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中国系统性红斑狼疮治疗与研究协作组登记研究IX:中国儿童期起病的系统性红斑狼疮的临床特征与生存情况

Chinese Systemic Lupus Erythematosus Treatment and Research Group Registry IX: Clinical Features and Survival of Childhood-Onset Systemic Lupus Erythematosus in China.

作者信息

Wu Chan-Yuan, Li Cai-Feng, Wu Qing-Jun, Xu Jian-Hua, Jiang Lin-Di, Gong Lu, Wu Feng-Qi, Gu Jie-Ruo, Zhao Jiu-Liang, Li Meng-Tao, Zhao Yan, Zeng Xiao-Feng

机构信息

Department of Rheumatology, Peking Union Medical College Hospital, Peking Union Medical College and Chinese Academy of Medical Sciences, Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, Beijing 100730, China.

Department of Rheumatology, Beijing Children Hospital Affiliated to Capital Medical University, Beijing 100045, China.

出版信息

Chin Med J (Engl). 2017 Jun 5;130(11):1276-1282. doi: 10.4103/0366-6999.206346.

DOI:10.4103/0366-6999.206346
PMID:28524825
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC5455035/
Abstract

BACKGROUND

Approximately 15-20% cases of systemic lupus erythematosus (SLE) are diagnosed in children. There have been a few studies reporting the epidemiological data of pediatric-onset SLE (cSLE) in China, neither comparing the differences between cSLE and adult-onset SLE (aSLE). The aim of this study was to describe the impact of age of onset on clinical features and survival in cSLE patients in China based on the Chinese SLE Treatment and Research group (CSTAR) database.

METHODS

We made a prospective study of 225 cSLE patients (aged Results: The mean age of cSLE patients was 12.16 ± 2.92 years, with 187 (83.1%) females. Fever (P < 0.001) as well as mucocutaneous (P < 0.001) and renal (P = 0.006) disorders were found to be significantly more frequent in cSLE patients as initial symptoms, while muscle and joint lesions were significantly less common compared to aSLE subjects (P < 0.001). The cSLE patients were found to present more frequently with malar rash (P = 0.001; odds ratio [OR], 0.624; 95% confidence interval [CI], 0.470-0.829) but less frequently with arthritis (P < 0.001; OR, 2.013; 95% CI, 1.512-2.679) and serositis (P = 0.030; OR, 1.629; 95% CI, 1.053-2.520). There was no significant difference in SLE disease activity index scores between cSLE and aSLE groups (P = 0.478). Cox regression indicated that childhood onset was the risk factor for organ damage in lupus patients (hazard ratio 0.335 [0.170-0.658], P = 0.001). The survival curves between the cSLE and aSLE groups had no significant difference as determined by the log-rank test (0.557, P = 0.455).

CONCLUSIONS

cSLE in China has different clinical features and more inflammation than aSLE patients. Damage may be less in children and there is no difference in 5- year survival between cSLE and aSLE groups.

摘要

背景

约15%-20%的系统性红斑狼疮(SLE)病例在儿童期被诊断出来。在中国,仅有少数研究报告了儿童期发病的SLE(cSLE)的流行病学数据,且均未比较cSLE与成人期发病的SLE(aSLE)之间的差异。本研究旨在基于中国SLE治疗与研究组(CSTAR)数据库,描述发病年龄对中国cSLE患者临床特征和生存情况的影响。

方法

我们对225例cSLE患者进行了一项前瞻性研究(年龄……结果:cSLE患者的平均年龄为12.16±2.92岁,其中187例(83.1%)为女性。发热(P<0.001)以及黏膜皮肤(P<0.001)和肾脏(P=0.006)疾病作为初始症状在cSLE患者中更为常见,而与aSLE患者相比,肌肉和关节病变明显较少见(P<0.001)。发现cSLE患者更常出现蝶形红斑(P=0.001;比值比[OR],0.624;95%置信区间[CI],0.470-0.829),但较少出现关节炎(P<0.001;OR,2.013;95%CI,1.512-2.679)和浆膜炎(P=0.030;OR,1.629;95%CI,1.053-2.520)。cSLE组和aSLE组之间的SLE疾病活动指数评分无显著差异(P=0.478)。Cox回归表明儿童期发病是狼疮患者器官损伤的危险因素(风险比0.335[0.170-0.658],P=0.001)。log-rank检验显示cSLE组和aSLE组之间的生存曲线无显著差异(0.557,P=0.455)。

结论

中国的cSLE与aSLE患者相比具有不同的临床特征且炎症反应更明显。儿童的损伤可能较少,cSLE组和aSLE组之间的5年生存率无差异。

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