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Anti-nephrin autoantibodies in steroid-resistant nephrotic syndrome may inform treatment strategy

Introduction - Autoantibodies against the podocyte protein nephrin were recently identified in a pediatric cohort primarily comprising steroid-sensitive (SSNS) and steroid-dependent (SDNS) nephrotic syndrome (NS). However, their prevalence across all NS subtypes, particularly in steroid-resistant ne...

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Main Authors: Hengel, Felicitas Eugenia (Author) , Dehde, Silke (Author) , Yilmaz, Alev (Author) , Bayazit, Aysun K. (Author) , Ozaltin, Fatih (Author) , Paripovic, Dusan (Author) , Emma, Francesco (Author) , Ronco, Pierre (Author) , Vivarelli, Marina (Author) , Hogan, Julien (Author) , Schaefer, Franz (Author) , Tomas, Nicola M. (Author) , Huber, Tobias B. (Author)
Format: Article (Journal)
Language:English
Published: June 2025
In: Kidney international
Year: 2025, Volume: 107, Issue: 6, Pages: 1099-1103
ISSN:1523-1755
DOI:10.1016/j.kint.2025.01.019
Online Access:Verlag, kostenfrei, Volltext: https://doi.org/10.1016/j.kint.2025.01.019
Verlag, kostenfrei, Volltext: https://www.sciencedirect.com/science/article/pii/S0085253825000821
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Author Notes:Felicitas E. Hengel, Silke Dehde, Alev Yilmaz, Aysun K. Bayazit, Fatih Ozaltin, Dusan Paripovic, Francesco Emma, Pierre Ronco, Marina Vivarelli, Julien Hogan, Franz Schaefer, Nicola M. Tomas and Tobias B. Huber, on behalf of the PodoNet Consortium and the International Society of Glomerular Disease
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Summary:Introduction - Autoantibodies against the podocyte protein nephrin were recently identified in a pediatric cohort primarily comprising steroid-sensitive (SSNS) and steroid-dependent (SDNS) nephrotic syndrome (NS). However, their prevalence across all NS subtypes, particularly in steroid-resistant nephrotic syndrome (SRNS), and their relation to therapy response need to be determined to advance pathophysiological understanding and refine treatment strategies. - Methods - A multicenter cohort study measuring anti-nephrin autoantibodies in samples from children with SSNS, SDNS, nongenetic and genetic SRNS was conducted. - Results - Sixty-nine of 101 (68%) patients with SSNS, 19 of 67 (28%) patients with SDNS, 14 of 103 patients (14%) with non-genetic SRNS, and 1 of 62 patients (2%) with genetic SRNS were positive for anti-nephrin autoantibodies. The prevalence of anti-nephrin autoantibodies increased with presence of active disease in cases of SSNS and SDNS. Within the group of non-genetic SRNS patients with active disease, anti-nephrin positivity was found in 13 of 74 (18%) patients responding to intensified immunosuppression compared to none of 17 patients with multidrug-resistant SRNS. - Conclusions - The prevalence of anti-nephrin antibodies is substantially higher in children with steroid responsive NS than in those with SRNS, suggesting that anti-nephrin antibodies primarily drive SSNS/SDNS. In contrast, NS due to podocyte gene mutations is primarily genotype-caused. Anti-nephrin autoantibodies may serve as a positive prognostic marker in pediatric NS, indicating a favorable response to immunosuppressive therapy.
Item Description:Online verfügbar: 6. Februar 2025, Artikelversion: 20. Mai 2025
Gesehen am 18.09.2025
Physical Description:Online Resource
ISSN:1523-1755
DOI:10.1016/j.kint.2025.01.019

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