Treating aHUS


Plasma Exchange/Infusion

  • Your physician may carry out a procedure known as plasma exchange. The purpose of this is to remove plasma from your body that contains the faulty proteins and replace it with fresh plasma containing non – faulty proteins.
  • This procedure normally lasts a few hours and you will need a line to be inserted into one of your larger blood vessels for it to be carried out.

Eculizumab and Ravulizumab

  • Eculizumab (Soliris©) and Ravulizumab are medications currently licensed for use in aHUS.
  • They work by blocking the action of complement and therefore preventing it from damaging the body’s own cells.
  • Eculizumab and Ravulizumab are given as an infusion that usually lasts up to an hour for most patients, but can be longer if the infusion is for a child.
  • For eculizumab, after the first doses have been given, the regular long-term dose interval is every two weeks in patients over 10kg and every three weeks for children under 10kg
  • Historically Eculizumab was given for the remainder of your life but this may change in the future depending on the results of a clinical trial currently running to if it is safe to stop Eculizumab / Ravulizumab infusions, and what monitoring should be done when the infusions stop.


Long - term monitoring on Eculizumab / Ravulizumab

When you are receiving Eculizumab / Ravulizumab treatment, your physician willcarry out some regular tests to ensure that the drug is working and that there is noevidence ofthe disease returning.

These will include:

  • Full blood count – to look at the level of platelets and haemoglobin in your blood
  • Urea & Electrolytes–to assess your kidney function
  • LDH – this should return to normal levels once the disease is no longer active or has been suppressed by Eculizumab/Ravulizumab
  • Urine Protein-Creatinine Ratio (PCR)–measures the amount of protein beingexcreted in urine which can be an indicator of renal damage
  • Complement studies–to ensure that Eculizumab is suppressing your complement activity (currently we cannot measure this for patients onRavulizumab but we hope to develop this test in the future)


  • aHUS can cause severe damage to the kidneys and dialysis may be required to do thejob of the kidneys.
  • Like plasma exchange, this will also take a few hours and will also require a line to beinserted into your blood vessel.
  • It is not unusual to need more than one session of dialysis and in a small number ofcases this may continue for a few weeks or months.
  • In someinstances, the damage to the kidneys does not get better and dialysis may berequired in the longer term.


  • Patients with end-stage kidney disease resulting from aHUS will usually be assessedfor their suitability for a kidney transplant.
  • Unfortunately aHUS can occur in a transplanted kidney as the faulty complement proteins are still produced by the liver.
  • Eculizumab can be given to prevent the recurrence of aHUS in the transplanted kidney
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