Department of Rheumatology, Great Ormond Street Hospital, and Juvenile Dermatomyositis Research Centre, Institute of Child Health, London, UK.
Curr Opin Rheumatol. 2009 Nov;21(6):575-80. doi: 10.1097/BOR.0b013e328331927e.
Over the last few years, there have been several collaborative efforts focusing on how to assess the many aspects of juvenile dermatomyositis (JDM). It has become apparent that skin manifestations are an important part of the disease process that need to be evaluated, treated and controlled. Other extramuscular manifestations, such as calcinosis, are particularly difficult to treat, and studies are aimed at trying to elucidate predictors.
Larger cohort studies have enabled work on predictors of disease course and severity to be carried out. These include new autoantibodies in JDM (p140, which appears to have an association with calcinosis and p155 with lipodystrophy), cytokine polymorphisms, which appear to be risk factors for developing JDM or for developing complications such as calcinosis, and the clinical findings of persistent nailfold capillary changes that are associated with a chronic disease course. Tools for more detailed assessment of skin disease have been developed and evaluated.
Improved collection of standardized clinical data regarding extramuscular manifestations of JDM should allow researchers to continue elucidating the prognostic factors in this rare disease and assist multicentred trials in the evaluation of different treatment options.
在过去的几年中，已经有几项合作研究集中在如何评估幼年皮肌炎（JDM）的多个方面。很明显，皮肤表现是疾病过程的一个重要组成部分，需要进行评估、治疗和控制。其他肌肉外表现，如钙质沉着症，特别难以治疗，研究旨在试图阐明预测因素。
更大的队列研究使对疾病过程和严重程度的预测因素的研究成为可能。这些包括 JDM 中的新自身抗体（p140，似乎与钙质沉着症有关，p155 与脂肪营养不良有关）、细胞因子多态性，这些似乎是发生 JDM 或发生钙质沉着症等并发症的危险因素，以及与慢性疾病过程相关的持续甲襞毛细血管变化的临床发现。已经开发和评估了用于更详细评估皮肤疾病的工具。
关于 JDM 肌肉外表现的更标准化临床数据的收集，应能使研究人员继续阐明这种罕见疾病的预后因素，并协助多中心试验评估不同的治疗选择。
