治疗类固醇抵抗型肾病综合征患儿的最新认识。
Current understandings in treating children with steroid-resistant nephrotic syndrome.
机构信息
Department of Pediatrics, Chungnam National University Hospital, Daejeon, South Korea.
Department of Internal Medicine IV (Nephrology and Hypertension), Medical University Innsbruck, Innsbruck, Austria.
出版信息
Pediatr Nephrol. 2021 Apr;36(4):747-761. doi: 10.1007/s00467-020-04476-9. Epub 2020 Feb 21.
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.
类固醇抵抗性肾病综合征（SRNS）仍然是儿科肾脏病学家面临的挑战。SRNS 被视为一种异质性疾病实体，包括免疫性和单基因病因。由于 SRNS 较为罕见，因此治疗策略是个体化的，并且在专业中心之间存在差异。钙调神经磷酸酶抑制剂（CNI）已被有效地用于诱导免疫性 SRNS 患者缓解；然而，对于那些成为 CNI 依赖性或 CNI 耐药性的患者，仍然没有共识。利妥昔单抗是治疗类固醇敏感性肾病综合征患者的类固醇保留剂，但它在 SRNS 中的疗效存在争议。最近，几种新型单克隆抗体作为治疗选择出现，但它们的疗效仍有待观察。非免疫治疗，如血管紧张素转换酶抑制剂或血管紧张素 II 受体阻滞剂，已被证明对 SRNS 患儿有效，并被推荐作为辅助药物。这篇综述总结和讨论了我们目前对治疗特发性 SRNS 患儿的理解。
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