Eculizumab and Atypical HUS

  • Eculizumab has shown greater efficacy than plasma therapy in the prevention and treatment of episodes of atypical haemolytic uraemic syndrome (aHUS)
  • Eculizumab may be considered as a first-line therapy in children with a first episode of aHUS as it avoids the complications associated with apheresis and central venous catheters
  • In adults, eculizumab can be used as a first-line therapy when aHUS diagnosis is undisputable, although plasma therapy should be used as a first-line therapy if uncertainty in diagnosis warrants further investigation
  • Evidence of plasma resistance or dependence should lead to a prompt switch to eculizumab; the sooner eculizumab is initiated, the better the recovery of renal function
  • Renal transplant candidates with a genetically determined risk of post-transplantation aHUS recurrence should be given a prophylactic protocol based on eculizumab if at high risk, and plasma exchange or eculizumab if at moderate risk
  • Eculizumab may be effective in curbing part of the inflammatory process involved in a subset of C3 glomerulopathies

Nature Reviews Nephrology 8, 643-657 (November 2012) | doi:10.1038/nrneph.2012.214

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