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Connective tissue diseases
Original article
EULAR/ACR classification criteria for adult and juvenile idiopathic inflammatory myopathies and their major subgroups: a methodology report
  1. Matteo Bottai1,
  2. Anna Tjärnlund2,
  3. Giola Santoni3,
  4. Victoria P Werth4,
  5. Clarissa Pilkington5,
  6. Marianne de Visser6,
  7. Lars Alfredsson7,
  8. Anthony A Amato8,
  9. Richard J Barohn9,
  10. Matthew H Liang10,
  11. Jasvinder A Singh11,
  12. Rohit Aggarwal12,
  13. Snjolaug Arnardottir13,
  14. Hector Chinoy14,
  15. Robert G Cooper15,
  16. Katalin Danko16,
  17. Mazen M Dimachkie9,
  18. Brian M Feldman17,
  19. Ignacio García-De La Torre18,
  20. Patrick Gordon19,
  21. Taichi Hayashi20,
  22. James D Katz21,
  23. Hitoshi Kohsaka22,
  24. Peter A Lachenbruch23,
  25. Bianca A Lang24,
  26. Yuhui Li25,
  27. Chester V Oddis12,
  28. Marzena Olesinka26,
  29. Ann M Reed27,
  30. Lidia Rutkowska-Sak28,
  31. Helga Sanner29,
  32. Albert Selva-O’Callaghan30,
  33. Yeong Wook Song31,
  34. Jiri Vencovsky32,
  35. Steven R Ytterberg33,
  36. Frederick W Miller34,
  37. Lisa G Rider34 and
  38. Ingrid E Lundberg2
  39. The International Myositis Classification Criteria Project consortium, the Euromyositis register and the Juvenile Dermatomyositis Cohort Biomarker Study and Repository (JDRG) (UK and Ireland)
    1. 1Unit of Biostatistics, Institute for Environmental Medicine, Karolinska Institutet, Stockholm, Sweden
    2. 2Rheumatology Unit, Department of Medicine, Karolinska University Hospital, Solna, Karolinska Institutet, Stockholm, Sweden
    3. 3Aging Research Center, Department of Neurobiology, Care Sciences and Society, Karolinska Institutet and Stockholm University, Stockholm, Sweden
    4. 4Department of Dermatology, Philadelphia VAMC and Hospital of the University of Pennsylvania, Philadelphia, Pennsylvania, USA
    5. 5Department of Rheumatology, Great Ormond Street Hospital for Children NHS Trust, London, UK
    6. 6Department of Neurology, Academic Medical Centre, Amsterdam, The Netherlands
    7. 7Institute of Environmental Medicine, Karolinska Institutet, Stockholm, Sweden
    8. 8Department of Neurology, Brigham and Women’s Hospital, Harvard Medical School, Boston, Massachusetts, USA
    9. 9Department of Neurology, University of Kansas Medical Center, Kansas City, Kansas, USA
    10. 10Division of Rheumatology, Immunology and Allergy, Brigham and Women’s Hospital, and Section of Rheumatology, Boston VA Healthcare System, Boston, Massachusetts, USA
    11. 11University of Alabama at Birmingham (UAB) and Birmingham VA Medical, Birmingham, Alabama, USA
    12. 12Division of Rheumatology and Clinical Rheumatology, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania, USA
    13. 13Department of Clinical Neuroscience, Karolinska Institutet, Stockholm, Sweden
    14. 14National Institute of Health Research, Manchester Musculoskeletal Biomedical Research Unit, Central Manchester University Hospitals NHS Foundation Trust, University of Manchester, Manchester, UK
    15. 15MRC/ARUK Institute of Ageing and Chronic Disease, Faculty of Health & Life Sciences, University of Liverpool, Liverpool, UK
    16. 16Division of Immunology, 3rd Department of Internal Medicine, Medical and Health Science Center, University of Debrecen, Debrecen, Hungary
    17. 17Department of Pediatrics, Division of Rheumatology, University of Toronto and The Hospital for Sick Children, Toronto, Canada
    18. 18Department of Immunology and Rheumatology, Hospital General de Occidente, Secretaría de Salud, and University of Guadalajara, Guadalajara, Mexico
    19. 19Department of Rheumatology, King’s College Hospital NHS Foundation Trust, London, UK
    20. 20Clinical Immunology, Doctoral Program in Clinical Sciences, Graduate School of Comprehensive Human Sciences, University of Tsukuba, Tsukuba, Japan
    21. 21US Department of Health and Human Services, National Institutes of Health, Bethesda, Maryland, USA
    22. 22Department of Rheumatology, Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University, Tokyo, Japan
    23. 23Department of Public Health, Oregon State University, Corvallis, Oregon, USA
    24. 24Department of Pediatrics, Division of Rheumatology, IWK Health Centre and Dalhousie University, Halifax, Canada
    25. 25Department of Rheumatology and Immunology, People’s Hospital of Beijing University, Beijing, China
    26. 26Connective Tissue Diseases Department, National Institute of Geriatrics, Rheumatology and Rehabilitation, Warsaw, Poland
    27. 27Department of Pediatrics, Duke University, Durham, North Carolina, USA
    28. 28Paediatric Clinic of Rheumatology, Institute of Rheumatology, Warsaw, Poland
    29. 29Section of Rheumatology, Oslo University Hospital–Rikshospitalet, Oslo, Norway
    30. 30Vall d’Hebron General Hospital, Barcelona, Spain
    31. 31Department of Internal Medicine, Medical Research Center, Clinical Research Institute, Seoul National University College of Medicine, Seoul, Republic of Korea
    32. 32Institute of Rheumatology and Department of Rheumatology, 1st Faculty of Medicine, Charles University, Prague, Czech Republic
    33. 33Division of Rheumatology, Mayo Clinic College of Medicine, Rochester, Minnesota, USA
    34. 34Environmental Autoimmunity Group, US Department of Health and Human Services, National Institute of Environmental Health Sciences, National Institutes of Health, Bethesda, Maryland, USA
    1. Correspondence to Professor Matteo Bottai; Matteo.Bottai{at}ki.se

