幼年皮肌炎不良预后的预测因素:我们知道些什么?叙述性综述。

IF 1.2 Q4 RHEUMATOLOGY
A Martins, S Ganhão, F Aguiar, M Rodrigues, I Brito
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引用次数: 0

摘要

目的:幼年皮肌炎(JDM)是一种罕见的慢性全身性炎症性疾病,临床病程多变。识别有可能发展成更严重疾病的患者非常重要。然而,根据现有文献,有关不良预后预测因素的数据还很缺乏。在疾病的早期阶段获得有关疾病进展和严重程度的临床和实验室风险因素的知识,有可能为 JDM 患者带来更好的长期预后:方法:我们进行了一项叙事性综述,旨在确定JDM患者不良预后的风险因素,如死亡、严重疾病、难治性疾病和功能障碍。共纳入 27 篇文章:结果:某些临床表现和免疫学特征似乎会使JDM患儿的预后恶化。对于所有儿科风湿病专家来说,识别这些风险因素至关重要,因为这样可以及早发现预后可能较差的患者。这些患者应接受更密切的随访和积极的个体化治疗,以降低其死亡率:我们需要开展更多的研究,不仅要找出更多预示不良后果的因素,还要找出针对这些患者的更有效的治疗方法。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Predictors of poor outcomes in juvenile dermatomyositis: what do we know? A narrative review.

Objective: Juvenile dermatomyositis (JDM) is a rare chronic systemic inflammatory disorder with a highly variable clinical course. It is important to identify the patients at risk of developing more severe disease. However, based on the existing literature, there is a lack of data regarding predictors of poor outcomes. Obtaining knowledge about clinical and laboratory risk factors for disease progression and severity at an earlier stage of the disease could potentially lead to a better long-term prognosis for patients with JDM.

Methods: A narrative review with the aim of identifying risk factors for poor outcomes in patients with JDM, such as death, severe disease, refractory disease, and functional impairment, was conducted. A total of 27 articles was included.

Results: Certain clinical manifestations and immunology features appear to worsen the prognosis in children with JDM. The recognition of these risk factors is essential for all pediatric rheumatologists as it allows the earlier identification of patients with potentially worse outcomes. These patients should receive closer follow-up and aggressive and individualized therapy in order to reduce their morbimortality.

Conclusions: Additional research is needed not only to identify more predictors of worse outcomes but also to identify more effective treatment approaches targeted toward these patients.

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来源期刊
Reumatismo
Reumatismo RHEUMATOLOGY-
CiteScore
2.10
自引率
7.10%
发文量
20
审稿时长
10 weeks
期刊介绍: Reumatismo is the official Journal of the Italian Society of Rheumatology (SIR). It publishes Abstracts and Proceedings of Italian Congresses and original papers concerning rheumatology. Reumatismo is published quarterly and is sent free of charge to the Members of the SIR who regularly pay the annual fee. Those who are not Members of the SIR as well as Corporations and Institutions may also subscribe to the Journal.
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