Background

Pediatric lupus nephritis (pLN) is one of the most refractory secondary kidney diseases in childhood. The treat-to-target (T2T) strategy has become the standard treatment for systemic lupus erythematosus (SLE). This study reviewed clinical features, overall remission status, and factors affecting prognosis, to guide pLN management according to T2T strategy.

Methods

This single-center retrospective study studied 220 children diagnosed with LN from January 2012 to December 2018, with > 6-month follow-up data on 173 and complete data on 137 patients. Primary outcome was treatment failure (deterioration or no response) at the latest follow-up.

Results

The most common pLN manifestation was proteinuria (81.36%). Females presented more often with rash (P<0.001) and alopecia (P=0.026) than males. Class IV LN (33.33%) was the most common grade on kidney biopsy. Median follow-up was 27.20 months (IQR, 15.78–44.45 months). One-, 3-, and 5-year cumulative overall survival rates were 93.5%, 87.8%, and 86.5%, respectively. The 5-year cumulative kidney survival rate was 97.1%. Regarding initial therapy, efficacy of corticosteroids combined with immunosuppressive agents was significantly better than corticosteroids alone (P=0.010). Factors with P<0.05 in univariate analysis, including hypoalbuminemia, higher SCr at diagnosis, lower eGFR at diagnosis, anti-dsDNA positivity, heavy proteinuria, hypertension, nervous-system involvement, treatment non-compliance, and SLEDAI-2K score, were used for logistic regression analysis. Logistic regression analysis showed hypertension (OR=0.845, P=0.011), nervous-system involvement (OR=4.240, P=0.005), treatment non-compliance (OR=6.433, P=0.001), and lower estimated glomerular filtration rate at diagnosis (OR=1.020, P=0.021) affected prognosis. At end of follow-up, 34.31% achieved varying levels of remission, and 8.76% were in low disease activity state (LDAS).

Conclusions

pLN usually presented with proteinuria, and class IV LN was the dominant pathology. Hypertension, nervous-system involvement, treatment non-compliance, and lower eGFR at diagnosis were independent risk factors for poor prognosis of kidney outcomes. Compared with renal remission rate and cumulative overall survival rate, the proportion of targets achieved was not ideal, suggesting T2T strategy should be used to guide pLN management.

Graphical abstract

中文翻译：

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小儿狼疮性肾炎的临床表现、预后和靶向治疗评估

背景

小儿狼疮性肾炎 (pLN) 是儿童期最难治的继发性肾脏疾病之一。靶向治疗 (T2T) 策略已成为系统性红斑狼疮 (SLE) 的标准治疗方法。本研究回顾了临床特征、总体缓解状态和影响预后的因素，以根据 T2T 策略指导 pLN 管理。

方法

这项单中心回顾性研究研究了 2012 年 1 月至 2018 年 12 月期间诊断为 LN 的 220 名儿童，其中 173 名患者的随访数据超过 6 个月，137 名患者的数据完整。主要结果是最近一次随访时治疗失败（恶化或无反应）。

结果

最常见的 pLN 表现是蛋白尿（81.36%）。女性比男性更常出现皮疹（P<0.001）和脱发（P=0.026）。IV 级 LN (33.33%) 是肾活检中最常见的等级。中位随访时间为 27.20 个月（IQR，15.78-44.45 个月）。一年、三年和五年累计总生存率分别为 93.5%、87.8% 和 86.5%。5年累积肾脏存活率为97.1%。关于初始治疗，皮质类固醇联合免疫抑制剂的疗效明显优于单独使用皮质类固醇（P=0.010）。单因素分析中P<0.05的因素包括低白蛋白血症、诊断时较高的SCr、诊断时较低的eGFR、抗dsDNA阳性、大量蛋白尿、高血压、神经系统受累、治疗不依从性和SLEDAI-2K评分，用于逻辑回归分析。Logistic 回归分析显示高血压（OR=0.845，P=0.011）、神经系统受累（OR=4.240，P=0.005）、治疗不依从性（OR=6.433，P=0.001）和较低的估计肾小球滤过率诊断（OR=1.020，P=0.021）影响预后。随访结束时，34.31% 的患者达到不同程度的缓解，8.76% 的患者处于低疾病活动状态（LDAS）。

结论

pLN常表现为蛋白尿，以Ⅳ级LN为主。高血压、神经系统受累、治疗不依从性和诊断时较低的 eGFR 是肾脏预后不良的独立危险因素。与肾脏缓解率和累积总生存率相比，达到的目标比例并不理想，提示应采用T2T策略指导pLN管理。

图形概要