ESPN 53rd Annual Meeting

ESPN 2021


 
Evaluation of the combination of immunoadsorptions with eculizumab in the treatment of neurological involvement in pediatric shigatoxin-associated hemolytic uremic syndrome (STEC-HUS)
CHARLOTTE DUNETON 1 FLORIAN MANCA BARAYRE 1 OLIVIA GILLION BOYER 2 THERESA KWON 1 JULIEN HOGAN 1

1- PEDIATRIC NEPHROLOGY DEPARTMENT, ROBERT DEBRé HOSPITAL, APHP, PARIS FRANCE
2- PEDIATRIC NEPHROLOGY DEPARTMENT, NECKER ENFANTS-MALADES HOSPITAL, APHP, PARIS FRANCE
 
Introduction:

Evidence to guide the treatment of STEC-HUS with neurological involvement are lacking. The use of Eculizumab, has been reported in a few pediatric series yielding conflicting results. Antibody-mediated mechanisms have also been suggested and immunoadsorption of immunoglobulins (IgIA) was tested in a cohort of adult patients. We evaluated the combination of IgIA with eculizumab (IgIA-eculi) as a recue therapy in pediatric patients.

Material and methods:

We retrospectively analyzed neurologically impaired HUS patients with confirmed STEC infections treated with IgIA-eculi (n=19) or with eculizumab (n=24) in two pediatric nephrology centers in Paris between 2010 and 2021. The primary outcome was the neurological evaluation at 1 year dichotomized as normal vs. abnormal.

Results:

Patients’ characteristics at presentation did not significantly defer between the groups, however patients in the IgIA-eculi tended to be older 3,6 vs. 2,1 years and have more severe neurological presentation with confusion in 37% (vs. 21%), paresis in 26% (vs. 4%), seizures in 84% (vs. 75%) and status epilepticus in 42% (vs. 21%). They also tend to require more often mechanical ventilation (44% vs. 35%) and dialysis (95% vs. 71%). Each patient received a mean of 7 sessions in the IgIA-eculi group and complement blockade occurred after the first injection of eculizumab in 70% of cases. No significant association between the treatment group and the neurological evaluation at 1 year of follow-up was found (OR for abnormal evaluation in the IgIA-eculi 1,4; IC95% 0,2-8,1) after adjustment for age, sex and initial neurological presentation. The only factor independently associated with neurological status at 1 year was the presentation with status epilepticus at diagnosis (OR 12,2; IC95% 2-76).

Conclusions:

This is the first pediatric study reporting on the use of immunoadsorptions in combination with eculizumab as a rescue therapy in STEC-HUS with neurological involvement. We found no benefit to the addition of immunoadsorptions to eculizumab therapy on neurological outcome after 1 year of follow-up.