Complement Mediated Endothelial Damage in Thrombotic Microangiopathies.

C5b-9 deposition complement blockade complement system activation endothelia cells membrane attack complex thrombotic microangiopathies

Journal

Frontiers in medicine
ISSN: 2296-858X
Titre abrégé: Front Med (Lausanne)
Pays: Switzerland
ID NLM: 101648047

Informations de publication

Date de publication:
2022
Historique:
received: 08 11 2021
accepted: 14 03 2022
entrez: 13 5 2022
pubmed: 14 5 2022
medline: 14 5 2022
Statut: epublish

Résumé

Thrombotic microangiopathies (TMA) constitute a group of different disorders that have a common underlying mechanism: the endothelial damage. These disorders may exhibit different mechanisms of endothelial injury depending on the pathological trigger. However, over the last decades, the potential role of the complement system (CS) has gained prominence in their pathogenesis. This is partly due to the great efficacy of complement-inhibitors in atypical hemolytic syndrome (aHUS), a TMA form where the primary defect is an alternative complement pathway dysregulation over endothelial cells (genetic and/or adquired). Complement involvement has also been demonstrated in other forms of TMA, such as thrombotic thrombocytopenic purpura (TTP) and in Shiga toxin-producing

Identifiants

pubmed: 35547236
doi: 10.3389/fmed.2022.811504
pmc: PMC9082680
doi:

Types de publication

Journal Article Review

Langues

eng

Pagination

811504

Informations de copyright

Copyright © 2022 Blasco, Guillén-Olmos, Diaz-Ricart and Palomo.

Déclaration de conflit d'intérêts

MB reports advisory boards and symposium speaker honoraria from Alexion Pharmaceuticals. MD-R has received research funding and speaker fees from Jazz Pharmaceuticals. The remaining authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.

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Auteurs

Miquel Blasco (M)

Department of Nephrology and Kidney Transplantation, Hospital Clínic, Centro de Referencia en Enfermedad Glomerular Compleja del Sistema Nacional de Salud (CSUR), University of Barcelona, Barcelona, Spain.
Institute of Biomedical Research August Pi i Sunyer (IDIPABS), Malalties Nefro-Urològiques i Trasplantament Renal, Barcelona, Spain.

Elena Guillén-Olmos (E)

Department of Nephrology and Kidney Transplantation, Hospital Clínic, Centro de Referencia en Enfermedad Glomerular Compleja del Sistema Nacional de Salud (CSUR), University of Barcelona, Barcelona, Spain.

Maribel Diaz-Ricart (M)

Hematopathology Unit, Department of Pathology, Hospital Clínic of Barcelona, Biomedical Diagnosis Centre (CDB), Institute of Biomedical Research August Pi i Sunyer (IDIBAPS), University of Barcelona, Barcelona, Spain.
Barcelona Endothelium Team, Barcelona, Spain.

Marta Palomo (M)

Hematopathology Unit, Department of Pathology, Hospital Clínic of Barcelona, Biomedical Diagnosis Centre (CDB), Institute of Biomedical Research August Pi i Sunyer (IDIBAPS), University of Barcelona, Barcelona, Spain.
Barcelona Endothelium Team, Barcelona, Spain.
Josep Carreras Leukaemia Research Institute, Hospital Clinic, University of Barcelona, Barcelona, Spain.

Classifications MeSH