Kidney transplantation in atypical haemolytic uraemic syndrome (aHUS): A cheap way out?

摘要

The C5 monoclonal antibody eculizumab is one of the newer biologicals that are the subject of many exciting discussions, not only because of their extraordinary high price, but also because of their high therapeutic potential in diseases in which uncontrolled activation of the complement system plays a central role. As a consequence, the list of difficult to treat inflammatory diseases eligible for treatment with this monoclonal antibody is steadily increasing. Examples are (catastrophic) anti-phospholipid syndrome, the HELLP syndrome, membranoproliferative glomerulonephritis type II, humoral transplant rejection and haemolytic uraemic syndrome (HUS). HUS is a rare disease, characterised by the occurrence of haemolytic anaemia, thrombocytopenia and acute renal failure, rapidly progressing to end-stage renal disease (ESRD). Histological examination of the kidney reveals, amongst other things, activation and damage of glomerular endothelial cells and consequent thrombotic micro-angiopathy (TMA). Glomerular tufts are destroyed and kidney failure ensues.