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An updated classification of thrombotic microangiopathies and treatment of complement gene variant-mediated thrombotic microangiopathy

Conditions presenting with signs of thrombotic microangiopathies (TMAs) comprise a wide spectrum of different diseases. While pathological hallmarks are thrombosis of arterioles and capillaries, clinical signs are mechanical haemolysis, thrombocytopenia and acute renal injury or neurological manifes...

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Autores principales: Aigner, Christof, Schmidt, Alice, Gaggl, Martina, Sunder-Plassmann, Gere
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Oxford University Press 2019
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6543965/
https://www.ncbi.nlm.nih.gov/pubmed/31198225
http://dx.doi.org/10.1093/ckj/sfz040
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author Aigner, Christof
Schmidt, Alice
Gaggl, Martina
Sunder-Plassmann, Gere
author_facet Aigner, Christof
Schmidt, Alice
Gaggl, Martina
Sunder-Plassmann, Gere
author_sort Aigner, Christof
collection PubMed
description Conditions presenting with signs of thrombotic microangiopathies (TMAs) comprise a wide spectrum of different diseases. While pathological hallmarks are thrombosis of arterioles and capillaries, clinical signs are mechanical haemolysis, thrombocytopenia and acute renal injury or neurological manifestations. The current classification of various syndromes of TMA is heterogeneous and often does not take the underlying pathophysiology into consideration. Therefore we propose a simplified classification based on the aetiology of different syndromes leading to TMA. We propose to categorize different TMA syndromes in hereditary and acquired forms and classify them based on the genetic background or underlying conditions. Of course, this classification is not always distinctly applicable in each case and from time to time reassessment of the established diagnosis is strongly recommended. The recommended treatment of TMA in the past was plasma exchange (PE). However, recently, the terminal complement inhibitor eculizumab became commercially available and has shown promising results in different open-label studies and case series. In our centre, first-line therapy is PE; however, patients are instantly switched to complement inhibitory therapy in case of treatment failure or intolerance.
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spelling pubmed-65439652019-06-13 An updated classification of thrombotic microangiopathies and treatment of complement gene variant-mediated thrombotic microangiopathy Aigner, Christof Schmidt, Alice Gaggl, Martina Sunder-Plassmann, Gere Clin Kidney J Thrombotic Microangiopathies Conditions presenting with signs of thrombotic microangiopathies (TMAs) comprise a wide spectrum of different diseases. While pathological hallmarks are thrombosis of arterioles and capillaries, clinical signs are mechanical haemolysis, thrombocytopenia and acute renal injury or neurological manifestations. The current classification of various syndromes of TMA is heterogeneous and often does not take the underlying pathophysiology into consideration. Therefore we propose a simplified classification based on the aetiology of different syndromes leading to TMA. We propose to categorize different TMA syndromes in hereditary and acquired forms and classify them based on the genetic background or underlying conditions. Of course, this classification is not always distinctly applicable in each case and from time to time reassessment of the established diagnosis is strongly recommended. The recommended treatment of TMA in the past was plasma exchange (PE). However, recently, the terminal complement inhibitor eculizumab became commercially available and has shown promising results in different open-label studies and case series. In our centre, first-line therapy is PE; however, patients are instantly switched to complement inhibitory therapy in case of treatment failure or intolerance. Oxford University Press 2019-04-21 /pmc/articles/PMC6543965/ /pubmed/31198225 http://dx.doi.org/10.1093/ckj/sfz040 Text en © The Author(s) 2019. Published by Oxford University Press on behalf of ERA-EDTA. http://creativecommons.org/licenses/by-nc/4.0/ This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/4.0/), which permits non-commercial re-use, distribution, and reproduction in any medium, provided the original work is properly cited. For commercial re-use, please contact journals.permissions@oup.com
spellingShingle Thrombotic Microangiopathies
Aigner, Christof
Schmidt, Alice
Gaggl, Martina
Sunder-Plassmann, Gere
An updated classification of thrombotic microangiopathies and treatment of complement gene variant-mediated thrombotic microangiopathy
title An updated classification of thrombotic microangiopathies and treatment of complement gene variant-mediated thrombotic microangiopathy
title_full An updated classification of thrombotic microangiopathies and treatment of complement gene variant-mediated thrombotic microangiopathy
title_fullStr An updated classification of thrombotic microangiopathies and treatment of complement gene variant-mediated thrombotic microangiopathy
title_full_unstemmed An updated classification of thrombotic microangiopathies and treatment of complement gene variant-mediated thrombotic microangiopathy
title_short An updated classification of thrombotic microangiopathies and treatment of complement gene variant-mediated thrombotic microangiopathy
title_sort updated classification of thrombotic microangiopathies and treatment of complement gene variant-mediated thrombotic microangiopathy
topic Thrombotic Microangiopathies
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6543965/
https://www.ncbi.nlm.nih.gov/pubmed/31198225
http://dx.doi.org/10.1093/ckj/sfz040
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