2017 European League Against Rheumatism/American College of Rheumatology Classification Criteria for Adult and Juvenile Idiopathic Inflammatory Myopathies and Their Major Subgroups.

Journal Article (Journal Article)

OBJECTIVE: To develop and validate new classification criteria for adult and juvenile idiopathic inflammatory myopathies (IIM) and their major subgroups. METHODS: Candidate variables were assembled from published criteria and expert opinion using consensus methodology. Data were collected from 47 rheumatology, dermatology, neurology, and pediatric clinics worldwide. Several statistical methods were utilized to derive the classification criteria. RESULTS: Based on data from 976 IIM patients (74% adults; 26% children) and 624 non-IIM patients with mimicking conditions (82% adults; 18% children), new criteria were derived. Each item is assigned a weighted score. The total score corresponds to a probability of having IIM. Subclassification is performed using a classification tree. A probability cutoff of 55%, corresponding to a score of 5.5 (6.7 with muscle biopsy) "probable IIM," had best sensitivity/specificity (87%/82% without biopsies, 93%/88% with biopsies) and is recommended as a minimum to classify a patient as having IIM. A probability of ≥90%, corresponding to a score of ≥7.5 (≥8.7 with muscle biopsy), corresponds to "definite IIM." A probability of <50%, corresponding to a score of <5.3 (<6.5 with muscle biopsy), rules out IIM, leaving a probability of ≥50-<55% as "possible IIM." CONCLUSION: The European League Against Rheumatism/American College of Rheumatology (EULAR/ACR) classification criteria for IIM have been endorsed by international rheumatology, dermatology, neurology, and pediatric groups. They employ easily accessible and operationally defined elements, and have been partially validated. They allow classification of "definite," "probable," and "possible" IIM, in addition to the major subgroups of IIM, including juvenile IIM. They generally perform better than existing criteria.

Full Text

Duke Authors

Cited Authors

  • Lundberg, IE; Tjärnlund, A; Bottai, M; Werth, VP; Pilkington, C; de Visser, M; Alfredsson, L; Amato, AA; Barohn, RJ; Liang, MH; Singh, JA; Aggarwal, R; Arnardottir, S; Chinoy, H; Cooper, RG; Dankó, K; Dimachkie, MM; Feldman, BM; Garcia-De La Torre, I; Gordon, P; Hayashi, T; Katz, JD; Kohsaka, H; Lachenbruch, PA; Lang, BA; Li, Y; Oddis, CV; Olesinska, M; Reed, AM; Rutkowska-Sak, L; Sanner, H; Selva-O'Callaghan, A; Song, Y-W; Vencovsky, J; Ytterberg, SR; Miller, FW; Rider, LG; International Myositis Classification Criteria Project Consortium, the Euromyositis Register, and the Juvenile Dermatomyositis Cohort Biomarker Study and Repository (UK and Ireland),

Published Date

  • December 2017

Published In

Volume / Issue

  • 69 / 12

Start / End Page

  • 2271 - 2282

PubMed ID

  • 29106061

Pubmed Central ID

  • PMC5846474

Electronic International Standard Serial Number (EISSN)

  • 2326-5205

Digital Object Identifier (DOI)

  • 10.1002/art.40320


  • eng

Conference Location

  • United States