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埃及系统性红斑狼疮患儿:全球形势下的本土特征。

Systemic lupus erythematosus children in Egypt: Homeland spectrum amid the global situation.

机构信息

Rheumatology Department, Faculty of Medicine, Cairo University, Cairo, Egypt.

Pediatrics Department, Rheumatology and Nephrology Unit, 68782Tanta University, Gharbia, Egypt.

出版信息

Lupus. 2021 Nov;30(13):2135-2143. doi: 10.1177/09612033211043010. Epub 2021 Sep 16.

DOI:10.1177/09612033211043010
PMID:34528835
Abstract

OBJECTIVES

This study aims to present the manifestations of juvenile systemic lupus erythematosus (JSLE) across Egypt, to focus on age at onset and gender-driven influence on disease characteristics, and to compare findings to other countries.

METHODS

The study included 404 Egyptian children with systemic lupus erythematosus (SLE) presenting to one of the specialized rheumatology centers corresponding to 13 major governorates. Juvenile cases age was ≤ 16°years at the time of recruitment. The SLE Disease Activity Index (SLEDAI) and damage index (DI) were assessed.

RESULTS

The mean age was 13.2 ± 2.4°years; 355 females and 49 males (7.2:1), and the disease duration was 2.3 ± 1.6 years, while age at disease onset was 11.1 ± 2.5°years. Their SLEDAI was 13.5 ± 12.3, and DI, 0.36 ± 0.78. The overall estimated prevalence of childhood-SLE patients in the recruited cohort in Egypt was 1/100,000 population (0.24/100000 males and 1.8/100000 females). 7.4% developed pre-pubertal SLE (≤ 7 years); 73.3%, peri-pubertal; and 19.3% during early adolescence. The differences according to age group were equal for gender and clinical manifestations except skin lesions present in 59.3% of pre-pubertal onset, 74.6% of peri-pubertal, and 84.2% of adolescents ( = 0.029), and renal involvement in 73.8% of peripubertal, 62.1% of pre-pubertal and 58.9% of adolescents ( = 0.03). Laboratory investigations, SLEDAI, and DI were similar among age categories. Lupus nephritis was more common in Egypt compared to JSLE from other countries.

CONCLUSION

Our large multicenter study identified that female gender influenced disease characteristics with more frequent skin involvement. Skin lesions were significantly higher in adolescents, while renal involvement in peri-pubertal children.

摘要

目的

本研究旨在展示埃及青少年系统性红斑狼疮 (JSLE) 的临床表现,重点关注发病年龄和性别对疾病特征的影响,并将研究结果与其他国家进行比较。

方法

本研究纳入了来自埃及 13 个主要省份的 404 名患有系统性红斑狼疮 (SLE) 的儿童患者,这些患者的年龄均在 16 岁以下。采用 SLE 疾病活动指数 (SLEDAI) 和损伤指数 (DI) 对患者进行评估。

结果

患者的平均年龄为 13.2 ± 2.4 岁,其中女性 355 例,男性 49 例(7.2:1),疾病持续时间为 2.3 ± 1.6 年,发病年龄为 11.1 ± 2.5 岁。SLEDAI 评分为 13.5 ± 12.3,DI 评分为 0.36 ± 0.78。研究发现,在招募的患者中,埃及儿童 SLE 的总体患病率为 1/100000 人(男性 0.24/100000 人,女性 1.8/100000 人)。7.4%的患者为青春期前发病(≤ 7 岁),73.3%为青春期发病,19.3%为青春期后发病。不同年龄组的性别和临床表现差异无统计学意义,但皮肤病变在青春期前发病、青春期发病和青春期后发病患者中的发生率分别为 59.3%、74.6%和 84.2%(=0.029),肾脏受累在青春期发病、青春期前发病和青春期后发病患者中的发生率分别为 73.8%、62.1%和 58.9%(=0.03)。实验室检查、SLEDAI 和 DI 在不同年龄组之间无差异。与其他国家的 JSLE 相比,埃及的狼疮肾炎更为常见。

结论

本研究发现,在埃及,女性性别影响疾病特征,使皮肤受累更为常见。皮肤病变在青少年患者中更为常见,而肾脏受累则在青春期前儿童中更为常见。

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Health Related Quality of Life in Juvenile-Onset Systemic Lupus Erythematosus: A Questionnaire-Based Study.
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