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.

中文翻译：

---

目前治疗类固醇抵抗性肾病综合征儿童的认识。

类固醇抵抗性肾病综合征 (SRNS) 仍然是儿科肾病学家面临的挑战。SRNS 被视为一种异质性疾病实体，包括基于免疫的和单基因病因。由于 SRNS 很少见，治疗策略因人而异，并且因专业中心而异。钙调神经磷酸酶抑制剂 (CNI) 已被有效地用于诱导基于免疫的 SRNS 患者的缓解；然而，对于 CNI 依赖或 CNI 耐药的儿童的治疗仍未达成共识。利妥昔单抗是类固醇敏感性肾病综合征患者的类固醇节约剂，但其在 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。非免疫疗法，例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。本综述总结并讨论了我们目前在治疗儿童特发性 SRN​​S 方面的认识。