Expert statements on the standard of care in critically ill adult patients with atypical haemolytic uraemic syndrome

Elie Azoulay; Paul Knoebl; José Garnacho-Montero; Katerina Rusinova; Gennadii Galstian; Philippe Eggimann; Fekri Abroug; Dominique Benoit; Michael von Bergwelt-Baildon; Julia Wendon; Marie Scully

doi:10.1016/j.chest.2017.03.055

Expert statements on the standard of care in critically ill adult patients with atypical haemolytic uraemic syndrome

Elie Azoulay, Paul Knoebl, José Garnacho-Montero, Katerina Rusinova, Gennadii Galstian, Philippe Eggimann, Fekri Abroug, Dominique Benoit, Michael von Bergwelt-Baildon, Julia Wendon, Marie Scully

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Abstract

Background Atypical haemolytic uraemic syndrome (aHUS) presents similarly to thrombotic thrombocytopenic purpura (TTP), and other causes or conditions with thrombotic microangiopathy (TMA) such as DIC or sepsis. Similarity in clinical presentation may hinder diagnosis and optimal treatment selection in the urgent setting in the ICU. However, there is currently no consensus on the diagnosis or treatment of aHUS for ICU specialists. This review aims to summarise available data on the diagnosis and treatment strategies of aHUS in the ICU in order to enhance the understanding of aHUS diagnosis and outcomes in patients managed in the ICU. Methods A review of the recent literature (January 2009 – March 2016) was performed to select the most relevant articles for ICU physicians. Results Based on the paucity of adult aHUS cases overall and within the ICU, no specific recommendations could be formally graded for the critical care setting. However, the expert panel recognises a core set of skills required by intensivists for diagnosing and managing patients with aHUS: recognising thrombotic microangiopathies, differentiating aHUS from related conditions, recognising involvement of other organ systems, understanding the pathophysiology of aHUS, knowing the diagnostic workup and relevant outcomes in critically ill aHUS patients, and knowing the standard of care for patients with aHUS based on available data and guidelines. Conclusions Managing critically ill patients with aHUS requires basic skills that, in the absence of sufficient data from patients treated within the ICU, can be gleaned from an increasingly relevant literature outside the ICU. More data on critically ill patients with aHUS are needed to validate these conclusions within the ICU setting.

Original language	English
Journal	Chest
Early online date	23 Apr 2017
DOIs	https://doi.org/10.1016/j.chest.2017.03.055
Publication status	E-pub ahead of print - 23 Apr 2017

Keywords

Atypical haemolytic uraemic syndrome
thrombocytopenia
thrombotic microangiopathy
organ failure
plasma exchange
eculizumab

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@article{30a34021a91f4db397820bf7fc5a94f5,

title = "Expert statements on the standard of care in critically ill adult patients with atypical haemolytic uraemic syndrome",

abstract = "Background Atypical haemolytic uraemic syndrome (aHUS) presents similarly to thrombotic thrombocytopenic purpura (TTP), and other causes or conditions with thrombotic microangiopathy (TMA) such as DIC or sepsis. Similarity in clinical presentation may hinder diagnosis and optimal treatment selection in the urgent setting in the ICU. However, there is currently no consensus on the diagnosis or treatment of aHUS for ICU specialists. This review aims to summarise available data on the diagnosis and treatment strategies of aHUS in the ICU in order to enhance the understanding of aHUS diagnosis and outcomes in patients managed in the ICU. Methods A review of the recent literature (January 2009 – March 2016) was performed to select the most relevant articles for ICU physicians. Results Based on the paucity of adult aHUS cases overall and within the ICU, no specific recommendations could be formally graded for the critical care setting. However, the expert panel recognises a core set of skills required by intensivists for diagnosing and managing patients with aHUS: recognising thrombotic microangiopathies, differentiating aHUS from related conditions, recognising involvement of other organ systems, understanding the pathophysiology of aHUS, knowing the diagnostic workup and relevant outcomes in critically ill aHUS patients, and knowing the standard of care for patients with aHUS based on available data and guidelines. Conclusions Managing critically ill patients with aHUS requires basic skills that, in the absence of sufficient data from patients treated within the ICU, can be gleaned from an increasingly relevant literature outside the ICU. More data on critically ill patients with aHUS are needed to validate these conclusions within the ICU setting.",

keywords = "Atypical haemolytic uraemic syndrome, thrombocytopenia, thrombotic microangiopathy, organ failure, plasma exchange, eculizumab",

author = "Elie Azoulay and Paul Knoebl and Jos{\'e} Garnacho-Montero and Katerina Rusinova and Gennadii Galstian and Philippe Eggimann and Fekri Abroug and Dominique Benoit and {von Bergwelt-Baildon}, Michael and Julia Wendon and Marie Scully",

year = "2017",

month = apr,

day = "23",

doi = "10.1016/j.chest.2017.03.055",

language = "English",

journal = "Chest",

issn = "0012-3692",

publisher = "American College of Chest Physicians",

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TY - JOUR

T1 - Expert statements on the standard of care in critically ill adult patients with atypical haemolytic uraemic syndrome

