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Update on the treatment of steroid-sensitive nephrotic syndrome

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Abstract

Steroid-sensitive nephrotic syndrome (SSNS) is a rare condition that develops primarily in preadolescent children after the age of 1 year. Since the 1950s, oral corticosteroids have been the mainstay of treatment of all children presenting with nephrotic syndrome, with most patients responding within 4 weeks to an oral course of prednisone (PDN). However, corticosteroids have important side effects and 60–80 % of patients relapse, developing frequently relapsing or steroid-dependent forms. For these reasons, many patients require second-line steroid-sparing immunosuppressive medications that have considerably improved relapse-free survival, while avoiding many PDN-related toxicities. Since most patients will eventually heal from their disease with a normal kidney function, the morbidity of SSNS is primarily related to side effects of drugs that are used to maintain prolonged remission. Therefore, treatment is essentially based on balancing the use of different drugs to achieve permanent remission with the lowest cumulative number of side effects. Treatment choice is based on the severity of SSNS, on patient age, and on drug tolerability. This review provides an update of currently available therapeutic strategies for SSNS.

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Abbreviations

CNIs:

Calcineurin inhibitors

CYP:

Cyclophosphamide

CsA:

Cyclosporine A

FRNS:

Frequently relapsing nephrotic syndrome

MSCs:

Mesenchymal stem cells

MMF:

Mycophenolate mofetil

PDN:

Prednisone

RCT:

Randomized controlled trial

RTX:

Rituximab

SDNS:

Steroid-dependent nephrotic syndrome

SRNS:

Steroid-resistant nephrotic syndrome

SSNS:

Steroid-sensitive nephrotic syndrome

Tac:

Tacrolimus

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  1. Department of Pediatric Subspecialties, Division of Nephrology, Bambino Gesù Children’s Hospital – IRCCS, Piazza S. Onofrio 4, 00165, Rome, Italy

    Federica Zotta, Marina Vivarelli & Francesco Emma

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  1. Federica Zotta
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  2. Marina Vivarelli
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Zotta, F., Vivarelli, M. & Emma, F. Update on the treatment of steroid-sensitive nephrotic syndrome. Pediatr Nephrol 37, 303–314 (2022). https://doi.org/10.1007/s00467-021-04983-3

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