Performance of ENMC and EULAR/ACR classification systems applied to a single tertiary center cohort of dermatomyositis patients

Abstract

Abstract Background 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. Methods 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. Results 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. Conclusions 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.

Keywords

• Dermatomyositis
• ENMC
• EULAR/ACR
• Idiopathic inflammatory myositis
• Muscle biopsy