AUTHOR=Topaloğlu Rezan , Gülhan Bora , Çelegen Kübra , İnözü Mihriban , Hayran Mutlu , Düzova Ali , Ozaltin Fatih 

TITLE=Rituximab for Children With Difficult-to-Treat Nephrotic Syndrome: Its Effects on Disease Progression and Growth

JOURNAL=Frontiers in Pediatrics

VOLUME=7

YEAR=2019

URL=https://www.frontiersin.org/journals/pediatrics/articles/10.3389/fped.2019.00313

DOI=10.3389/fped.2019.00313

ISSN=2296-2360

ABSTRACT=<p><bold>Background:</bold> Since the early 2000s rituximab (RTX) has been thought of as an alternative treatment for steroid-sensitive nephrotic syndrome (SSNS) and steroid-resistant nephrotic syndrome (SRNS).</p><p><bold>Objective:</bold> This study aimed to determine the effects of RTX treatment on disease outcome and growth in pediatric SSNS and SRNS patients.</p><p><bold>Materials and Methods:</bold> The medical records of pediatric SSNS and SRNS patients that began RTX treatment at the mean age of 10.8 ± 5.1 years between 2009 and 2017 were retrospectively reviewed. Additionally, the effect of RTX on growth was evaluated based on patient height, weight, and BMI z scores.</p><p><bold>Results:</bold> The study included 41 children, of which 21 had SSNS and 20 had SRNS. Mean age at diagnosis of NS was 5.8 ± 4.7 years. Mean duration of post-RTX treatment follow-up was 2.3 ± 1.6 years. Among the SSNS patients, 6 and 11 patients were steroid free and calcineurin inhibitor free at the last follow-up visit, respectively. The 1-year cumulative steroid and calcineurin inhibitor doses both decreased after RTX treatment, as compared to before RTX (<italic>P</italic> = 0.001 and <italic>P</italic> = 0.015, respectively). The median height <italic>z</italic>-score at the time of RTX initiation was −1.2 and the median height <italic>z</italic>-score at the last follow-up visit was −0.6 (<italic>P</italic> = 0.044). The median BMI <italic>z</italic>-score decreased from 1.6 (IQR; 0.9–3.0) at the time RTX was initiated to 1.1 IQR; [(−0.7)−2.5] at the last follow-up visit (<italic>P</italic> = 0.007). At the last follow-up visit 4 SRNS patients had complete remission and 4 had partial remission. The 1-year cumulative steroid dosage in the SRNS patients decreased significantly after RTX, as compared to before RTX (<italic>P</italic> = 0.001). The median height <italic>z</italic>-score at the time of RTX initiation was −0.8 and the median height <italic>z</italic>-score at the last follow-up visit was −0.7 (<italic>P</italic> = 0.81). The median BMI <italic>z</italic>-score decreased from 0.3 at the time RTX was initiated to −0.1 at the last follow-up visit (<italic>P</italic> = 0.11).</p><p><bold>Conclusion:</bold> RTX has a more positive effect on disease outcome and growth in SSNS patients than in those with SRNS.</p>