Systemic lupus erythematosus is a rare lifelong multi-systemic autoimmune condition. Juvenile-onset SLE (JSLE) is recognized to have a more active disease course when compared with adult-onset disease and patients have a worse long-term survival. Kidney involvement occurs in over 50% of children and treatment decisions are guided by the histological classification. Several international groups have produced treatment protocols that rely on an intense period of immunosuppression to halt the acute kidney inflammatory process, followed by maintenance therapy with close observation for disease improvement and prompt evaluation of disease flares. A reduced glomerular filtration rate at presentation is predictive of later stage chronic kidney disease (CKD) in multivariate analysis. Kidney remission remains suboptimal with only 40-60% of patients achieving complete remission. Kidney flares are seen in over a third of patients. The rate of CKD 5 is reported to be up to 15% and the presence of lupus nephritis (LN) has an established link with an associated increase in mortality. In established kidney failure, transplantation seems to be the optimal kidney replacement modality for this group of patients, ideally after a period of disease quiescence. Modified outcome measures in clinical trials have demonstrated that biologic agents can be effective in this disease. Current biologic agents under investigation include obinutuzimab, belimumab, atacicept, anifrolumab, tocilizumab, eculizumab, dapirolizumab, and abatacept. Future research should focus on discovering early disease biomarkers, including surrogates for later cardiovascular disease, and evaluating biological agents as adjuncts to improve the rates of complete remission and subsequently influence the kidney outcome. The aim of this review article is to summarize the current kidney outcomes for this disease with a view to identifying key areas that may help to reduce the risk of long-term CKD.

中文翻译：

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狼疮性肾炎儿童的肾脏结局。

系统性红斑狼疮是一种罕见的终生多系统自身免疫性疾病。与成人发病的疾病相比，青少年发病的 SLE (JSLE) 被认为具有更活跃的病程，并且患者的长期存活率更差。超过 50% 的儿童发生肾脏受累，治疗决定由组织学分类指导。一些国际组织已经制定了治疗方案，这些方案依赖于强烈的免疫抑制期来阻止急性肾脏炎症过程，然后进行维持治疗，密切观察疾病的改善情况并及时评估疾病发作。在多变量分析中，呈现时降低的肾小球滤过率可预测晚期慢性肾脏病 (CKD)。肾脏缓解仍然欠佳，只有 40-60% 的患者实现完全缓解。超过三分之一的患者出现肾脏耀斑。据报道，CKD 5 的发生率高达 15%，狼疮性肾炎 (LN) 的存在与死亡率的相关增加有确定的联系。在确定的肾功能衰竭中，移植似乎是这组患者的最佳肾脏替代方式，理想情况下是在疾病静止期之后。临床试验中改进的结果测量表明，生物制剂可以有效治疗这种疾病。目前正在研究的生物制剂包括obinutuzimab、belimumab、atacicept、anifrolumab、tocilizumab、eculizumab、dapirolizumab和abatacept。未来的研究应侧重于发现早期疾病生物标志物，包括后来心血管疾病的替代物，并评估生物制剂作为辅助手段以提高完全缓解率并随后影响肾脏结果。这篇评论文章的目的是总结目前这种疾病的肾脏结果，以确定可能有助于降低长期 CKD 风险的关键领域。