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Steroid Resistant Nephrotic Syndrome

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Pediatric Kidney Disease

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Abstract

Nephrotic syndrome is among the most common forms of kidney disease seen in children. Steroid resistant nephrotic syndrome (SRNS), which accounts for up to 20% of all cases of childhood NS, is the most common glomerular cause of end stage kidney disease (ESKD) in children. The pathogenesis of FSGS has not been completely delineated; however, there is growing evidence to suggest that it is a primary defect of the podocyte. Up to 30% of cases are caused by monogenic hereditary podocyte disorders. A role for circulating factors has also been implicated in disease pathogenesis. Histopathological changes vary; focal segmental glomerulosclerosis (FSGS) and minimal change disease (MCD) are the most common findings. The treatment of SRNS is challenging, as only about 30–60% of all cases will respond completely or partially to currently available therapies, yet response to therapy is the most important determinant of future progression to end-stage kidney disease (ESKD). The poorest long-term prognosis is associated with genetic disease forms (80% ESKD 15 years after disease onset), followed by patients without a genetic cause who show resistance to intensified immunosuppressive therapeutic attempts (multidrug resistant nephrotic syndrome, 60% 15-year ESKD risk), whereas patients achieving full disease remission upon intensified immunosuppression rarely develop ESKD (<10% at 15 years). The risk of SRNS recurrence in kidney transplant is estimated at 30%, with a lower risk (0–5%) in patients with monogenic SRNS and close to 80% in patients with secondary steroid resistance and progression to ESKD. Further studies of familial and non-familial forms of SRNS are needed in order to elucidate disease mechanisms, develop a robust pathogenesis based classification, develop personalized approach to therapy, and identify specific non-toxic therapeutic targets.

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Authors and Affiliations

  1. Division of Nephrology, Department of Pediatrics, Duke University, Durham, NC, USA

    Rasheed Gbadegesin

  2. Division of nephrology, Department of Pediatrics and Medicine, University of North Carolina, Chapel Hill, NC, USA

    Keisha Gibson

  3. Division of Nephrology, Children’s Hospital at Montefiore, Albert Einstein College of Medicine, Bronx, NY, USA

    Kimberly Reidy

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  1. Rasheed Gbadegesin
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  3. Kimberly Reidy
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Correspondence to Rasheed Gbadegesin .

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  1. Division of Pediatric Nephrology, Heidelberg University, Heidelberg, Germany

    Franz Schaefer

  2. Emory School of Medicine, Emory University, Atlanta, GA, USA

    Larry A. Greenbaum

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Gbadegesin, R., Gibson, K., Reidy, K. (2023). Steroid Resistant Nephrotic Syndrome. In: Schaefer, F., Greenbaum, L.A. (eds) Pediatric Kidney Disease. Springer, Cham. https://doi.org/10.1007/978-3-031-11665-0_14

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  • DOI: https://doi.org/10.1007/978-3-031-11665-0_14

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