Treatment of atypical hemolytic uremic syndrome and thrombotic microangiopathies: a focus on eculizumab.

Détails

ID Serval
serval:BIB_1C5156F18BCC
Type
Article: article d'un périodique ou d'un magazine.
Collection
Publications
Titre
Treatment of atypical hemolytic uremic syndrome and thrombotic microangiopathies: a focus on eculizumab.
Périodique
American Journal of Kidney Diseases
Auteur(s)
Schmidtko J., Peine S., El-Housseini Y., Pascual M., Meier P.
ISSN
1523-6838 (Electronic)
ISSN-L
0272-6386
Statut éditorial
Publié
Date de publication
2013
Peer-reviewed
Oui
Volume
61
Numéro
2
Pages
289-299
Langue
anglais
Notes
Publication types: Journal Article
Résumé
Uncontrolled complement activation is central to the occurrence of atypical hemolytic uremic syndrome (aHUS) and can result in thrombotic microangiopathies (TMAs).These terms encompass a group of heterogenic inherited or acquired diseases that recent research suggests may be triggered by the complement cascade. Pathogenetic triggers of complement activation include immunologic disorders, genetics, infections, systemic diseases, pregnancy, drug administration, metabolic diseases, transplantation, or triggers of mixed cause. Hallmarks of aHUS and other TMAs include increased vascular endothelium thromboresistance, leukocyte adhesion to damaged endothelium, complement consumption, coagulation abnormalities, and vascular shear stress, whereas common end points of these mechanisms include hemolytic anemia, thrombocytopenia with microvascular infarction, and predisposition for decreased kidney function and other organ involvement. The central role of the complement cascade as a disease trigger suggests a possible therapeutic target. Eculizumab, a first-in-class humanized monoclonal anti-C5 antibody that has been successful in the treatment of paroxysmal nocturnal hemoglobinuria, a disorder of complement-induced hemolytic anemia, received approval for the treatment of aHUS in the United States and Europe in late 2011. We review the treatment of aHUS and other TMAs, focusing on the role of eculizumab, including its pharmacology, mechanism of action, and approved dosing recommendations and health economic considerations. Finally, the potential for future indications for eculizumab use in other complement-driven diseases is discussed.
Pubmed
Web of science
Création de la notice
05/02/2013 22:25
Dernière modification de la notice
20/08/2019 12:52
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