Current understandings in treating children with steroid-resistant nephrotic syndrome
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Current understandings in treating children with steroid-resistant nephrotic syndrome. / Lee, Jiwon M; Kronbichler, Andreas; Shin, Jae Il; Oh, Jun.
In: PEDIATR NEPHROL, Vol. 36, No. 4, 04.2021, p. 747-761.Research output: SCORING: Contribution to journal › SCORING: Review article › Research
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TY - JOUR
T1 - Current understandings in treating children with steroid-resistant nephrotic syndrome
AU - Lee, Jiwon M
AU - Kronbichler, Andreas
AU - Shin, Jae Il
AU - Oh, Jun
PY - 2021/4
Y1 - 2021/4
N2 - Steroid-resistant nephrotic syndrome (SRNS) remains a challenge for paediatric nephrologists. SRNS is viewed as a heterogeneous disease entity including immune-based and monogenic aetiologies. Because SRNS is rare, treatment strategies are individualized and vary among centres of expertise. Calcineurin inhibitors (CNI) have been effectively used to induce remission in patients with immune-based SRNS; however, there is still no consensus on treating children who become either CNI-dependent or CNI-resistant. Rituximab is a steroid-sparing agent for patients with steroid-sensitive nephrotic syndrome, but its efficacy in SRNS is controversial. Recently, several novel monoclonal antibodies are emerging as treatment option, but their efficacy remains to be seen. Non-immune therapies, such as angiotensin-converting enzyme inhibitors or angiotensin II receptor blockers, have been proven efficacious in children with SRNS and are recommended as adjuvant agents. This review summarizes and discusses our current understandings in treating children with idiopathic SRNS.
AB - Steroid-resistant nephrotic syndrome (SRNS) remains a challenge for paediatric nephrologists. SRNS is viewed as a heterogeneous disease entity including immune-based and monogenic aetiologies. Because SRNS is rare, treatment strategies are individualized and vary among centres of expertise. Calcineurin inhibitors (CNI) have been effectively used to induce remission in patients with immune-based SRNS; however, there is still no consensus on treating children who become either CNI-dependent or CNI-resistant. Rituximab is a steroid-sparing agent for patients with steroid-sensitive nephrotic syndrome, but its efficacy in SRNS is controversial. Recently, several novel monoclonal antibodies are emerging as treatment option, but their efficacy remains to be seen. Non-immune therapies, such as angiotensin-converting enzyme inhibitors or angiotensin II receptor blockers, have been proven efficacious in children with SRNS and are recommended as adjuvant agents. This review summarizes and discusses our current understandings in treating children with idiopathic SRNS.
U2 - 10.1007/s00467-020-04476-9
DO - 10.1007/s00467-020-04476-9
M3 - SCORING: Review article
C2 - 32086590
VL - 36
SP - 747
EP - 761
JO - PEDIATR NEPHROL
JF - PEDIATR NEPHROL
SN - 0931-041X
IS - 4
ER -