文献简介

出版社:Springer International Publishing Switzerland

作  者:

编  号:

关键字:幼年型皮肌炎

年  份:2017   点击量:5

文献摘要 全文翻译

Abstract

The idiopathic inflammatory myopathies of childhood consist of a heterogeneous group of autoimmune diseases characterised by proximal muscle weakness and pathognomonic skin rashes. The overall prognosis of juvenile myositis has improved significantly over recent years, but the long-term outcome differs substantially from patient to patient, suggestive of distinct clinical phenotypes with variable responses to treatment. High doses of corticosteroids remain the cornerstone of therapy along with other immunosuppressant therapies depending on disease severity and response. The advent of biological drugs has revolutionised the management of various paediatric rheumatologic diseases, including inflammatory myopathies. There are few data from randomised controlled trials to guide management decisions; thus, several algorithms for the treatment of juvenile myositis have been developed using international expert opinion. The general treatment goals now include elimination of active disease and normalisation of physical function, so as to preserve normal growth and development, and to prevent long-term damage and deformities. This review summarises the newer and possible future therapies of juvenile inflammatory myopathies, including evidence supporting their efficacy and safety.

摘要

儿童特发性炎性肌病是一种异质群体的自身免疫性疾病,其特征为近端肌肉无力和出现特征性皮疹。近年来,幼年型肌炎整体预后显著提高,但长期预后因人而异,这表明不同临床表型对治疗有不同的反应。高剂量糖皮质激素仍是治疗基础,同时根据疾病严重度和治疗反应可联合其他免疫抑制剂治疗。生物制剂的出现彻底改变了各种小儿风湿性疾病的管理,包括炎性肌病。几乎没有指导疾病管理的随机对照试验数据;因此,根据国际专家意见提出了几种治疗幼年型肌炎的疗法。目前一般治疗目标包括消除活动性疾病,使身体功能正常化,从而维持正常生长发育,防止长期损伤和畸形。本文综述了幼年型炎性肌病最新疗法和未来可能的疗法,包括证明其疗效和安全性的证据。