Complement Mediated Endothelial Damage in Thrombotic Microangiopathies
Blasco Pelicano, Miquel (Institut d'Investigacions Biomèdiques August Pi i Sunyer)
Guillén-Olmos, Elena (Hospital Clínic i Provincial de Barcelona)
Diaz-Ricart, Maribel (Institut Germans Trias i Pujol. Institut de Recerca contra la Leucèmia Josep Carreras)
Palomo, Marta (Institut Germans Trias i Pujol. Institut de Recerca contra la Leucèmia Josep Carreras)
Universitat Autònoma de Barcelona

Data:	2022
Resum:	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 Escherichia coli hemolytic uremic syndrome (STEC-HUS), as well as in secondary TMAs, in which complement activation occurs in the context of other diseases. However, at present, there is scarce evidence about the efficacy of complement-targeted therapies in these entities. The relationship between complement dysregulation and endothelial damage as the main causes of TMA will be reviewed here. Moreover, the different clinical trials evaluating the use of complement-inhibitors for the treatment of patients suffering from different TMA-associated disorders are summarized, as a clear example of the entry into a new era of personalized medicine in its management.
Ajuts:	Instituto de Salud Carlos III PI19/00888 Fundació la Marató de TV3 202026-10
Drets:	Aquest document està subjecte a una llicència d'ús Creative Commons. Es permet la reproducció total o parcial, la distribució, la comunicació pública de l'obra i la creació d'obres derivades, fins i tot amb finalitats comercials, sempre i quan es reconegui l'autoria de l'obra original.
Llengua:	Anglès
Document:	Article ; recerca ; Versió publicada
Publicat a:	Frontiers in Medicine, Vol. 9 (2022) , p. 811504, ISSN 2296-858X

DOI: 10.3389/fmed.2022.811504
PMID: 35547236

12 p, 1.7 MB

El registre apareix a les col·leccions:
Articles > Articles de recerca
Articles > Articles publicats