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.

Academic Medical Centre Amsterdam The Netherlands

Brigham and Women's Hospital and Boston VA Healthcare Boston Massachusetts

Brigham and Women's Hospital Harvard Medical School Boston Massachusetts

Central Manchester University Hospitals NHS Foundation Trust University of Manchester Manchester UK

Charles University Prague Czech Republic

Duke University Durham North Carolina

Great Ormond Street Hospital for Children NHS Trust London UK

Hospital General de Occidente Secretaría de Salud and University of Guadalajara Guadalajara Mexico

Institute of Rheumatology Warsaw Poland

IWK Health Centre and Dalhousie University Halifax Nova Scotia Canada

Karolinska Institutet Stockholm Sweden

Karolinska University Hospital and Karolinska Institutet Stockholm Sweden

King's College Hospital NHS Foundation Trust London UK

Mayo Clinic College of Medicine Rochester Minnesota

Mayo Clinic College of Medicine Rochester Minnesota and University of Alabama and Birmingham VA Medical Center Birmingham Alabama

National Institute of Arthritis and Musculoskeletal and Skin Diseases NIH Bethesda Maryland

National Institute of Environmental Health Sciences NIH Bethesda Maryland

National Institute of Geriatrics Rheumatology and Rehabilitation Warsaw Poland

Oregon State University Corvallis

Oslo University Hospital Rikshospitalet Oslo Norway

People's Hospital of Beijing University Beijing China

Philadelphia VA Medical Center and Hospital of the University of Pennsylvania Philadelphia

Seoul National University College of Medicine Seoul Republic of Korea

Tokyo Medical and Dental University Tokyo Japan

University of Debrecen Debrecen Hungary

University of Kansas Medical Center Kansas City

University of Liverpool Liverpool UK

University of Pittsburgh School of Medicine Pittsburgh Pennsylvania

University of Toronto and The Hospital for Sick Children Toronto Ontario Canada

University of Tsukuba Tsukuba Japan

Vall d'Hebron General Hospital Barcelona Spain

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Nat Rev Rheumatol. 2017 Nov 21;13(12):694. doi: 10.1038/nrrheum.2017.192. PubMed

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Arthritis Rheumatol. 2018 Jun;70(6):976. doi: 10.1002/art.40473. PubMed

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Arthritis Rheumatol. 2018 Jun;70(6):975-976. doi: 10.1002/art.40478. PubMed

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Ann Rheum Dis. 2019 Apr;78(4):e30. doi: 10.1136/annrheumdis-2018-213341. PubMed

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Arthritis Rheumatol. 2018 Sep;70(9):1532. doi: 10.1002/art.40691. PubMed

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Effect of an 8-Week Tailored Physiotherapy Program on Sexual Health in Women with Scleroderma and Myositis: A Controlled Pilot Study

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The EuroMyositis registry: an international collaborative tool to facilitate myositis research