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抗 C1q 自身抗体作为儿童起病系统性红斑狼疮肾损害的标志物。

Anti-C1q autoantibodies as markers of renal involvement in childhood-onset systemic lupus erythematosus.

机构信息

Department of Pathology, Hôpital Femme-Mère-Enfant, Hospices Civils de Lyon, 59 boulevard Pinel, 69500, Bron, France.

Université Claude Bernard Lyon 1, Lyon, France.

出版信息

Pediatr Nephrol. 2017 Sep;32(9):1537-1545. doi: 10.1007/s00467-017-3646-z. Epub 2017 Mar 25.

Abstract

BACKGROUND

Childhood-onset systemic lupus erythematosus (cSLE) is rare, and considered more severe than its adult-onset counterpart. Lupus nephritis (LN) occurs more frequently in children, accounting for higher long-term morbidity and mortality compared with adults. Thus, reliable biological markers are needed to predict disease course. This study aimed to investigate the capacity of anti-C1q autoantibodies (Abs) to predict renal flare and global disease activity in cSLE patients, and association with disease activity and kidney involvement.

METHODS

Twenty-eight patients with cSLE including 19 patients (68%) with a history of LN were included retrospectively. Anti-C1q Abs were analysed by ELISA at renal flare-up or in the quiescent phase of disease and compared with Farr dsDNA assay.

RESULTS

Thirty-one flares occurred during follow-up: anti-C1q Abs were positive in 26 (84%), strongly associated with active disease status (p < 0.0001), and correlated with global disease activity score (p < 0.0001) and anti-dsDNA Abs presence (p < 0.0001). The specificity of anti-C1q Abs was higher than anti-dsDNA (73% vs 19%) in discriminating LN patients, whereas the receiver operating characteristic curves were not statistically different (0.83 ± 0.06 vs 0.78 ± 0.08 respectively), similar to C3 dosage. The presence of anti-C1q Abs at diagnosis was not predictive for global or renal flare. Introduction of a modified SLEDAI score excluding dsDNA Abs, demonstrated a stronger correlation of anti-C1q Abs titres with SLEDAI score in comparison with the Farr test.

CONCLUSION

Anti-C1q Abs seem very specific to flares, including LN in children, and their role in daily practice compared with the Farr dsDNA assay needs to be defined.

摘要

背景

儿童发病的系统性红斑狼疮(cSLE)较为罕见,且被认为比成人发病的系统性红斑狼疮更为严重。狼疮肾炎(LN)在儿童中更为常见,与成人相比,其长期发病率和死亡率更高。因此,需要可靠的生物标志物来预测疾病进程。本研究旨在探讨抗 C1q 自身抗体(Abs)预测儿童发病的系统性红斑狼疮患者肾复发和整体疾病活动的能力,并探讨其与疾病活动和肾脏受累的关系。

方法

回顾性纳入 28 例儿童发病的系统性红斑狼疮患者,包括 19 例(68%)有狼疮肾炎病史。采用 ELISA 法检测抗 C1q Abs,在疾病复发期或缓解期与 Farr dsDNA 检测结果进行比较。

结果

随访期间发生 31 次复发:抗 C1q Abs 阳性 26 例(84%),与疾病活动状态密切相关(p<0.0001),与整体疾病活动评分(p<0.0001)和抗 dsDNA Abs 存在(p<0.0001)相关。抗 C1q Abs 对狼疮肾炎患者的特异性高于抗 dsDNA(73%比 19%),但两者的受试者工作特征曲线差异无统计学意义(分别为 0.83±0.06 与 0.78±0.08),与 C3 剂量相似。发病时存在抗 C1q Abs 对整体或肾复发无预测作用。排除 dsDNA Abs 后引入改良的 SLEDAI 评分,与 Farr 试验相比,抗 C1q Abs 滴度与 SLEDAI 评分的相关性更强。

结论

抗 C1q Abs 似乎对包括儿童狼疮肾炎在内的复发非常特异,与 Farr dsDNA 检测相比,其在日常实践中的作用需要进一步明确。

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