【摘要】 作为特发性炎症性肌病(ⅡM)和幼年ⅡM(JⅡM)类别之一的幼年皮肌炎(JDM)是一种罕见、可危及生命的全身性自身免疫疾病,代表了儿童期主要的肌病类型,JDM、JⅡM不仅影响肌肉和皮肤,其他系统器官(肺、心脏和肠道)也可受累,但有可能被低估。最新机制研究和大型合作研究网络极大促进了JDM、ⅡM及其亚型的识别、标准化评估和治疗进展。也定义了一些免疫风险因素和JDM、ⅡM病因机制的潜在途径。在新标准中,应用标准化评估、肌炎相关性和肌炎特异性抗体细分亚组和疾病的严重度,显示了这些因素的价值。这些亮点深入验证后,将有助于疾病活动和损害评估的标准化,促进精准治疗制定。这是儿科风湿病医生需要关注的方面。更多还原

【Abstract】 Juvenile dermatomyositis(JDM) is a rare,life-threatening systemic autoimmune disease,which is one of the categories of idiopathic inflammatory myopathy(IIM) and juvenile IIM(JIIM).JDM/JIIM affects not only muscles and skin,but also other system organs(lungs,heart and intestines),which may be underestimated.The studies on the latest pathogenesis and large collaborative research networks have greatly facilitated the identification of the subtypes,standardized assessment and treatment of JDM/IIM.Some immune risk factors have been defined,and potential pathways may be found in the pathogenesis of JDM/IIM.In the new criteria,the use of standardized assessments,myositis-associated and myositis-specific antibody subgroups and disease severity revealed the value of these factors.These validation will contribute to the standardization of assessing disease activity and damage,and facilitate the development of targeted therapies.These are the aspects for pediatric rheumatologists to pay attention to.更多还原