Pediatric idiopathic steroid-sensitive nephrotic syndrome: diagnosis and therapy -short version of the updated German best practice guideline (S2e) - AWMF register no. 166-001, 6/2020

Rasmus Ehren, Marcus R Benz, Paul T Brinkkötter, Jörg Dötsch, Wolfgang R Eberl, Jutta Gellermann, Peter F Hoyer, Isabelle Jordans, Clemens Kamrath, Markus J Kemper, Kay Latta, Dominik Müller, Jun Oh, Burkhard Tönshoff, Stefanie Weber, Lutz T Weber, German Society for Pediatric Nephrology, Rasmus Ehren, Marcus R Benz, Paul T Brinkkötter, Jörg Dötsch, Wolfgang R Eberl, Jutta Gellermann, Peter F Hoyer, Isabelle Jordans, Clemens Kamrath, Markus J Kemper, Kay Latta, Dominik Müller, Jun Oh, Burkhard Tönshoff, Stefanie Weber, Lutz T Weber, German Society for Pediatric Nephrology

Abstract

Idiopathic nephrotic syndrome is the most frequent glomerular disease in children in most parts of the world. Children with steroid-sensitive nephrotic syndrome (SSNS) generally have a good prognosis regarding the maintenance of normal kidney function even in the case of frequent relapses. The course of SSNS is often complicated by a high rate of relapses and the associated side effects of repeated glucocorticoid (steroid) therapy. The following recommendations for the treatment of SSNS are based on the comprehensive consideration of published evidence by a working group of the German Society for Pediatric Nephrology (GPN) based on the systematic Cochrane reviews on SSNS and the guidelines of the KDIGO working group (Kidney Disease - Improving Global Outcomes).

Keywords: Frequently relapsing nephrotic syndrome; Guideline; Steroid-dependent nephrotic syndrome; Steroid-sensitive nephrotic syndrome; Treatment.

Conflict of interest statement

RE, MRB, BT, and LTW receive financial support from the BMBF (German Ministry of Education and Research) for the implementation of the INTENT study (EudraCT No.: 2014-001991-76). RE, PTB, and LTW receive financial support from the DFG (German Research Foundation) for their work within the Clinical Research Unit 329 (“Disease pathways in podocyte injury — from molecular mechanisms to individualized treatment options”) at the University Hospital of Cologne.

JD is a consultant and expert for Nestlé Germany AG, Danone SA and Novartis Pharma GmbH (Advisory Board). WRE is a consultant and expert at Bayer AG, Biotest AG and Sobi AG and has received honoraria for lectures from CSL Behring GmbH, Bayer AG and Novo Nordisk Pharma GmbH. CK is on the scientific advisory board of BioMarin Pharmaceutical Inc., Aegerion Pharmaceuticals GmbH, and Sobi AG and has received honoraria for lectures and training from Aegerion Pharmaceuticals GmbH and Diurnal Ltd. Jun Oh is a consultant and reviewer for Alnylam Germany GmbH and Chiesi GmbH (Advisory Board) and has received lecture fees from Recordati Pharma GmbH. BT is a consultant and expert at Bristol-Myers Squibb GmbH. He is on the scientific advisory board of “transplant campus” and conducts clinical studies with the companies Novartis Pharma GmbH and Astellas Pharma GmbH. LTW is a consultant and expert for Alexion-Pharma GmbH, Alnylam Germany GmbH, Greenovation Biotech GmbH and Chiesi GmbH. JG, PFH, IJ, MJK, KL, DM, and SW declare no conflict of interests.

© 2021. The Author(s).

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