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系统性幼年特发性关节炎的疾病演变:通过 JIRcohort 进行的国际观察性队列研究。

Disease evolution in systemic juvenile idiopathic arthritis: an international, observational cohort study through JIRcohort.

机构信息

Department of Woman, Mother, Child, Unit of Pediatric Immunology, Allergology and Rheumatology, Lausanne University Hospital and University of Lausanne, Lausanne, Switzerland.

Department of Pediatrics, Unit of Rheumatology and Nephrology, Mother and Child University Hospital A. Harouchi, CHU Ibn Rochd, Casablanca, Morocco.

出版信息

Pediatr Rheumatol Online J. 2023 Sep 7;21(1):96. doi: 10.1186/s12969-023-00886-9.

DOI:10.1186/s12969-023-00886-9
PMID:37679749
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC10485973/
Abstract

BACKGROUND

Systemic juvenile idiopathic arthritis (systemic JIA) is a severe disease with both systemic and joint inflammation. This study aims to identify predictors of disease evolution within the systemic JIA population enrolled in the Juvenile Inflammatory Rheumatism cohort (JIRcohort).

METHODS

Observational patient cohort study with 201 recruited children from 4 countries (3 European, 1 North Africa) from 2005 until 2019, using retrospectively (2005-2015) and prospectively (2015-2019) routine care collected data.

RESULTS

Sixty-five patients with complete follow-up data for 24 months after first diagnosis were classified as monophasic (n = 23), polyphasic (n = 6) or persistent group (n = 36) corresponding to their evolution (unique flare, recurrent flares, or persistent disease activity respectively). The patients of the persistent group were more likely to have an earlier disease onset, before the age of 6 (OR 2.57, 95%-CI 0.70-9.46), persistence of arthritis at 12-months post-diagnosis (OR 4.45, 95%-CI 0.58-34.20) and higher use of synthetic DMARD (sDMARD, OR 5.28, 95%-CI 1.39-20.01). Other variables like global assessment by physician and by patient and C Reactive Protein levels at 12-months post-diagnosis were assessed but without any predictive value after adjusting for confounding factors.

CONCLUSIONS

Our results suggest that the earlier disease onset, the persistence of arthritis throughout the first year of disease evolution and the need of sDMARD might predict a persistent disease course.

摘要

背景

全身性幼年特发性关节炎(systemic JIA)是一种严重的疾病,既有全身炎症又有关节炎症。本研究旨在确定纳入幼年炎症性风湿病队列(JIRcohort)的全身性 JIA 人群中疾病演变的预测因素。

方法

这是一项观察性患者队列研究,共纳入了来自 4 个国家(3 个欧洲国家,1 个北非国家)的 201 名儿童,这些儿童于 2005 年至 2019 年入组,使用回顾性(2005-2015 年)和前瞻性(2015-2019 年)收集的常规护理数据。

结果

65 名患者在首次诊断后 24 个月有完整的随访数据,根据其演变情况(唯一的发作、反复发作或持续的疾病活动)分为单相组(n=23)、多相组(n=6)或持续组(n=36)。持续组的患者更有可能在 6 岁之前(OR 2.57,95%CI 0.70-9.46)、诊断后 12 个月时(OR 4.45,95%CI 0.58-34.20)存在关节炎持续存在和(OR 5.28,95%CI 1.39-20.01)更高频率地使用合成 DMARD(sDMARD)。虽然还评估了医生和患者的整体评估以及诊断后 12 个月的 C 反应蛋白水平等其他变量,但在调整混杂因素后,这些变量没有任何预测价值。

结论

我们的研究结果表明,疾病早期发作、疾病演变的第一年中关节炎持续存在以及需要使用 sDMARD 可能预示着疾病的持续病程。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2f43/10485973/04fb5b9fe5b4/12969_2023_886_Fig1_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2f43/10485973/04fb5b9fe5b4/12969_2023_886_Fig1_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2f43/10485973/04fb5b9fe5b4/12969_2023_886_Fig1_HTML.jpg

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本文引用的文献

1
Toward New Classification Criteria for Juvenile Idiopathic Arthritis: First Steps, Pediatric Rheumatology International Trials Organization International Consensus.迈向幼年特发性关节炎新分类标准:初步步骤,儿科风湿病学国际试验组织国际共识。
J Rheumatol. 2019 Feb;46(2):190-197. doi: 10.3899/jrheum.180168. Epub 2018 Oct 1.
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Impact of biologics on disease course in systemic onset juvenile idiopathic arthritis.生物制剂对全身型幼年特发性关节炎病程的影响。
Clin Rheumatol. 2018 Dec;37(12):3263-3273. doi: 10.1007/s10067-018-4297-6. Epub 2018 Sep 20.
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Mortality rates are increased in patients with systemic juvenile idiopathic arthritis.
全身型幼年特发性关节炎患者的死亡率会升高。
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Arthritis Rheumatol. 2016 Dec;68(12):3023-3034. doi: 10.1002/art.39796.
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Current understanding of the pathophysiology of systemic juvenile idiopathic arthritis (sJIA) and target-directed therapeutic approaches.目前对全身型幼年特发性关节炎(sJIA)病理生理学的理解及靶向治疗方法。
Clin Immunol. 2015 Jul;159(1):72-83. doi: 10.1016/j.clim.2015.04.018. Epub 2015 May 6.
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Pathogenesis of adult-onset Still's disease: new insights from the juvenile counterpart.成人斯蒂尔病的发病机制:来自青少年型成人斯蒂尔病的新见解。
Immunol Res. 2015 Feb;61(1-2):53-62. doi: 10.1007/s12026-014-8561-9.
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Trends in prescription of biological agents and outcomes of juvenile idiopathic arthritis: results of the Dutch national Arthritis and Biologics in Children Register.生物制剂处方趋势和青少年特发性关节炎的结局:荷兰儿童关节炎和生物制剂登记处的结果。
Ann Rheum Dis. 2015 Jul;74(7):1379-86. doi: 10.1136/annrheumdis-2013-204641. Epub 2014 Mar 18.
8
Two randomized trials of canakinumab in systemic juvenile idiopathic arthritis.两项卡那单抗治疗全身型幼年特发性关节炎的随机临床试验。
N Engl J Med. 2012 Dec 20;367(25):2396-406. doi: 10.1056/NEJMoa1205099.
9
Randomized trial of tocilizumab in systemic juvenile idiopathic arthritis.托珠单抗治疗全身型幼年特发性关节炎的随机临床试验。
N Engl J Med. 2012 Dec 20;367(25):2385-95. doi: 10.1056/NEJMoa1112802.
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Disease-modifying antirheumatic drug use in the treatment of juvenile idiopathic arthritis: a cross-sectional analysis of the CARRA Registry.疾病修正抗风湿药物在幼年特发性关节炎治疗中的应用:CARRA 登记处的横断面分析。
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