Journal
ANNALS OF THE RHEUMATIC DISEASES
Volume 76, Issue 12, Pages 1955-1964Publisher
BMJ PUBLISHING GROUP
DOI: 10.1136/annrheumdis-2017-211468
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Funding
- European League Against Rheumatism (EULAR)
- American College of Rheumatology (ACR)
- Myositis Association (TMA)
- Intramural Research Program of the NIH, National Institute of Environmental Health Sciences
- European Science Foundation for the Euromyositis Register
- Swedish Research Council [K2014-52X-14045-14-3]
- Stockholm County Council
- Karolinska Institutet
- American Academy of Neurology (AAN)
- Childhood Arthritis and Rheumatology Research Alliance (CARRA),
- NIH-NIAMS
- Friends of CARRA
- Arthritis Foundation
- European Neuromuscular Centre (ENMC)
- International Myositis Assessment and Clinical Studies Group (IMACS)
- Muscle Study Group (MSG)
- Rheumatologic Dermatology Society (RDS)
- Pediatric RheumatologyEuropean Society (PReS) network for JDM
- Pediatric Rheumatology International Trials Organization (PRINTO)
- MRC [MR/N003322/1, MR/P020941/1] Funding Source: UKRI
- Medical Research Council [MR/N003322/1, MR/P020941/1] Funding Source: researchfish
- National Institute for Health Research [CL-2006-06-010] Funding Source: researchfish
- ReumaFonds [LLP-10] Funding Source: researchfish
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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.
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