一组罗马尼亚幼年皮肌炎患儿完全临床应答和缓解的相关因素

IF 2.4 4区 医学 Q2 RHEUMATOLOGY JCR: Journal of Clinical Rheumatology Pub Date : 2024-09-01 Epub Date: 2024-05-16 DOI:10.1097/RHU.0000000000002092
Andreea Ioan, Oana M Farkas, Alexis V Cochino
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引用次数: 0

摘要

目的描述罗马尼亚幼年皮肌炎(JDM)患者队列,并确定与疾病严重程度、完全临床反应和持续缓解相关的因素:我们回顾性审查了30名幼年皮肌炎患者在2013年至2022年期间的数据。非活动性疾病状态的定义是无活动性皮疹、肌无力或肌酶升高。完全临床应答意味着在服药期间连续6个月保持非活动状态,并且在治疗后至少连续6个月非活动状态缓解。通过双变量相关性分析(皮尔逊系数)和单变量生存分析(卡普兰-梅尔分析),得出了完全临床应答时间和缓解时间的相关因素和预测因素:整个组群的完全临床应答时间和缓解时间的中位数分别为 30.5 个月(2.5 年)和 48.5 个月(4.04 年)。9名患者(30%)病程严重,21名患者(70%)病程为轻度/中度。钙化的存在、停用皮质类固醇激素的时间、前18个月的治疗升级史以及硫唑嘌呤或生物DMARDs治疗与临床缓解时间的延长密切相关(Pearson's > 0.5,P < 0.05)。7名患者(23%)获得了缓解,在随后19个月的中位随访期间无一复发。
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Factors Associated With Complete Clinical Response and Remission in a Cohort of Romanian Children With Juvenile Dermatomyositis.

Objectives: To describe a Romanian cohort of patients with juvenile dermatomyositis (JDM) and to identify factors associated with disease severity, complete clinical response, and sustained remission.

Methods: We retrospectively reviewed data from 30 JDM patients from 2013 to 2022. The inactive disease state was defined as no active skin rash, muscle weakness, or elevated muscle enzymes. A complete clinical response implied a status of inactive disease maintained for six consecutive months while on medication and remission of inactive disease for at least six consecutive months after treatment. Association factors and predictors of time to complete clinical response and time to remission emerged from bivariate correlation (Pearson's coefficient) and univariate survival analysis (Kaplan-Meier analysis).

Results: The median times to complete clinical response and time to remission for the entire cohort were 30.5 months (2.5 years) and 48.5 months (4.04 years), respectively. Nine patients (30%) had a severe disease course, while twenty-one patients (70%) had a mild/moderate course. The presence of calcinosis, time to corticosteroid discontinuation, history of treatment escalation in the first 18 months, and treatment with azathioprine or biologic DMARDs were strongly associated with a longer time to clinical remission (Pearson's > 0.5, p < 0.05). Seven patients (23%) achieved remission, and none of them relapsed during the subsequent median follow-up of 19 months.

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来源期刊
CiteScore
3.50
自引率
2.90%
发文量
228
审稿时长
4-8 weeks
期刊介绍: JCR: Journal of Clinical Rheumatology the peer-reviewed, bimonthly journal that rheumatologists asked for. Each issue contains practical information on patient care in a clinically oriented, easy-to-read format. Our commitment is to timely, relevant coverage of the topics and issues shaping current practice. We pack each issue with original articles, case reports, reviews, brief reports, expert commentary, letters to the editor, and more. This is where you''ll find the answers to tough patient management issues as well as the latest information about technological advances affecting your practice.
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