2017 European League Against Rheumatism/American College of Rheumatology classification criteria for adult and juvenile idiopathic inflammatory myopathies and their major subgroups

Ingrid E Lundberg, Anna Tjärnlund, Matteo Bottai, Victoria P Werth, Clarissa Pilkington, Marianne de Visser, Lars Alfredsson, Anthony A Amato, Richard J Barohn, Matthew H. Liang, Jasvinder A. Singh, Rohit Aggarwal, Snjolaug Arnardottir, Hector Chinoy, Robert G. Cooper, Katalin Dankó, Mazen Dimachkie, Brian M. Feldman, Ignacio Garcia-De La Torre, Patrick GordonTaichi Hayashi, James D Katz, Hitoshi Kohsaka, Peter A. Lachenbruch, Bianca A Lang, Yuhui Li, Chester V. Oddis, Marzena Olesinska, Ann M. Reed, Lidia Rutkowska-Sak, Helga Sanner, Albert Selva-O'Callaghan, Yeong-Wook Song, Jiri Vencovsky, Steven R. Ytterberg, Frederick W. Miller, Lisa G. Rider, , Annet van Royen

Research output: Contribution to journalArticleAcademicpeer-review

Abstract

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 paediatric clinics worldwide. Several statistical methods were used 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 cut-off 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 to <55% as 'possible IIM'.

CONCLUSIONS: The European League Against Rheumatism/American College of Rheumatology (EULAR/ACR) classification criteria for IIM have been endorsed by international rheumatology, dermatology, neurology and paediatric 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.

Original languageEnglish
Pages (from-to)1955-1964
Number of pages10
JournalAnnals of the Rheumatic Diseases
Volume76
Issue number12
DOIs
Publication statusPublished - Dec 2017

Keywords

  • Adult
  • Biopsy
  • Child
  • Consensus
  • Consensus Development Conference
  • Diagnosis, Differential
  • Europe
  • Humans
  • Muscle, Skeletal
  • Myositis
  • Practice Guideline
  • Probability
  • Reference Values
  • Rheumatology
  • Sensitivity and Specificity
  • Societies, Medical
  • United States
  • Validation Studies

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