ERKNET: aHUS and transplants

ERKNET’s website hosts a programme of webinars ( live talks over the  internet) about Rare Renal Diseases . The talk today was given by Professor Marius  Miglinas , Head of Nephrology Centre at Vilnius University Hospital. Vilnius is the Capital City of Latvia.

His talk is entitled “ Spectrum of Complement- Mediated Thrombotic  Microangiopathies after Kidney transplantation”
“Complement – mediated TMA” being a new name for aHUS.Kidney transplant with or without Complement inhibitors being the top research topic chosen by the aHUS alliance. aHUS dialysis patients are a discriminated against cohort in many counties, even those with access to eculizumab,   and are often being left behind.
The alliance registered to listen to what Prof. Miglinas had to say.
The following observations were made from the talk.

  • Primary TMA’s include aHUS with complement mutations but Secondary TMAs do not rule out Primary involvement ( about 33%)
  • Incidence of TMA in Transplant 5.6 per 1000 , 50% mortality
  • Post Transplant TMA incidents could be  recurrence or “de novo” ( although diagnosis in native kidney loss could have been missed).
  • Conditions of transplant are going to result in a endolaethial damage.
  • It could be one of the two hits needed for aHUS to onset.
  • Risks vary by genetic mutation but not all genetic testing is standardised.
  • No bio-markers for specific diagnosis
  • No consensus on how long eculizumab is needed post transplant – 6 months too soon , stronger case for 14 months before termination of treatment in some cases.
  • There are a range of serology tests for complement activity and eculizumab trough.
  • Prior to eculizumab alograft loss was as much as 60 to 80%
  • Transplant for DGKE patients seems to be successful without eculizumab
  • Kidney transplant for C3G not so ,and disease recurrence is high.
  • Living kidney donation for aHUS patients is no longer ruled out but genetic testing and counselling is very essential.
  • The debate about whether eculizumab should be given prophylacticly or as a “rescue” therapy continues.
  • The Bergamo Group reported no relapse in kidney transplants at their hospital.

A very fast paced ,demanding and clear talk about this complex subject.
The risks need understanding and may not be as great as thought. Different eculizumab treatment strategies to fit the risks could make use of eculizumab in a lot more cost effective way  and for the alliance this could influence health funding decisions accordingly.
ERKNET record these webinars and then provide access to both  an audio file of ,and the set of slides used in, the talk. A link to this webinar can be found at September 19 using by clicking here.
The next webinar relating to HUS is on 13th Novemeber and will feature Prof. Guiseppe Remuzzi presenting “Unraveling the pathophysiology of HUS in the light of recent discoveries on complement activation”.

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