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儿童系统性红斑狼疮中的急性肾衰竭:治疗与预后

Acute renal failure in paediatric systemic lupus erythematosus: treatment and outcome.

作者信息

Benseler S M, Bargman J M, Feldman B M, Tyrrell P N, Harvey E, Hebert D, Silverman E D

机构信息

Division of Rheumatology, Department of Pediatrics, The Hospital for Sick Children, Toronto, Ontario M5G1X8, Canada.

出版信息

Rheumatology (Oxford). 2009 Feb;48(2):176-82. doi: 10.1093/rheumatology/ken445.

Abstract

OBJECTIVE

To determine the outcome of paediatric SLE (pSLE) patients with nephritis who developed acute renal failure (ARF). Efficacy and safety of treatment regimens were compared.

METHODS

A total of 249 pSLE patients were diagnosed and prospectively followed at a single centre between July 1973 and July 2003; 127 children (51%) had lupus nephritis. ARF was defined as serum creatinine of > 250 micromol/l or > 75% above baseline. Standardized assessments included clinical data and medications, laboratory testing, disease activity and damage scores were obtained. Subsequent renal flares were documented.

PRIMARY OUTCOME

renal function at last follow-up.

SECONDARY OUTCOMES

treatment efficacy and safety. AZA- and cyclophosphamide (CYCLO)-treated patients were compared. Propensity score methods were applied to balance covariates. An intention to treat approach was chosen.

RESULTS

The ARF study cohort included 50 patients; 13 boys and 37 girls with a median age of 13.2 yrs at diagnosis and a mean follow-up of 45 months. Renal histology: Class III nephritis in 16; Class IV in 34. Dialysis requirement and disease activity were similar in both groups.

TREATMENT

AZA in 33 patients, CYCLO in 9 and corticosteroids only in 8.

OUTCOME

no statistically significant or clinically relevant differences were found for any of the outcome measures including last serum creatinine, time to renal flare, overall renal survival, disease activity over time, disease damage, mean annual corticosteroid dose and rate of infection.

CONCLUSION

The treatment of renal failure in this pSLE cohort was associated with an excellent outcome. AZA and CYCLO were equally efficacious.

摘要

目的

确定患有肾炎且发生急性肾衰竭(ARF)的儿童系统性红斑狼疮(pSLE)患者的预后情况。比较治疗方案的疗效和安全性。

方法

1973年7月至2003年7月期间,在单一中心对249例pSLE患者进行诊断并进行前瞻性随访;127名儿童(51%)患有狼疮性肾炎。ARF定义为血清肌酐>250微摩尔/升或高于基线水平75%以上。标准化评估包括临床数据和用药情况、实验室检查、疾病活动度和损伤评分。记录随后的肾脏病情复发情况。

主要结局

最后一次随访时的肾功能。

次要结局

治疗疗效和安全性。对接受硫唑嘌呤(AZA)和环磷酰胺(CYCLO)治疗的患者进行比较。采用倾向评分法平衡协变量。采用意向性治疗方法。

结果

ARF研究队列包括50例患者;13名男孩和37名女孩,诊断时的中位年龄为13.2岁,平均随访45个月。肾脏组织学检查:Ⅲ级肾炎16例;Ⅳ级34例。两组的透析需求和疾病活动度相似。

治疗

33例患者使用AZA,9例使用CYCLO,8例仅使用皮质类固醇。

结局

在任何结局指标上均未发现统计学上显著或临床相关的差异,包括末次血清肌酐、肾脏病情复发时间、总体肾脏生存率、随时间的疾病活动度、疾病损伤、平均每年皮质类固醇剂量和感染率。

结论

该pSLE队列中肾衰竭的治疗预后良好。AZA和CYCLO疗效相当。

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