Table 4.

Approach to kidney transplantation when thrombotic microangiopathy results in established renal disease

Complete Complement Evaluation before Renal Transplant Listing Is Recommended if Thrombotic Microangiopathy Results in ESRDa
Risk StratificationInclusion CriteriaManagement Strategy
HighPathogenic complement mutationsProphylaxis with eculizumab (KDIGO global panel suggest plasma exchange and liver transplantation may also be considered)
Previous early recurrence
ModerateNo complement mutation, or variant of unknown significance
Isolated CFI mutation
Detectable anti-factor H antibody
LowIsolated CD46 mutationbNo prophylaxis
Previously positive but now consistently negative anti-factor H antibody
  • Delay transplantation until at least 6 months after starting dialysis as late renal recovery with eculizumab treatment has been reported. Living related kidney donation should only be considered if a genetic or acquired cause is identified in the recipient and is not present in the intended donor. Recommendations are on the basis of the 2015 Kidney Disease: Improving Global Outcomes (KDIGO) Controversies Conference consensus recommendations (6).

  • a Complete complement evaluation should include: serum levels of C3, C4, factor H, factor I; anti-factor H antibody; CD46 FACS; and CFH, CFI, CD46, C3, and CFB genetics.

  • b Allograft cells express functional CD46.