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儿童和青少年狼疮性肾炎:治疗、临床病程及预后

[Lupus nephritis in children and adolescents: therapy, clinical course and prognosis].

作者信息

Bogdanović Radovan, Nikolić Vesna, Ognjanović Milos, Dimitrijević Jovan, Marković-Lipkovski Jasmina, Pasić Srdjan, Minić Aleksandra, Stajić Natasa

机构信息

Dr. Vukan Chupitsh Institute of Mother and Child Health of Serbia, Belgrade.

出版信息

Srp Arh Celok Lek. 2002 Aug;130 Suppl 3:6-12.

PMID:12583307
Abstract

In 53 children and adolescents (47 males, 6 females) with lupus nephritis, clinical features at the time of renal biopsy were analyzed and correlated with pathohistological findings. Therapeutic regimens used and the renal status at the end of follow-up are presented, and factors significantly associated with adverse outcome are analyzed. The mean age at the time of diagnosis of systemic lupus was 12.9 +/- 2.6 (SD) years, the mean ages at the time of diagnosis of lupus nephritis and renal biopsy were 13.5 +/- 2.6 and 13.6 +/- 2.5 years, respectively. The patients having WHO classes I and II of lupus nephritis were treated with prednisone and/or azathioprine; those with WHO classes IV and III were treated with cyclophosphamide (25) or with corticosteroids (5) or with corticosteroids and azathioprine (4), five of them were also treated with plasmapheresis; the patients with WHO class V were treated with prednisone and azathioprine or cyclosporine. Repeated renal biopsies were performed in 12 patients: worsening of morphological lesions was found in four patients, improvement in two and no change was observed in six patients (all with WHO class IV). At the end of follow-up lasting from 0.1 to 14.6 years (mean 4.8 +/- 3.2 years) 80% of patients were in complete (49%) or partial (30%) remission, in 8% of patients the renal disease was clinically active, and in 13% of patients the adverse outcome was noted: one patient died in the first month after diagnosis from extrarenal complications, two patients were in preterminal and the other four in terminal renal failure. Adverse outcome was significantly associated with the presence of nephrotic syndrome at the time of biopsy and with class IV nephritis. The five-year patient's survival rate was 98.1%. The five-year kidney survival rate was 88.6% and 82.4% for the whole group and for the subgroup of patients having classes III and IV nephritis, respectively.

摘要

对53例狼疮性肾炎患儿及青少年(47例男性,6例女性)肾活检时的临床特征进行分析,并与病理组织学结果进行关联。介绍了所采用的治疗方案及随访结束时的肾脏状况,并分析了与不良结局显著相关的因素。系统性红斑狼疮诊断时的平均年龄为12.9±2.6(标准差)岁,狼疮性肾炎诊断时和肾活检时的平均年龄分别为13.5±2.6岁和13.6±2.5岁。世界卫生组织(WHO)狼疮性肾炎Ⅰ级和Ⅱ级的患者采用泼尼松和/或硫唑嘌呤治疗;WHOⅢ级和Ⅳ级的患者采用环磷酰胺(25例)或皮质类固醇(5例)或皮质类固醇与硫唑嘌呤(4例)治疗,其中5例还接受了血浆置换治疗;WHOⅤ级的患者采用泼尼松和硫唑嘌呤或环孢素治疗。12例患者进行了重复肾活检:4例患者形态学病变恶化,2例改善,6例无变化(均为WHOⅣ级)。随访时间为0.1至14.6年(平均4.8±3.2年),80%的患者完全缓解(49%)或部分缓解(30%),8%的患者肾脏疾病临床活动,13%的患者出现不良结局:1例患者在诊断后第一个月死于肾外并发症,2例患者处于终末期前,另外4例处于终末期肾衰竭。不良结局与活检时肾病综合征的存在以及Ⅳ级肾炎显著相关。患者的五年生存率为98.1%。整个组以及Ⅲ级和Ⅳ级肾炎患者亚组的五年肾脏生存率分别为88.6%和82.4%。

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