Update on the treatment of steroid-sensitive nephrotic syndrome
- 5 March 2021
- journal article
- review article
- Published by Springer Science and Business Media LLC in Pediatric Nephrology
- Vol. 37 (2), 303-314
- https://doi.org/10.1007/s00467-021-04983-3
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.Keywords
This publication has 99 references indexed in Scilit:
- Rituximab is a safe and effective long-term treatment for children with steroid and calcineurin inhibitor–dependent idiopathic nephrotic syndromeKidney International, 2013
- Treatment of tacrolimus or cyclosporine A in children with idiopathic nephrotic syndromePediatric Nephrology, 2012
- Short-Term Effects of Rituximab in Children with Steroid- and Calcineurin-Dependent Nephrotic SyndromeClinical Journal of the American Society of Nephrology, 2011
- New therapies in steroid-sensitive and steroid-resistant idiopathic nephrotic syndromePediatric Nephrology, 2011
- Efficacy and Safety of Treatment with Rituximab for Difficult Steroid-Resistant and -Dependent Nephrotic SyndromeClinical Journal of the American Society of Nephrology, 2010
- Early age at debut is a predictor of steroid-dependent and frequent relapsing nephrotic syndromePediatric Nephrology, 2010
- Fatal pulmonary fibrosis after rituximab administrationPediatric Nephrology, 2009
- Single dose of rituximab for refractory steroid-dependent nephrotic syndrome in childrenPediatric Nephrology, 2009
- Mycophenolate Mofetil versus Cyclophosphamide for Induction Treatment of Lupus NephritisJournal of the American Society of Nephrology, 2009
- The primary nephrotic syndrome in children. Identification of patients with minimal change nephrotic syndrome from initial response to prednisone: A report of the international study of kidney disease in childrenThe Journal of Pediatrics, 1981