Aragon E, Resontoc L P, Chan Y H, Lau Y W, Tan P H, Loh H L, Ng K H, Yap H K
Shaw-NKF-NUH Children's Kidney Centre, Khoo Teck Puat-National University Children's Medical Institute, National University Health System, Singapore.
Biostatistics Unit, Yong Loo Lin School of Medicine, National University of Singapore, Singapore.
Lupus. 2016 Apr;25(4):399-406. doi: 10.1177/0961203315615220. Epub 2015 Nov 3.
We have previously reported the one-year outcomes of 16 children with severe proliferative lupus nephritis (LN) who were treated using a multi-targeted induction protocol based on intravenous (IV) pulse methylprednisolone (MP), mycophenolate mofetil (MMF) and cyclosporine (CSA). This study examined the long-term renal outcomes of these 16 children, followed up for a median duration of 9.2 years (range 5.8-14.2 years). Primary treatment outcome was complete renal remission. Secondary outcomes included patient and renal survival as well as relapse-free and event-free survival. All patients achieved complete renal remission within 24 months (median 8.7 months, range 4.0-24.0 months). Comparing clinical and laboratory parameters at induction and last follow-up, respectively, Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) score (25.4 ± 8.7 vs. 0.4 ± 0.8), serum complement C3 (47 ± 21 vs. 107 ± 27 mg/dL), estimated glomerular filtration rate (eGFR) (72 ± 57 vs. 109.7 ± 43 ml/min/1.73 m2) and urine protein (6.97 ± 7.09 vs. 0.2 ± 0.02 g/day/1.73 m2) improved significantly (p < 0.05). Kaplan-Meier survival analysis showed a cumulative ten-year renal relapse-free survival of 73.3% when considering relapses with severe proteinuria >1 g/day/1.73 m2. Cumulative probability that hospitalization would not be required was 93.8% at one year, and 71.4% at ten years. Our multi-targeted protocol for induction and maintenance therapy in Asian children with severe proliferative LN resulted in good long-term patient survival and renal preservation, with a good safety profile.
我们之前报道过16例重症增殖性狼疮性肾炎（LN）患儿采用基于静脉注射（IV）甲泼尼龙（MP）、霉酚酸酯（MMF）和环孢素（CSA）的多靶点诱导方案治疗的1年结局。本研究考察了这16例患儿的长期肾脏结局，随访中位时间为9.2年（范围5.8 - 14.2年）。主要治疗结局为完全肾脏缓解。次要结局包括患者生存率和肾脏生存率以及无复发和无事件生存率。所有患者均在24个月内实现完全肾脏缓解（中位时间8.7个月，范围4.0 - 24.0个月）。分别比较诱导期和末次随访时的临床和实验室参数，系统性红斑狼疮疾病活动指数（SLEDAI）评分（25.4±8.7对0.4±0.8）、血清补体C3（47±21对107±27mg/dL）、估计肾小球滤过率（eGFR）（72±57对109.7±43ml/min/1.73m²）和尿蛋白（6.97±7.09对0.2±0.02g/天/1.73m²）均显著改善（p<0.05）。Kaplan-Meier生存分析显示，当将严重蛋白尿>1g/天/1.73m²的复发考虑在内时，累积十年无肾脏复发生存率为7三个月内实现完全肾脏缓解（中位时间8.7个月，范围4.0 - 24.0个月）。分别比较诱导期和末次随访时的临床和实验室参数，系统性红斑狼疮疾病活动指数（SLEDAI）评分（25.4±8.7对0.4±0.8）、血清补体C3（47±21对107±27mg/dL）、估计肾小球滤过率（eGFR）（72±57对109.7±43ml/min/1.73m²）和尿蛋白（6.97±7.09对0.2±0.02g/天/1.73m²）均显著改善（p<0.05）。Kaplan-Meier生存分析显示，当将严重蛋白尿>1g/天/1.73m²的复发考虑在内时，累积十年无肾脏复发生存率为73.3%。一年时无需住院的累积概率为93.8%，十年时为71.4%。我们针对亚洲重症增殖性LN患儿的诱导和维持治疗多靶点方案导致了良好的长期患者生存和肾脏保留，且安全性良好。 3.3%。一年时无需住院的累积概率为93.8%，十年时为71.4%。我们针对亚洲重症增殖性LN患儿的诱导和维持治疗多靶点方案导致了良好的长期患者生存和肾脏保留，且安全性良好。
