| Literature DB >> 33665752 |
Federica Zotta1, Marina Vivarelli1, Francesco Emma2.
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.Entities:
Keywords: Calcineurin inhibitor; Cyclophosphamide; Cyclosporine A; Mycophenolate mofetil; Prednisone; Relapse; Rituximab; Tacrolimus
Mesh:
Substances:
Year: 2021 PMID: 33665752 DOI: 10.1007/s00467-021-04983-3
Source DB: PubMed Journal: Pediatr Nephrol ISSN: 0931-041X Impact factor: 3.714