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. 2023 Sep;38(9):3035-3042.
doi: 10.1007/s00467-023-05913-1. Epub 2023 Apr 4.

Rituximab-associated hypogammaglobulinemia in children with idiopathic nephrotic syndrome: results of an ESPN survey

Aleksandra Zurowska  1   2 , Magdalena Drozynska-Duklas  3 , Rezan Topaloglu  4 , Antonia Bouts  5 , Olivia Boyer  6   7 , Mohan Shenoy  8 , Marina Vivarelli  9 ; ESPN Glomerulonephritis Working Group
Collaborators, Affiliations

Rituximab-associated hypogammaglobulinemia in children with idiopathic nephrotic syndrome: results of an ESPN survey

Aleksandra Zurowska et al. Pediatr Nephrol. 2023 Sep.

Erratum in

Abstract

Background: There is paucity of information on rituximab-associated hypogammaglobulinemia (HGG) and its potential infectious consequences in children treated for idiopathic nephrotic syndrome (INS).

Methods: A survey was distributed by the European Society Pediatric Nephrology to its members. It addressed the screening and management practices of pediatric nephrology units for recognizing and treating RTX-associated HGG and its morbidity and mortality. Eighty-four centers which had treated an overall 1328 INS children with RTX responded.

Results: The majority of centers administered several courses of RTX and continued concomitant immunosuppressive therapy. Sixty-five percent of centers routinely screened children for HGG prior to RTX infusion, 59% during, and 52% following RTX treatment. Forty-seven percent had observed HGG prior to RTX administration, 61% during and 47% >9 months following treatment in 121, 210, and 128 subjects respectively. Thirty-three severe infections were reported among the cohort of 1328 RTX-treated subjects, of whom 3 children died. HGG had been recognized in 30/33 (80%) of them.

Conclusions: HGG in steroid-dependent/frequently relapsing nephrotic syndrome (SDNS/FRNS) children is probably multifactorial and can be observed prior to RTX administration in children with SDNS/FRNS. Persistent HGG lasting >9 months from RTX infusion is not uncommon and may increase the risk of severe infections in this cohort. We advocate for the obligatory screening for HGG in children with SDNS/FRNS prior to, during, and following RTX treatment. Further research is necessary to identify risk factors for developing both HGG and severe infections before recommendations are made for its optimal management. A higher resolution version of the Graphical abstract is available as Supplementary information.

Keywords: Children; Hypogammaglobulinemia; Nephrotic syndrome; Rituximab.

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Conflict of interest statement

1. Marina Vivarelli declared potential conflict of interests:

- Grants – Alexion

- Sponsored clinical trials – Chinook, Novartis, Bayer, Rche, Apellis, Travere, Alexion, Chemocentrix

- Lectures – Travere, Alexion, WebMD

- Consultancy fees – Novartis, Biocryst, Roche

- Advisory board – Novartis

2. Aleksandra Zurowska declared potential conflict of interests: lectures for Swixx BioPharma

3. Mohan Shenoy declared potential conflict of interests: advisory board – Novartis

4. Olivia Boyer declared potential conflict of interests: consultancy fees – Rocher

5. Magdalena Drozynska-Duklas – no conflict of interests to disclose

Figures

None
A higher resolution version of the Graphical abstract is available as Supplementary information
Fig. 1
Fig. 1
Screening policies for hypogammaglobulinemia in SDNS/FRNS children treated with rituximab in 84 European centers (pre, during, and post treatment)
Fig. 2
Fig. 2
Number of centers reporting hypogammaglobulinemia in RTX-treated children with SDNS/FRNS. Survey results from 84 European pediatric nephrology centers
Fig. 3
Fig. 3
Number of centers reporting severe infections (morbidity) and death from severe infections (mortality) in children with SDNS/FRNS treated with RTX. Severe infections reported by 20/84 centers in 33/over 1300 children treated with RTX; deaths reported by 3/82 centers in 3/ over 1300 children treated with RTX
See this image and copyright information in PMC

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