Lujayn Akbar, Majd S Khader, Rawan Elshaer, Samar Jaber, Abrar K Khader, Sameeha Abdulwali, Raghad Alhuthil, Sulaiman M Al-Mayouf
{"title":"Prepubertal to adolescent lupus: Age-related variations in clinical, laboratory, and outcome profiles.","authors":"Lujayn Akbar, Majd S Khader, Rawan Elshaer, Samar Jaber, Abrar K Khader, Sameeha Abdulwali, Raghad Alhuthil, Sulaiman M Al-Mayouf","doi":"10.1177/09612033251357629","DOIUrl":null,"url":null,"abstract":"<p><p>BackgroundChildhood-onset systemic lupus erythematosus (cSLE) is a heterogeneous autoimmune disease with age-related variations. Older children often exhibit higher disease activity, whereas early-onset cSLE is associated with worse outcomes. However, most existing studies are retrospective and based on single-nation cohorts, yiedling inconsistent findings.ObjectiveTo investigate age-related variations in cSLE subgroups, namely prepubertal, peripubertal, and adolescent-onset cases.MethodsThis systematic review, registered in PROSPERO and conducted in accordance with PRISMA guidelines, searched PubMed, the Cochrane Library, and Web of Science for English-language studies pubslished between January 2000- February 2025. Eligible studies examined age-related variations in cSLE diagnosed before 18 years of age. Data were categorized into prepubertal, peri-pubertal, and adolescent-onset groups. Exclusion criteria included case reports, comments, editorials, viewpoint articles, conference abstracts, and incomplete studies. Four reviewers independently screened the articles, with discrepancies resolved by a fifth reviewer. Study quality was assessed using the NHLBI criteria.ResultsOf 16,313 studies screened, 13 met the inclusion criteria, comprising a total of 3920 cSLE cases. Among these, 464 (11.8%) were prepubertal, 1943 (49.6%) peripubertal, and 1513 (38.6%) adolescent-onset. The mean age at diagnosis was 5.8 ± 2.3, 9.4 ± 2.5, and 13.9 ± 1.1 years, respectively. The female-to-male ratio was highest in the adolescent-onset group (<i>p</i> = .002). Clinical manifestations showed no significant age-related differences; however, musculoskeletal involvement increased with age, while neuropsychiatric symptoms and fever decreased. Lymphopenia was more frequent in the adolescent-onset group (60% vs 25.6%, <i>p</i> = .016). Although disease activity and damage did not differ significantly across age groups, mortality was signficantly higher in the prepubertal group (16.5%) compared to the adolescent-onset group (2.9%) (<i>p</i> = .014).ConclusionThis review underscores both similarities and differences in cSLE across age groups. Prepubertal onset was associated with higher mortality, emphasizing the need for timely diagnosis and early intervention in this subgroup.</p>","PeriodicalId":18044,"journal":{"name":"Lupus","volume":" ","pages":"1049-1056"},"PeriodicalIF":1.9000,"publicationDate":"2025-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":null,"platform":"Semanticscholar","paperid":null,"PeriodicalName":"Lupus","FirstCategoryId":"3","ListUrlMain":"https://doi.org/10.1177/09612033251357629","RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"2025/7/11 0:00:00","PubModel":"Epub","JCR":"Q3","JCRName":"RHEUMATOLOGY","Score":null,"Total":0}
引用次数: 0
Abstract
