Complement C5-inhibiting therapy for the thrombotic microangiopathies: accumulating evidence, but not a panacea.

ABSTRACT: Thrombotic microangiopathy (TMA), characterized by organ injury occurring consequent to severe endothelial damage, can manifest in a diverse range of diseases. In complement-mediated atypical haemolytic uraemic syndrome (aHUS) a primary defect in complement, such as a mutation or autoantibody leading to over activation of the alternative pathway, predisposes to the development of disease, usually following exposure to an environmental trigger. The elucidation of the pathogenesis of aHUS resulted in the successful introduction of the complement inhibitor eculizumab into clinical practice. In other TMAs, although complement activation may be seen, its role in the pathogenesis remains to be confirmed by an interventional trial. Although many case reports in TMAs other than complement-mediated

SUBMITTER: Brocklebank V 

PROVIDER: S-EPMC5622895 | biostudies-literature | 2017 Oct

REPOSITORIES: biostudies-literature