ESPN 53rd Annual Meeting

ESPN 2021


 
Efficacy of immunoglobulin immunoadsorption in the treatment of multidrug resistant idiopathic nephrotic syndrome
STEPHANIE BONNERIC 1 THERESA KWON 1 ELODIE NATTES 1 CLAIRE DOSSIER 1 JULIEN HOGAN 1

1- PEDIATRIC NEPHROLOGY DEPARTMENT, ROBERT DEBRé HOSPITAL
 
Introduction:

Idiopathic nephrotic syndrome is mostly a chronic disease, which can lead to end-stage renal disease in case of cortico-resistance and multidrug resistance. The aim of this study was to analyze the evolution of pediatric patients with multidrug resistant nephrotic syndrome treated by immunoglobulin immunoadsorptions (IA) associated with intravenous immunoglobulins (IvIg).

Material and methods:

In this monocentric retrospective study, 23 patients with steroid and multidrug resistant nephrotic syndrome were treated with an association of IA and IvIg, followed by B cell depletion when in remission. The primary outcome was the remission of proteinuria (uPCR<0,05g/mmol).

Results:

A remission was obtained in 17 patients. 14 patients responded during the first cycle of treatment (10 daily sessions of IA), and 3 after a more prolonged treatment (up to 3 months). Anti-CD20 monoclonal antibodies were administered to maintain depletion in patients in remission. 10 patients relapsed after the first cycle of IA, and 6 developed a dependency to IA to remain in remission. Only 6 patients did not respond to IA therapy and 2 progressed towards end-stage renal disease. Out of 18 patients with long-term follow-up data (median 22,5 months), 10 patients were in remission (7 of them with no immunosuppressive therapies).

Conclusions:

IA therapy is effective in inducing remission in children with steroid and multidrug resistant nephrotic syndrome. However, more than 50% of the patients relapse despite B cell depletion and a significant number of these patients become dependent on IA to maintain remission. Newer treatment strategies are needed to maintain remission and allow IA discontinuation in children with steroid and multidrug resistant nephrotic syndrome.