AU - Azoulay, Elie

AU - Knoebl, Paul

AU - Garnacho-Montero, José

AU - Rusinova, Katerina

AU - Galstian, Gennadii

AU - Eggimann, Philippe

AU - Abroug, Fekri

AU - Benoit, Dominique

AU - von Bergwelt-Baildon, Michael

AU - Wendon, Julia

AU - Scully, Marie

PY - 2017/4/23

Y1 - 2017/4/23

N2 - Background Atypical haemolytic uraemic syndrome (aHUS) presents similarly to thrombotic thrombocytopenic purpura (TTP), and other causes or conditions with thrombotic microangiopathy (TMA) such as DIC or sepsis. Similarity in clinical presentation may hinder diagnosis and optimal treatment selection in the urgent setting in the ICU. However, there is currently no consensus on the diagnosis or treatment of aHUS for ICU specialists. This review aims to summarise available data on the diagnosis and treatment strategies of aHUS in the ICU in order to enhance the understanding of aHUS diagnosis and outcomes in patients managed in the ICU. Methods A review of the recent literature (January 2009 – March 2016) was performed to select the most relevant articles for ICU physicians. Results Based on the paucity of adult aHUS cases overall and within the ICU, no specific recommendations could be formally graded for the critical care setting. However, the expert panel recognises a core set of skills required by intensivists for diagnosing and managing patients with aHUS: recognising thrombotic microangiopathies, differentiating aHUS from related conditions, recognising involvement of other organ systems, understanding the pathophysiology of aHUS, knowing the diagnostic workup and relevant outcomes in critically ill aHUS patients, and knowing the standard of care for patients with aHUS based on available data and guidelines. Conclusions Managing critically ill patients with aHUS requires basic skills that, in the absence of sufficient data from patients treated within the ICU, can be gleaned from an increasingly relevant literature outside the ICU. More data on critically ill patients with aHUS are needed to validate these conclusions within the ICU setting.

AB - Background Atypical haemolytic uraemic syndrome (aHUS) presents similarly to thrombotic thrombocytopenic purpura (TTP), and other causes or conditions with thrombotic microangiopathy (TMA) such as DIC or sepsis. Similarity in clinical presentation may hinder diagnosis and optimal treatment selection in the urgent setting in the ICU. However, there is currently no consensus on the diagnosis or treatment of aHUS for ICU specialists. This review aims to summarise available data on the diagnosis and treatment strategies of aHUS in the ICU in order to enhance the understanding of aHUS diagnosis and outcomes in patients managed in the ICU. Methods A review of the recent literature (January 2009 – March 2016) was performed to select the most relevant articles for ICU physicians. Results Based on the paucity of adult aHUS cases overall and within the ICU, no specific recommendations could be formally graded for the critical care setting. However, the expert panel recognises a core set of skills required by intensivists for diagnosing and managing patients with aHUS: recognising thrombotic microangiopathies, differentiating aHUS from related conditions, recognising involvement of other organ systems, understanding the pathophysiology of aHUS, knowing the diagnostic workup and relevant outcomes in critically ill aHUS patients, and knowing the standard of care for patients with aHUS based on available data and guidelines. Conclusions Managing critically ill patients with aHUS requires basic skills that, in the absence of sufficient data from patients treated within the ICU, can be gleaned from an increasingly relevant literature outside the ICU. More data on critically ill patients with aHUS are needed to validate these conclusions within the ICU setting.

KW - Atypical haemolytic uraemic syndrome

KW - thrombocytopenia

KW - thrombotic microangiopathy

KW - organ failure

KW - plasma exchange

KW - eculizumab

U2 - 10.1016/j.chest.2017.03.055

DO - 10.1016/j.chest.2017.03.055

M3 - Article

SN - 0012-3692

JO - Chest

JF - Chest

ER -

Expert statements on the standard of care in critically ill adult patients with atypical haemolytic uraemic syndrome

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