Dowsett Thomas, Oni Louise
Department of Paediatric Nephrology, Royal Manchester Children's NHS Trust Hospital, Manchester.
Department of Paediatric Nephrology, Alder Hey Children's NHS Foundation Trust Hospital.
Curr Opin Pediatr. 2022 Apr 1;34(2):203-208. doi: 10.1097/MOP.0000000000001101.
Systemic lupus erythematosus is a lifelong, multisystemic disease. Around a fifth of patients present during childhood. Children are recognized to have a more active disease course with more renal involvement (lupus nephritis) when compared with adults. This review article summarizes the current literature surrounding the management of paediatric lupus nephritis.
International recommendations agree that active, proliferative forms of lupus nephritis are treated with a period of intense induction therapy aimed at inducing remission followed by maintenance immunosuppressive therapy for at least 3 years. Complete response rates in lupus nephritis remain inadequate, in the region of 40-60%, and disease flares are common. Revised histological classification have been proposed but they are yet to be adopted in clinical practice. Lupus nephritis progresses to chronic kidney disease (CKD) stage 5 (kidney failure) in more than 10% of patients within 10 years however the rates of CKD stages 1-4 remain largely unknown. Current trials are focused on the use of biologic agents as adjuncts to current therapy.
There is an urgent need for better outcomes in paediatric lupus nephritis. The use of biomarkers to monitor lupus nephritis and scientific studies to improve our understanding of the pathogenesis offer hope of improved outcomes.
系统性红斑狼疮是一种终身的多系统疾病。约五分之一的患者在儿童期发病。与成人相比，儿童的病情发展更为活跃，肾脏受累（狼疮性肾炎）更为常见。这篇综述文章总结了目前关于儿童狼疮性肾炎治疗的文献。
国际指南一致认为，对于活动性、增殖性狼疮性肾炎，应先进行一段时间的强化诱导治疗以诱导缓解，随后进行至少3年的维持免疫抑制治疗。狼疮性肾炎的完全缓解率仍然不尽人意，在40%-60%之间，且疾病复发很常见。虽然已提出修订后的组织学分类，但尚未在临床实践中采用。超过10%的患者在10年内狼疮性肾炎会进展为慢性肾脏病5期（肾衰竭），然而1-4期慢性肾脏病的发生率在很大程度上仍不清楚。目前的试验主要集中在使用生物制剂作为现有治疗的辅助手段。
迫切需要改善儿童狼疮性肾炎的治疗效果。使用生物标志物监测狼疮性肾炎以及开展科学研究以增进我们对发病机制的理解，有望改善治疗效果。
