Childhood idiopathic nephrotic syndrome: recent advancements shaping future guidelines

Abstract

Childhood idiopathic nephrotic syndrome is an important pediatric kidney disease associated with significant morbidities and even mortality. Several guidelines have been developed to standardize the terminology and patient care among the pediatric nephrology community. Since the publication of these guidelines, there have been major breakthroughs in the disease management and the understanding of underlying pathogenesis through multi-omics investigations, including the identification of anti-nephrin autoantibodies, genetic susceptibility loci, and the pathogenic role of B cell subsets. In this educational review, we summarize the recent major advancements in idiopathic nephrotic syndrome and attempt to provide potential therapeutic approaches in both steroid-sensitive and steroid-resistant nephrotic syndrome that may shape future guideline development.

Keywords: FSGS; Minimal change disease; Monogenic podocytopathy; Nephrotic syndrome; Pediatric nephrology; Rituximab.

A higher resolution version of the Graphical abstract is available as Supplementary information

Fig. 1

Current IPNA recommendations and potential therapeutic treatment approaches for steroid-sensitive nephrotic syndrome (SSNS). FR, frequently relapsing; MMF, mycophenolate mofetil; SD, steroid-dependent

Fig. 2

Current IPNA recommendations and potential therapeutic treatment approaches for steroid-resistant nephrotic syndrome (SRNS). CNIs, calcineurin inhibitors; IVMP, intravenous pulse methylprednisolone; RAASi, renin–angiotensin–aldosterone-system inhibitors