Complement activation plays a major role in several renal pathophysiological conditions. The three pathways of complement lead to C3 activation, followed by the formation of the anaphylatoxin C5a and the terminal membrane attack complex (MAC) in blood and at complement activating surfaces, lead to a cascade of events responsible for @entity32 and for the induction of cell lysis. In case of ongoing uncontrolled complement activation, endothelial cells activation takes place, leading to events in which at the end @entity9818 can occur. Atypical @entity1381 (aHUS) is a @entity9818 characterized by excessive complement activation on the surface of the microcirculation. It is a severe, @entity395 which leads to @entity454 ( @entity454 ) and/or to @entity204 in more than 50% of @entity1 without treatment. In the first decade of the second millennium, huge progress in understanding the aetiology of this disease was made, which paved the way to better treatment. First, protocols of plasma therapy for treatment, prevention of relapses and for renal transplantation in those @entity1 were set up. Secondly, in some severe cases, combined kidney and liver transplantation was reported. Finally, at the end of this decade, the era of complement inhibitors, as anti-C5 monoclonal antibody (anti-C5 mAb) began. The past five years have seen growing evidence of the favourable effect of anti-C5 mAb in aHUS which has made this drug the first-line treatment in this disease. The possible complication of @entity7135 needs appropriate vaccination before its use. Unfortunately, the worldwide use of anti-C5 mAb is limited by its very high price. In the future, extension of indications for anti-C5 mAb use, the elaboration of generics and of mAbs directed towards other complement factors of the terminal pathway of the complement system might succeed in reducing the cost of this new valuable therapeutic approach and render it available worldwide for @entity1 from all social classes.

Advances and challenges in the management of complement-mediated XXXX .

@entity9818 :: ('MESH:D057049', 'Disease') :: ['thrombotic microangiopathy'] @entity32 :: ('MESH:D007249', 'Disease') :: ['inflammation'] @entity1 :: ('9606', 'Species') :: ['patients'] @entity7135 :: ('MESH:D008589', 'Disease') :: ['meningococcal infection'] @entity1381 :: ('MESH:D006463', 'Disease') :: ['haemolytic uremic syndrome'] @entity395 :: ('MESH:D035583', 'Disease') :: ['rare disease'] @entity204 :: ('MESH:D003643', 'Disease') :: ['death'] @entity454 :: ('MESH:D007676', 'Disease') :: ['ESRF', 'end-stage renal failure']
@entity9818 :: (MESH:D057049,Disease) :: ['thrombotic microangiopathies']
