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在一个包含 428 名狼疮性肾炎患儿的国际队列中开发临床和实验室生物标志物。

Development of clinical and laboratory biomarkers in an international cohort of 428 children with lupus nephritis.

机构信息

Paediatric Unit, Maggiore Hospital, Azienda USL, Bologna, Italy.

Pediatric Nephrology, The University of British Columbia, BC Children's Hospital, Vancouver, BC, Canada.

出版信息

Pediatr Nephrol. 2024 Oct;39(10):2959-2968. doi: 10.1007/s00467-024-06405-6. Epub 2024 May 28.

Abstract

BACKGROUND

Lupus nephritis (LN) is a very severe manifestation of lupus. There is no consensus on which treatment goals should be achieved to protect kidney function in children with LN.

METHODS

We retrospectively analyzed trends of commonly used laboratory biomarkers of 428 patients (≤ 18 years old) with biopsy-proven LN class ≥ III. We compared data of patients who developed stable kidney remission from 6 to 24 months with those who did not.

RESULTS

Twenty-five percent of patients maintained kidney stable remission while 75% did not. More patients with stable kidney remission showed normal hemoglobin and erythrocyte sedimentation rate from 6 to 24 months compared to the group without stable kidney remission. eGFR ≥ 90 ml/min/1.73m at onset predicted the development of stable kidney remission (93.8%) compared to 64.7% in those without stable remission (P < 0.00001). At diagnosis, 5.9% and 20.2% of the patients showed no proteinuria in the group with and without stable kidney remission, respectively (P = 0.0001). dsDNA antibodies decreased from onset of treatment mainly during the first 3 months in all groups, but more than 50% of all patients in both groups never normalized after 6 months. Complement C3 and C4 increased mainly in the first 3 months in all patients without any significant difference.

CONCLUSIONS

Normal eGFR and the absence of proteinuria at onset were predictors of stable kidney remission. Significantly more children showed normal levels of Hb and erythrocyte sedimentation rate (ESR) from 6 to 24 months in the group with stable kidney remission.

摘要

背景

狼疮肾炎(LN)是狼疮的一种非常严重的表现。对于 LN 患儿,在保护肾功能方面,尚无关于应实现哪些治疗目标的共识。

方法

我们回顾性分析了 428 例经活检证实的 LN 分级≥III 级的患儿的常用实验室生物标志物的变化趋势。我们比较了在 6 至 24 个月时达到稳定肾脏缓解的患者与未达到稳定肾脏缓解的患者的情况。

结果

25%的患者维持稳定的肾脏缓解,而 75%的患者未达到稳定的肾脏缓解。与未达到稳定肾脏缓解的患者相比,在 6 至 24 个月时,有更多稳定肾脏缓解的患者的血红蛋白和红细胞沉降率正常。发病时 eGFR≥90ml/min/1.73m2 预测了稳定肾脏缓解的发生(93.8%),而在未达到稳定缓解的患者中这一比例为 64.7%(P<0.00001)。在诊断时,有稳定肾脏缓解组和无稳定肾脏缓解组分别有 5.9%和 20.2%的患者无蛋白尿(P=0.0001)。dsDNA 抗体从治疗开始主要在头 3 个月下降,但在治疗后 6 个月后,两组中仍有超过 50%的患者未恢复正常。在所有患者中,补体 C3 和 C4 在头 3 个月主要增加,但两组之间没有显著差异。

结论

正常的 eGFR 和发病时无蛋白尿是稳定肾脏缓解的预测指标。在有稳定肾脏缓解的患者中,从 6 至 24 个月时,更多的儿童的血红蛋白和红细胞沉降率(ESR)水平正常。

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