Watson Louise, Leone Valentina, Pilkington Clarissa, Tullus Kjell, Rangaraj Satyapal, McDonagh Janet E, Gardner-Medwin Janet, Wilkinson Nick, Riley Phil, Tizard Jane, Armon Kate, Sinha Manish D, Ioannou Yiannis, Archer Neil, Bailey Kathryn, Davidson Joyce, Baildam Eileen M, Cleary Gavin, McCann Liza J, Beresford Michael W
University of Liverpool and Alder Hey Children's NHS Foundation Trust Hospital, Liverpool, UK.
Arthritis Rheum. 2012 Jul;64(7):2356-65. doi: 10.1002/art.34410.
The UK Juvenile-Onset Systemic Lupus Erythematosus (JSLE) Cohort Study is a multicenter collaborative network established with the aim of improving the understanding of juvenile SLE. The present study was undertaken to describe the clinical manifestations and disease course in patients with juvenile SLE from this large, national inception cohort.
Detailed data on clinical phenotype were collected at baseline and at regular clinic reviews and annual followup assessments in 232 patients from 14 centers across the UK over 4.5 years. Patients with SLE were identified according to the American College of Rheumatology (ACR) SLE classification criteria. The present cohort comprised children with juvenile SLE (n=198) whose diagnosis fulfilled ≥4 of the ACR criteria for SLE.
Among patients with juvenile SLE, the female:male sex distribution was 5.6:1 and the median age at diagnosis was 12.6 years (interquartile range 10.4-14.5 years). Male patients were younger than female patients (P<0.01). Standardized ethnicity data demonstrated a greater risk of juvenile SLE in non-Caucasian UK patients (P<0.05). Scores on the pediatric adaptation of the 2004 British Isles Lupus Assessment Group disease activity index demonstrated significantly increased frequencies of musculoskeletal (82%), renal (80%), hematologic (91%), immunologic (54%), and neurologic (26%) involvement among the patients over time. A large proportion of the patients (93%) were taking steroids and 24% of the patients required treatment with cyclophosphamide. Disease damage was common, with 28% of the patients having a Systemic Lupus International Collaborating Clinics/ACR damage score of ≥1.
The data on these patients from the UK JSLE Cohort Study, comprising one of the largest national inception cohorts of patients with juvenile SLE to date, indicate that severe organ involvement and significant disease activity are primary characteristics in children with juvenile SLE. In addition, accumulation of disease-associated damage could be seen.
英国青少年起病的系统性红斑狼疮（JSLE）队列研究是一个多中心合作网络，旨在增进对青少年系统性红斑狼疮的了解。本研究旨在描述来自这个大型全国性起始队列的青少年系统性红斑狼疮患者的临床表现和疾病进程。
在4.5年的时间里，从英国14个中心的232名患者的基线、定期门诊复查和年度随访评估中收集了详细的临床表型数据。根据美国风湿病学会（ACR）系统性红斑狼疮分类标准确定系统性红斑狼疮患者。本队列包括青少年系统性红斑狼疮患儿（n = 198），其诊断符合≥4条ACR系统性红斑狼疮标准。
在青少年系统性红斑狼疮患者中，女性与男性的性别分布为5.6:1，诊断时的中位年龄为12.6岁（四分位间距10.4 - 14.5岁）。男性患者比女性患者年轻（P < 0.01）。标准化种族数据显示，非白种英国患者患青少年系统性红斑狼疮的风险更高（P < 0.05）。2004年英伦诸岛狼疮评估组疾病活动指数儿科版评分显示，随着时间推移，患者中肌肉骨骼（82%）、肾脏（80%）、血液学（91%）、免疫学（54%）和神经学（26%）受累的频率显著增加。很大一部分患者（93%）正在服用类固醇，24%的患者需要环磷酰胺治疗。疾病损害很常见，28%的患者系统性红斑狼疮国际协作临床/ACR损害评分为≥1。
来自英国JSLE队列研究的这些患者的数据，是迄今为止最大的全国性青少年系统性红斑狼疮起始队列之一，表明严重器官受累和显著的疾病活动是青少年系统性红斑狼疮患儿的主要特征。此外，可见与疾病相关的损害积累。
