic Uremic Syndrome: A Canadian Case Series
title_sort complement-amplifying conditions in atypical hemolytic uremic syndrome: a canadian case series
topic Original Basic Research
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9125052/
https://www.ncbi.nlm.nih.gov/pubmed/35615072
http://dx.doi.org/10.1177/20543581221100288
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