Background

Existing classification systems for idiopathic inflammatory myopathies (IIMs) fail to permit classification and/or diagnosis of amyopathic dermatomyositis (ADM) in patients.

Objective

In light of the new European League Against Rheumatism/American College of Rheumatology (EULAR/ACR) classification criteria for IIM, we evaluated the likelihood of the skin variables included in the EULAR/ACR criteria (Gottron's sign, Gottron's papules, and heliotrope rash) to give a high probability of classifying patients with ADM.

Methods

This retrospective study evaluated 211 adult patients with dermatomyositis at the University of Pennsylvania. The EULAR/ACR criteria were used to determine the probability of classification for patients with ADM.

Results

Of patients with ADM, 73.7% would be classified as having a reasonable probability of dermatomyositis on the new EULAR/ACR criteria and 26.3% would not meet the suggested 55% minimum probability cutoff to be classified as having it on the basis of the EULAR/ACR criteria.

Limitations

This study was conducted with a retrospective design at a tertiary academic medical center.

Conclusions

The 3 skin variables included in the EULAR/ACR classification criteria for IIM improve on previous criteria but miss classifying some patients with ADM. It is important to consider additional variables such as skin biopsy results to encompass more of these patients and prevent the inclusion of any skin diseases mimicking ADM.

中文翻译：

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EULAR / ACR分类标准对皮肌炎对肌肌病的适用性

背景

现有的用于特发性炎性肌病（IIM）的分类系统无法对患者的肌病性皮肌炎（ADM）进行分类和/或诊断。

客观的

根据针对IIM的新的欧洲风湿病联盟/美国风湿病学会（EULAR / ACR）分类标准，我们评估了EULAR / ACR标准（Gottron体征，Gottron丘疹性丘疹和天疱疮皮疹）中包含的皮肤变量的可能性可以对ADM患者进行分类。

方法

这项回顾性研究评估了宾夕法尼亚大学的211名成人皮肌炎患者。使用EULAR / ACR标准来确定ADM患者分类的可能性。

结果

在新的EULAR / ACR标准中，有ADM的患者中，有73.7％被归类为具有合理的皮肌炎可能性，而在EULAR / ACR标准的基础上，有26.3％的患者不符合建议的55％最小概率界限。 ACR标准。

局限性

这项研究是在三级学术医学中心进行的回顾性设计。

结论

IIM的EULAR / ACR分类标准中包括的3种皮肤变量在以前的标准上有所改善，但未对一些ADM患者进行分类。重要的是要考虑其他变量，例如皮肤活检结果，以涵盖更多的此类患者，并防止包括任何模仿ADM的皮肤疾病。