Background

Lupus low disease activity state (LLDAS) is a treatment target for patients with SLE and is associated with decreased risk for severe flare and new damage. We investigated the utility of the achievement of LLDAS in children with lupus nephritis and whether attainment of LLDAS is associated with more favorable outcomes.

Methods

Data of children, diagnosed with biopsy-proven lupus nephritis between January 2012 and December 2020, were retrospectively analyzed.

Results

For patients who did not achieve LLDAS after initial treatment (first 6 months), presence of autoimmune hemolytic anemia (62% vs. 18%, p = 0.047), anti-Sm (85% vs. 18%, p = 0.003) and anti-dsDNA (77% vs. 27%, p = 0.038) antibodies, proliferative lupus nephritis (77% vs. 27%, p = 0.038), and hypertension (69% vs. 9%, p = 0.005) at onset were more frequently encountered. Also, a lower rate of complete kidney response (43% vs. 100%, p = 0.005) and a higher rate of hypertension (86% vs. 13%, p = 0.002) were observed in patients who did not achieve LLDAS-50, defined as being in LLDAS at least 50% of the observation time. Attainment of both LLDAS after initial treatment and LLDAS-50 were associated with lower rates of kidney flare (p = 0.001 and p = 0.002, respectively) and damage accrual (p = 0.007 and p = 0.02, respectively) through the observation period.

Conclusions

LLDAS is an attainable treatment target for children with lupus nephritis and associated with lower rates of kidney flare and damage. Presence of hematologic involvement, hypertension, and proliferative lupus nephritis at onset adversely influenced the early achievement of LLDAS.

Graphical abstract

中文翻译：

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狼疮低疾病活动状态作为小儿狼疮性肾炎患者的治疗目标

背景

狼疮低疾病活动状态 (LLDAS) 是 SLE 患者的治疗目标，与严重发作和新损伤风险降低有关。我们调查了 LLDAS 对狼疮性肾炎儿童的效用，以及 LLDAS 的实现是否与更有利的结果相关。

方法

回顾性分析了 2012 年 1 月至 2020 年 12 月期间经活检证实患有狼疮性肾炎的儿童的数据。

结果

对于初始治疗后（前 6 个月）未达到 LLDAS 的患者，存在自身免疫性溶血性贫血（62% 对 18%，p  = 0.047）、抗 Sm（85% 对 18%，p  = 0.003）和抗 dsDNA（77% 对 27%，p  = 0.038）抗体、增殖性狼疮性肾炎（77% 对 27%，p  = 0.038）和高血压（69% 对 9%，p  = 0.005）在发病时是遇到的比较频繁。此外，较低的肾脏完全反应率（43% 对 100%，p  = 0.005）和较高的高血压率（86% 对 13%，p = 0.002) 在未达到 LLDAS-50 的患者中观察到，LLDAS-50 定义为至少 50% 的观察时间处于 LLDAS 中。 初始治疗后达到 LLDAS 和 LLDAS-50 与整个观察期间较低的肾耀斑率（分别为p  = 0.001 和p  = 0.002）和应计损伤率（分别为p  = 0.007 和p = 0.02）相关。

结论

LLDAS 是狼疮性肾炎患儿可达到的治疗目标，并且与较低的肾脏复发和损伤率相关。发病时存在血液学受累、高血压和增生性狼疮性肾炎对 LLDAS 的早期实现产生不利影响。

图形概要