- The risk of recurrence should be assessed based on genetic screening, presence of autoantibodies and previous transplant history.
- Patients with high or medium risk of recurrence should be offered prophylactic Eculizumab treatment.
- Patients at low risk should be warned of the risk of recurrence and monitored closely.
- Recommendation: Adult patients should receive eculizumab as follows:
Day 0 – 900mg to be completed prior to the start of surgery
Day 1* – 900mg
Day 7 – 900mg
Day 14 – 900mg
Day 21 – 900mg
Day 28 – 1200mg
Day 42 – 1200mg
*A further dose of eculizumab should be considered if there is significant blood loss requiring administration of FFP or equivalent. The dose and dosing schedule should be adjusted for body weight in children as per paediatric dosing schedule.
- Post-Transplant complement blockade monitoring
We would recommend complement blockade is measured in patients undergoing kidney transplantation with concomitant eculizumab as below:
Day 7 – Pre-dose (trough) eculizumab
Day 42 – Immediately prior (trough) to 2nd 1200mg eculizumab dose being given
Annually thereafter [or as clinically indicated where there is suspicion that the complement system may not be adequately blocked]
The blood form for requesting the collection and processing of complement blockade bloods can be found here or by contacting the aHUS Specialist Nurses on firstname.lastname@example.org
Treatment: Additional Considerations
- A further dose of Eculizumab should be considered if there is significant blood loss requiring administration of FFP or equivalent.
- Treatment with Eculizumab should continue unless withdrawn with close monitoring as part of a clinical study.
- Patients should receive a Tacrolimus based immunosuppressive regime. The use of anti-IL2 receptor blocking antibody, anti-proliferative agent and steroids should be as per local protocols.
- Rapamycin should be avoided post-transplant in patients at risk of recurrent aHUS.
- Pre-transplant plasma exchange is not required in patients with aHUS prior to transplantation when Eculizumab is being used.
- The guidelines for the prevention of meningococcal disease should be adhered to in all patients with aHUS who are being assessed for kidney transplantation.
- There is a relative contraindication with respect to living related donation but this can be considered in certain circumstances.
- The possibility of liver transplantation should be discussed with all patients considering transplantation but is not the recommended option for most patients.