University of Manchester



EULAR/ACR Classification Criteria for Adult and Juvenile Idiopathic Inflammatory Myopathies and their Major Subgroups

Authors:

Ingrid E Lundberg1, Anna Tjärnlund1*, Matteo Bottai2*, Victoria P Werth3, Clarissa Pilkington4, Marianne de Visser5, Lars Alfredsson2, Anthony A Amato6, Richard J Barohn7,

Matthew H Liang8, Jasvinder A Singh9, Rohit Aggarwal10, Snjolaug Arnardottir11, Hector Chinoy12, Robert G Cooper13, Katalin Dankó14, Mazen M Dimachkie7, Brian M Feldman15, Ignacio Garcia-De La Torre16, Patrick Gordon17, Taichi Hayashi18, James D Katz19, Hitoshi Kohsaka20, Peter A Lachenbruch21, Bianca A Lang22, Yuhui Li23, Chester V Oddis10, Marzena Olesinska24, Ann M Reed25, Lidia Rutkowska-Sak26, Helga Sanner27, Albert Selva-O'Callaghan28, Yeong Wook Song29, Jiri Vencovsky30, Steven R Ytterberg31, Frederick W Miller32§, Lisa G Rider32§; the International Myositis Classification Criteria Project consortium†, the Euromyositis register† and the Juvenile dermatomyositis cohort biomarker study and repository (JDRG) (United Kingdom and Ireland)† († see Appendix)

Affiliations:

1Rheumatology Unit, Department of Medicine, Karolinska University Hospital, Solna, Karolinska Institutet, Stockholm, Sweden

2Institute for Environmental Medicine, Karolinska Institutet, Stockholm, Sweden

3Department of Dermatology, Philadelphia VAMC and Hospital of the University of Pennsylvania, Philadelphia, USA

4Department of Rheumatology, Great Ormond Street Hospital for Children NHS Trust, London, United Kingdom

5Department of Neurology, Academic Medical Centre, Amsterdam, Netherlands

6Department of Neurology, Brigham and Women’s Hospital, Harvard Medical School, Boston, USA

7Department of Neurology, University of Kansas Medical Center, Kansas City, USA

8Division of Rheumatology, Immunology and Allergy, Brigham and Women´s Hospital, and Section of Rheumatology, Boston VA Healthcare, Boston, USA

9University of Alabama and Birmingham VA Medical Center, Birmingham, USA & Mayo Clinic College of Medicine, Rochester, USA

10Division of Rheumatology and Clinical Rheumatology, University of Pittsburgh School of Medicine, Pittsburgh, USA

11Department of Clinical Neuroscience, Karolinska Institutet, Stockholm, Sweden

12National Institute of Health Research Manchester Musculoskeletal Biomedical Research Unit, Central Manchester University Hospitals NHS Foundation Trust, University of Manchester, Manchester, United Kingdom

13MRC/ARUK Institute of Ageing and Chronic Disease, Faculty of Health & Life Sciences, University of Liverpool, Liverpool, United Kingdom

14Division of Immunology, 3rd Department of Internal Medicine, Medical and Health Science Center, University of Debrecen, Debrecen, Hungary

15Division of Rheumatology, Department of Pediatrics, University of Toronto and The Hospital for Sick Children, Toronto, Canada

16Department of Immunology and Rheumatology, Hospital General de Occidente, Secretaría de Salud, and University of Guadalajara, Guadalajara, Jalisco, México

17Department of Rheumatology, King`s College Hospital NHS Foundation Trust, London, United Kingdom

18Clinical Immunology, Doctoral Program in Clinical Sciences, Graduate School of Comprehensive Human Sciences, University of Tsukuba, Tsukuba, Japan

19National Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health, US Department of Health and Human Services, Bethesda, USA

20Department of Rheumatology, Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University, Tokyo, Japan

21Department of Public Health, Oregon State University, Corvallis, USA

22Division of Rheumatology, Department of Pediatrics, IWK Health Centre and Dalhousie University, Halifax, Canada

23Department of Rheumatology and Immunology, People´s Hospital of Beijing University, Beijing, China

24Connective Tissue Diseases Department, National Institute of Geriatrics, Rheumatology and Rehabilitation, Warsaw, Poland

25Department of Pediatrics, Duke University, Durham, USA

26Paediatric Clinic of Rheumatology, Institute of Rheumatology, Warsaw, Poland

27Section of Rheumatology, Oslo University Hospital–Rikshospitalet, Oslo, Norway

28Vall d'Hebron General Hospital, Barcelona, Spain

29Department of Internal Medicine, Medical Research Center, Clinical Research Institute, Seoul National University College of Medicine, Seoul, Republic of Korea

30Institute of Rheumatology and Department of Rheumatology, 1st Faculty of Medicine, Charles University, Prague, Czech Republic

31Division of Rheumatology, Mayo Clinic College of Medicine, Rochester, USA

32Environmental Autoimmunity Group, Clinical Research Branch, National Institute of Environmental Health Sciences, National Institutes of Health, US Department of Health and Human Services, Bethesda, USA

*Contributed equally

§Contributed equally

Corresponding author:

Ingrid E. Lundberg

Rheumatology Unit, D2:01

Karolinska University Hospital, Solna

S-171 76 Stockholm

Sweden

E-mail: Ingrid.Lundberg@ki.se

Telephone number: +46 8 517 760 87

Key words:

Dermatomyositis, Polymyositis, Autoimmune diseases

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 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. Sub-classification 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 ................
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