Validation of the 2018 (New) ENMC Classification Criteria for Dermatomyositis in Chinese Patients with Idiopathic Inflammatory Myopathies.

Abstract

Objectives: To validate the 2018 European Neuromuscular Centre classification (ENMC) criteria, compare its performance to the 1975 Bohan & Peter (B&P) and 2017 European League Against Rheumatism/American College of Rheumatology (EULAR/ACR) criteria for dermatomyositis (DM), and describe characteristics of different myositis-specific autoantibody (MSA)-positive patients defined by the ENMC-DM criteria.

Methods: Medical records and data on MSAs and muscle biopsies were retrospectively obtained from 1370 Chinese patients with idiopathic inflammatory myopathy (IIM) between 2008 and 2020. Patients were diagnosed with DM by at least two rheumatologists and classified according to the ENMC-DM, EULAR/ACR, and B&P criteria.

Results: Of the 1370 patients, 857, 671, 693, and 913 were diagnosed with DM using the specialists' gold standard, ENMC-DM, EULAR/ACR, and B&P criteria, respectively. Significant between-group differences were observed in the clinical symptoms, serum creatine kinase levels, and MSAs (P < 0.05). Based on muscle biopsy data, the B&P criteria had the highest sensitivity (94%) but lowest specificity (65%). Without muscle biopsy data, the ENMC-DM criteria had the highest specificity (92%) but lowest sensitivity (61%). The sensitivity and specificity of the EULAR/ACR criteria were intermediate (72% and 86%, respectively) regardless of muscle biopsy data availability. With MSA data, the sensitivity and specificity of the ENMC-DM criteria were 73% and 91% and increased to 76% and 97%, respectively, with both muscle biopsy and MSA data.

Conclusions: The ENMC-DM criteria had higher specificity than the other criteria, especially in the absence of muscle biopsy data. Sensitivity and specificity improved when both muscle biopsy and MSA data were available. Key Points • Idiopathic inflammatory myopathy presents diagnostic challenges due to its variable features and dermatomyositis has distinct subtypes based on myositis-specific autoantibodies (MSAs) with unique clinical phenotypes. • This study validates the ENMC-DM criteria in Chinese patients and provides a comprehensive comparison with the B&P and EULAR/ACR criteria. • It demonstrates that the new ENMC-DM criteria exhibit higher specificity, especially noteworthy in cases without muscle biopsy, and the study further highlights the improved sensitivity and specificity when combining muscle biopsy and MSAs, offering a refined approach for accurate DM classification.