Concha J S S, Pena S, Gaffney R G, Patel B, Tarazi M, Kushner C J, Merola J F, Fiorentino D, Dutz J P, Goodfield M, Nyberg F, Volc-Platzer B, Fujimoto M, Ang C C, Werth V P
Michael J. Crescenz VAMC, Philadelphia, PA, U.S.A.
Department of Dermatology, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA, U.S.A.
Br J Dermatol. 2020 Feb;182(2):410-417. doi: 10.1111/bjd.18096. Epub 2019 Sep 29.
The European League Against Rheumatism/American College of Rheumatology classification criteria for inflammatory myopathies are able to classify patients with skin-predominant dermatomyositis (DM). However, approximately 25% of patients with skin-predominant DM do not meet two of the three hallmark skin signs and fail to meet the criteria.
To develop a set of skin-focused classification criteria that will distinguish cutaneous DM from mimickers and allow a more inclusive definition of skin-predominant disease.
An extensive literature review was done to generate items for the Delphi process. Items were grouped into categories of distribution, morphology, symptoms, antibodies, histology and contextual factors. Using REDCap™, participants rated these items in terms of appropriateness and distinguishing ability from mimickers. The relevance score ranged from 1 to 100, and the median score determined a rank-ordered list. A prespecified median score cut-off was decided by the steering committee and the participants. There was a pre-Delphi and two rounds of actual Delphi.
There were 50 participating dermatologists and rheumatologists from North America, South America, Europe and Asia. After a cut-off score of 70 during the first round, 37 of the initial 54 items were retained and carried over to the next round. The cut-off was raised to 80 during round two and a list of 25 items was generated.
This project is a key step in the development of prospectively validated classification criteria that will create a more inclusive population of patients with DM for clinical research. What's already known about this topic? Proper classification of patients with skin-predominant dermatomyositis (DM) is indispensable in the appropriate conduct of clinical/translational research in the field. The only validated European League Against Rheumatism/American College of Rheumatology criteria for idiopathic inflammatory myopathies are able to classify skin-predominant DM. However, a quarter of amyopathic patients still fail the criteria and does not meet the disease classification. What does this study add? A list of 25 potential criteria divided into categories of distribution, morphology, symptomatology, pathology and contextual factors has been generated after several rounds of consensus exercise among experts in the field of DM. This Delphi project is a prerequisite to the development of a validated classification criteria set for skin-predominant DM.
欧洲抗风湿病联盟/美国风湿病学会制定的炎性肌病分类标准能够对以皮肤为主的皮肌炎（DM）患者进行分类。然而，约25%以皮肤为主的DM患者不符合三项标志性皮肤体征中的两项，因而无法满足该标准。
制定一套以皮肤为重点的分类标准，以区分皮肤型DM与其他类似疾病，并对以皮肤为主的疾病给出更具包容性的定义。
进行广泛的文献综述以生成用于德尔菲法的条目。条目被分为分布、形态、症状、抗体、组织学和背景因素等类别。使用REDCap™，参与者对这些条目在适用性以及与其他类似疾病的区分能力方面进行评分。相关性评分范围为1至100，中位数评分确定了一个按顺序排列的列表。指导委员会和参与者确定了一个预先设定的中位数评分临界值。进行了一轮预德尔菲法和两轮实际的德尔菲法。
有来自北美、南美、欧洲和亚洲的50名皮肤科医生和风湿病学家参与。在第一轮中，临界值设定为70分后，最初的54个条目中有37个被保留并进入下一轮。第二轮临界值提高到80分，并生成了一份包含25个条目的列表。
该项目是制定经过前瞻性验证的分类标准的关键一步，这将为临床研究创建一个更具包容性的皮肌炎患者群体。关于该主题已知的信息有哪些？对以皮肤为主的皮肌炎（DM）患者进行正确分类对于该领域临床/转化研究的恰当开展必不可少。唯一经过验证的欧洲抗风湿病联盟/美国风湿病学会特发性炎性肌病标准能够对以皮肤为主的DM进行分类。然而，四分之一的无肌病患者仍不符合该标准，无法进行疾病分类。这项研究增加了什么？经过DM领域专家几轮共识性练习后，生成了一份包含25项潜在标准的列表，这些标准分为分布、形态、症状学、病理学和背景因素等类别。这个德尔菲项目是为以皮肤为主的DM制定经过验证的分类标准集的先决条件。
