青少年系统性红斑狼疮疾病负担的纵向评估:活动和损伤评分的多中心研究。

IF 1.9 4区 医学 Q3 RHEUMATOLOGY
Lupus Pub Date : 2025-09-29 DOI:10.1177/09612033251386091
Duygu Aydın, Eray Tunce, Gülşah Kavrul Kayaalp, Hande Ilgaz Tüzen, Dorukcan Alkan, Görkem Oğuz, Yasin Karali, Sıla Atamyıldız Uçar, Gülçin Otar Yener, Hatice Kübra Dursun, Tolga Kasap, Mustafa Çakan, Ferhat Demir, Hatice Adıgüzel Dündar, Burcu Bozkaya Yücel, Figen Çakmak, Nihal Şahin, Selçuk Yüksel, Kübra Öztürk, Sara Sebnem Kilic, Serkan Türkuçar, Semanur Özdel, Belde Kasap Demir, Nuray Aktay Ayaz, Betül Sözeri, Hafize Emine Sönmez
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引用次数: 0

摘要

青少年系统性红斑狼疮(jSLE)是一种罕见的儿童风湿性疾病,其特征是全身性炎症,可导致器官损害。与成人相比,儿童的病情往往更为严重。疾病活动和治疗都可能导致暂时或永久性损伤。目的评价与jSLE患者损伤发生相关的危险因素。方法该多中心回顾性研究纳入jSLE患者,随访时间至少12个月。低剂量皮质类固醇治疗定义为强的松龙0.01-0.03 mg/kg/天(最大7.5 mg/天)。通过将类固醇总摄入量除以365.25乘以随访年数,计算出年度累积类固醇剂量。收集的数据包括首次和最终就诊时的SLEDAI和SDI评分、实验室参数和耀斑特征。结果共纳入17个中心158例患者,其中女性占86.7%。诊断时的中位年龄为13.8岁,中位随访时间为35个月。14例患者在诊断时出现器官损伤,23例患者在最后一次就诊时出现器官损伤。损伤类型包括蛋白尿、认知功能障碍(各3.2%),以及其他如白内障、糜烂性关节炎、缺血性坏死、视神经萎缩和椎体塌陷。损伤患者在两个时间点的SLEDAI评分均显著较高,向低剂量类固醇的过渡延迟,达到狼疮低疾病活动状态(LLDAS)的比率较低(p = 0.006)。结论持续的疾病活动和延迟的控制是jSLE器官损害的主要原因。早期和持续的疾病抑制对预防长期并发症至关重要。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Longitudinal assessment of disease burden in juvenile systemic lupus erythematosus: A multicenter study of activity and damage scores.

IntroductionJuvenile Systemic Lupus Erythematosus (jSLE) is a rare pediatric rheumatic disease characterized by systemic inflammation that can lead to organ damage. Compared to adults, it often has a more severe course in children. Both disease activity and treatments may result in temporary or permanent damage.ObjectivesTo evaluate risk factors associated with damage occurrence in patients with jSLE.MethodsThis multicenter, retrospective study included patients with jSLE followed for at least 12 months. Low-dose corticosteroid therapy was defined as prednisolone 0.01-0.03 mg/kg/day (max 7.5 mg/day). The annual cumulative steroid dose was calculated by dividing the total steroid intake by 365.25 times the number of follow-up years. Collected data included SLEDAI and SDI scores at initial and final visits, laboratory parameters, and flare characteristics.ResultsA total of 158 patients (86.7% female) from 17 centers were included. Median age at diagnosis was 13.8 years, with a median follow-up of 35 months. Organ damage was present in 14 patients at diagnosis and in 23 at final visit. Damage types included proteinuria, cognitive dysfunction (each 3.2%), and others such as cataracts, erosive arthritis, avascular necrosis, optic atrophy, and vertebral collapse. Patients with damage had significantly higher SLEDAI scores at both time points, delayed transition to low-dose steroids, and a lower rate of achieving Lupus Low Disease Activity State (LLDAS) (p = .006).ConclusionPersistent disease activity and delayed control are major contributors to organ damage in jSLE. Early and sustained disease suppression is critical to prevent long-term complications.

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来源期刊
Lupus
Lupus 医学-风湿病学
CiteScore
4.20
自引率
11.50%
发文量
225
审稿时长
1 months
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…
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