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.

Clinical Immunology Doctoral Program in Clinical Sciences Graduate School of Comprehensive Human Sciences University of Tsukuba Tsukuba Japan

Connective Tissue Diseases Department National Institute of Geriatrics Rheumatology and Rehabilitation Warsaw Poland

Department of Clinical Neuroscience Karolinska Institutet Stockholm Sweden

Department of Dermatology Philadelphia VAMC and Hospital of the University of Pennsylvania Philadelphia Pennsylvania USA

Department of Immunology and Rheumatology Hospital General de Occidente Secretaría de Salud and University of Guadalajara Guadalajara Jalisco Mexico

Department of Internal Medicine Medical Research Center Clinical Research Institute Seoul National University College of Medicine Seoul Republic of Korea

Department of Neurology Academic Medical Centre Amsterdam The Netherlands

Department of Neurology Brigham and Women's Hospital Harvard Medical School Boston Massachusetts USA

Department of Neurology University of Kansas Medical Center Kansas City Kansas USA

Department of Pediatrics Duke University Durham North Carolina USA

Department of Public Health Oregon State University Corvallis Oregon USA

Department of Rheumatology and Immunology People's Hospital of Beijing University Beijing China

Department of Rheumatology Graduate School of Medical and Dental Sciences Tokyo Medical and Dental University Tokyo Japan

Department of Rheumatology Great Ormond Street Hospital for Children NHS Trust London UK

Department of Rheumatology Institute of Rheumatology 1st Faculty of Medicine Charles University Prague Czech Republic

Department of Rheumatology King's College Hospital NHS Foundation Trust London UK

Division of Immunology 3rd Department of Internal Medicine Medical and Health Science Center University of Debrecen Debrecen Hungary

Division of Rheumatology and Clinical Rheumatology University of Pittsburgh School of Medicine Pittsburgh Pennsylvania USA

Division of Rheumatology Department of Pediatrics IWK Health Centre and Dalhousie University Halifax Canada

Division of Rheumatology Department of Pediatrics University of Toronto and The Hospital for Sick Children Toronto Canada

Division of Rheumatology Immunology and Allergy Brigham and Women's Hospital and Section of Rheumatology Boston VA Healthcare Boston Massachusetts USA

Division of Rheumatology Mayo Clinic College of Medicine Rochester New York USA

Institute for Environmental Medicine Karolinska Institutet Stockholm Sweden

Mayo Clinic College of Medicine Rochester Minnesota USA

MRC ARUK Institute of Ageing and Chronic Disease Faculty of Health and Life Sciences University of Liverpool Liverpool UK

National Institute of Arthritis and Musculoskeletal and Skin Diseases National Institutes of Health US Department of Health and Human Services Bethesda Maryland USA

National Institute of Health Research Manchester Musculoskeletal Biomedical Research Unit Central Manchester University Hospitals NHS Foundation Trust University of Manchester Manchester UK

Paediatric Clinic of Rheumatology Institute of Rheumatology Warsaw Poland

Rheumatology Unit Department of Medicine Karolinska University Hospital Karolinska Institutet Stockholm Sweden

Section of Rheumatology Oslo University Hospital Rikshospitalet Oslo Norway

University of Alabama and Birmingham VA Medical Center Birmingham USA

US Department of Health and Human Services Environmental Autoimmunity Group Clinical Research Branch National Institute of Environmental Health Sciences National Institutes of Health Bethesda Maryland USA

Vall d'Hebron General Hospital Barcelona Spain

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