Zoske Jan, Schneider Udo, Siegert Elise, Kleefeld Felix, Preuße Corinna, Stenzel Werner, Hahn Katrin
Department of Periodontology, Oral Medicine and Oral Surgery, Charité - Universitätsmedizin Berlin, Corporate Member of Freie Universität Berlin, Humboldt-Universität Zu Berlin, and Berlin Institute of Health, 14197, Berlin, Germany.
Department of Rheumatology and Clinical Immunology, Charité - Universitätsmedizin Berlin, Charitéplatz 1, 10117, Berlin, Germany.
Neurol Res Pract. 2021 Nov 15;3(1):59. doi: 10.1186/s42466-021-00159-4.
There have been numerous classification systems to diagnose corresponding myositis subtypes and select appropriate therapeutic measures. However, the lack of a broad consensus on diagnostic criteria has led to clinical uncertainties. The objective of this study was to compare two commonly used dermatomyositis-classification systems regarding their clinical practicability and to point out their specific advantages and disadvantages.
This study included 30 patients diagnosed with dermatomyositis at the Charité university hospital, Berlin, Germany from 2010 to 2017. Patient files with complete data and defined historical classifications were enrolled and ENMC (2003) and EULAR/ACR (2017) criteria retrospectively applied.
According to the ENMC approach, 14 patients were classified as "definite" and 12 as "probable" dermatomyositis. One patient exhibited an "amyopathic dermatomyositis" and three a "DM without dermatitis". Regarding the criteria probability of the EULAR/ACR set, 16 patients had a "high", 13 a "medium" and one a "low probability". There was a significant difference (p = 0.004) between the subclasses of the ENMC in relation to the EULAR/ACR score. The agreement between the classification probabilities of "definite/high" (κ = 0.400) and "possible/medium" (κ = 0.324) was fair.
It is important to find a consensus among the medical disciplines involved and to establish a structured procedure. Future studies with newer approaches are warranted to conclusively decide which system to use for the physician.
已有众多分类系统用于诊断相应的肌炎亚型并选择合适的治疗措施。然而，诊断标准缺乏广泛共识导致了临床的不确定性。本研究的目的是比较两种常用的皮肌炎分类系统在临床实用性方面的差异，并指出它们各自的优缺点。
本研究纳入了2010年至2017年在德国柏林夏里特大学医院诊断为皮肌炎的30例患者。纳入具有完整数据和明确历史分类的患者档案，并回顾性应用欧洲神经肌肉中心（ENMC，2003年）和欧洲抗风湿病联盟/美国风湿病学会（EULAR/ACR，2017年）标准。
根据ENMC方法，14例患者被分类为“确诊”皮肌炎，12例为“可能”皮肌炎。1例患者表现为“无肌病性皮肌炎”，3例为“无皮疹的皮肌炎”。关于EULAR/ACR标准的概率，16例患者为“高”概率，13例为“中”概率，1例为“低”概率。ENMC的亚类与EULAR/ACR评分之间存在显著差异（p = 0.004）。“确诊/高”（κ = 0.400）和“可能/中”（κ = 0.324）分类概率之间的一致性一般。
在相关医学学科之间达成共识并建立结构化程序非常重要。有必要开展采用更新方法的未来研究，以便最终确定医生应使用哪种系统。