BackgroundChildhood-onset systemic lupus erythematosus (cSLE) is a heterogeneous autoimmune disease with age-related variations. Older children often exhibit higher disease activity, whereas early-onset cSLE is associated with worse outcomes. However, most existing studies are retrospective and based on single-nation cohorts, yiedling inconsistent findings.ObjectiveTo investigate age-related variations in cSLE subgroups, namely prepubertal, peripubertal, and adolescent-onset cases.MethodsThis systematic review, registered in PROSPERO and conducted in accordance with PRISMA guidelines, searched PubMed, the Cochrane Library, and Web of Science for English-language studies pubslished between January 2000- February 2025. Eligible studies examined age-related variations in cSLE diagnosed before 18 years of age. Data were categorized into prepubertal, peri-pubertal, and adolescent-onset groups. Exclusion criteria included case reports, comments, editorials, viewpoint articles, conference abstracts, and incomplete studies. Four reviewers independently screened the articles, with discrepancies resolved by a fifth reviewer. Study quality was assessed using the NHLBI criteria.ResultsOf 16,313 studies screened, 13 met the inclusion criteria, comprising a total of 3920 cSLE cases. Among these, 464 (11.8%) were prepubertal, 1943 (49.6%) peripubertal, and 1513 (38.6%) adolescent-onset. The mean age at diagnosis was 5.8 ± 2.3, 9.4 ± 2.5, and 13.9 ± 1.1 years, respectively. The female-to-male ratio was highest in the adolescent-onset group (p = .002). Clinical manifestations showed no significant age-related differences; however, musculoskeletal involvement increased with age, while neuropsychiatric symptoms and fever decreased. Lymphopenia was more frequent in the adolescent-onset group (60% vs 25.6%, p = .016). Although disease activity and damage did not differ significantly across age groups, mortality was signficantly higher in the prepubertal group (16.5%) compared to the adolescent-onset group (2.9%) (p = .014).ConclusionThis review underscores both similarities and differences in cSLE across age groups. Prepubertal onset was associated with higher mortality, emphasizing the need for timely diagnosis and early intervention in this subgroup.
背景儿童期发病的系统性红斑狼疮(cSLE)是一种具有年龄相关变异的异质自身免疫性疾病。年龄较大的儿童通常表现出较高的疾病活动性,而早发性cSLE的预后较差。然而,大多数现有的研究都是回顾性的,并且基于单一国家的队列,结果不一致。目的探讨cSLE亚组(即青春期前、青春期周围和青春期发病病例)的年龄相关变化。方法本系统综述在PROSPERO上注册,按照PRISMA指南进行,检索PubMed、Cochrane图书馆和Web of Science,检索2000年1月至2025年2月间发表的英语研究。符合条件的研究检查了18岁之前诊断的cSLE的年龄相关变化。数据被分为青春期前、青春期前后和青春期发病组。排除标准包括病例报告、评论、社论、观点文章、会议摘要和不完整的研究。四名审稿人独立筛选了这些文章,其中的差异由第五名审稿人解决。采用NHLBI标准评估研究质量。结果在筛选的16,313项研究中,13项符合纳入标准,共计3920例cSLE病例。其中青春期前发病464例(11.8%),青春期周围发病1943例(49.6%),青春期发病1513例(38.6%)。平均诊断年龄分别为5.8±2.3岁、9.4±2.5岁和13.9±1.1岁。男女比例在青少年发病组最高(p = 0.002)。临床表现无明显年龄相关差异;然而,肌肉骨骼受累随着年龄的增长而增加,而神经精神症状和发烧则减少。淋巴细胞减少症在青少年发病组更为常见(60% vs 25.6%, p = 0.016)。尽管不同年龄组的疾病活动度和损害程度没有显著差异,但青春期前组的死亡率(16.5%)明显高于青春期发病组(2.9%)(p = 0.014)。结论本综述强调了不同年龄组cSLE的相似性和差异性。青春期前发病与较高的死亡率相关,强调在这一亚组中需要及时诊断和早期干预。
期刊介绍:
The only fully peer reviewed international journal devoted exclusively to lupus (and related disease) research. Lupus includes the most promising new clinical and laboratory-based studies from leading specialists in all lupus-related disciplines. Invaluable reading, with extended coverage, lupus-related disciplines include: Rheumatology, Dermatology, Immunology, Obstetrics, Psychiatry and Cardiovascular Research…