Eculizumab is licenced for long term treatment of complement-mediated aHUS.
1. The decision to stop Eculizumab should be made on an individual patient basis considering factors that will influence the likelihood and consequences of a relapse.
a. The genetic / autoantibody status of the patient
b. The severity of the initial presentation
c. A previous history of relapsing TMA causing organ failure, for example transplant loss
2. Eculizumab should be continued for 6 months before withdrawal is considered. There is no evidence to support a recommendation of 6 months treatment. However it is known that there is a high risk of recurrence in patients treated with a short course of Eculizumab, suggesting that the trigger that precipitated the treated TMA may persist. In addition, after transplantation most recurrences of aHUS (>70%) occur within the first 6 months.
3. Patients should be monitored closely after withdrawal for evidence of relapse. This should be most intensive immediately after withdrawal as it is not known whether disease is controlled or in remission. However, there is no evidence to inform the frequency of testing, therefore the following is a suggested monitoring regime that is based on the monitoring protocol used in the SETS-aHUS study. In some patients, particularly after transplant, there is limited systemic evidence of a recurrent renal thrombotic microangiopathy (ie normal platelet, LDH, blood film and haptoglobins) and the only indication is a deterioration in renal function.
| Time since last Eculizumab dose |
Time since last Ravulizumab dose |
Suggested monitoring |
| Week 0 |
Week 0 |
Baseline – FBC, U&E, LDH and urine P:Cr |
| Week 2 to 6 |
Weeks 8 to 12 |
Weekly – FBC, U&E, LDH and urine P:Cr |
| Week 6 to 26 |
Weeks 12 to 32 |
Fortnightly – FBC, U&E, LDH and urine P:Cr |
| Week 26 to 104 |
Weeks 32 to 110 |
Monthly – FBC, U&E, LDH and urine P:Cr |
Monitoring of patients following withdrawal of eculizumab
- Home monitoring with thrice weekly urinalysis is recommended. The patient is advised to return to hospital for evaluation if microscopic haematuria develops.
- Patients should be informed about potential symptoms of relapse and advised to seek medical advice if these occur. They should also have the patient alert card (available from the NRCTC) to present to medical staff.
- Regular blood test monitoring as suggested in the table.
- Antibiotic prophylaxis should be continued for 4 weeks after the last dose of Eculizumab and 4 months after the last dose of Ravulizumab
- Eculizumab should be available to treat relapse
