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英国不同种族青少年发病系统性红斑狼疮的临床和实验室表型。

Clinical and laboratory phenotypes in juvenile-onset Systemic Lupus Erythematosus across ethnicities in the UK.

机构信息

School of Medicine, University of Liverpool, Liverpool, UK.

Department of Women's & Children's Health, Institute of Translational Medicine, University of Liverpool, Liverpool, UK.

出版信息

Lupus. 2021 Apr;30(4):597-607. doi: 10.1177/0961203320984251. Epub 2021 Jan 7.

Abstract

Systemic lupus erythematosus (SLE) is a systemic autoimmune/inflammatory disease. Patients diagnosed with juvenile-onset SLE (jSLE), when compared to individuals with adult-onset SLE, develop more severe organ involvement, increased disease activity and greater tissue and organ damage. In adult-onset SLE, clinical characteristics, pathomechanisms, disease progression and outcomes do not only vary between individuals and age groups, but also ethnicities. However, in children and young people, the influence of ethnicity on disease onset, phenotype and outcome has not been investigated in detail. In this study, we investigated clinical and laboratory characteristics in pediatric SLE patients from different ethnic backgrounds (White Caucasian, Asian, Black African/Caribbean) accessing data from a national cohort of jSLE patients (the UK JSLE Cohort Study). Among jSLE patients in the UK, ethnicity affects both the disease's clinical course and outcomes. At diagnosis, Black African/Caribbean jSLE patients show more "classical" laboratory and clinical features when compared to White Caucasian or Asian patients. Black African/Caribbean jSLE patients exhibit more renal involvement and more frequently receive cyclophosphamide and rituximab. Studies targeting ethnicity-specific contributors to disease expression and phenotypes are necessary to improve our pathophysiological understanding, diagnosis and treatment of jSLE.

摘要

系统性红斑狼疮(SLE)是一种系统性自身免疫性/炎症性疾病。与成年发病的系统性红斑狼疮(SLE)患者相比,青少年发病的 SLE(jSLE)患者更易发生严重的器官受累、疾病活动度增加以及更大的组织和器官损伤。在成年发病的 SLE 中,临床特征、发病机制、疾病进展和结局不仅在个体和年龄组之间存在差异,而且在不同种族之间也存在差异。然而,在儿童和青少年中,种族对疾病发病、表型和结局的影响尚未得到详细研究。在这项研究中,我们从不同种族背景(白种人、亚洲人、黑非洲/加勒比人)的儿科 SLE 患者中调查了临床和实验室特征,这些患者的数据来自一个全国性的 jSLE 患者队列(英国 jSLE 队列研究)。在英国的 jSLE 患者中,种族影响疾病的临床过程和结局。在诊断时,与白种人或亚洲人患者相比,黑非洲/加勒比人 jSLE 患者表现出更多的“经典”实验室和临床特征。黑非洲/加勒比 jSLE 患者更易发生肾脏受累,更常接受环磷酰胺和利妥昔单抗治疗。针对疾病表型和表型的种族特异性发病机制的研究对于改善我们对 jSLE 的病理生理学认识、诊断和治疗是必要的。

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