    Abstract

    Objective To describe the methodology used to develop new classification criteria for adult and juvenile idiopathic inflammatory myopathies (IIMs) and their major subgroups.

    Methods An international, multidisciplinary group of myositis experts produced a set of 93 potentially relevant variables to be tested for inclusion in the criteria. Rheumatology, dermatology, neurology and paediatric clinics worldwide collected data on 976 IIM cases (74% adults, 26% children) and 624 non-IIM comparator cases with mimicking conditions (82% adults, 18% children). The participating clinicians classified each case as IIM or non-IIM. Generally, the classification of any given patient was based on few variables, leaving remaining variables unmeasured. We investigated the strength of the association between all variables and between these and the disease status as determined by the physician. We considered three approaches: (1) a probability-score approach, (2) a sum-of-items approach criteria and (3) a classification-tree approach.

    Results The approaches yielded several candidate models that were scrutinised with respect to statistical performance and clinical relevance. The probability-score approach showed superior statistical performance and clinical practicability and was therefore preferred over the others. We developed a classification tree for subclassification of patients with IIM. A calculator for electronic devices, such as computers and smartphones, facilitates the use of the European League Against Rheumatism/American College of Rheumatology (EULAR/ACR) classification criteria.

    Conclusions The new EULAR/ACR classification criteria provide a patient’s probability of having IIM for use in clinical and research settings. The probability is based on a score obtained by summing the weights associated with a set of criteria items.

    • dermatomyositis
    • polymyositis
    • autoimmune diseases

    This is an Open Access article distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/

    https://doi.org/10.1136/rmdopen-2017-000507

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      Footnotes

      • Contributors All authors were involved in drafting the article or revising it critically for important intellectual content. All authors approved the final version to be published and had full access to all of the data in the study and take responsibility for the integrity of the data and the accuracy of the data analysis. Study conception and design: MB, AT, VPW, CP, MdeV, LA, AAA, RJB, MHL, JAS, KD, BMF, HK, PAL, BAL, FWM, LGR, IEL. Acquisition of data: MB, AT, VPW, CP, MdeV, LA, AAA, RJB, MHL, JAS, RA, SA, HC, RGC, KD, MMD, BMF, IG-DLT, PG, TH, JDK, HK, PAL, BAL, YL, CVO, MO, AMR, LR-S, HS, AS-OC, YWS, JV, SRY, FWM, LGR, IEL, The International Myositis Classification Criteria Consortium, working committee members. Analysis and interpretation of data: MB, AT, GS, VPW, CP, MdeV, LA, AAA, RJB, MHL, JAS, RA, BMF, IG-DLT, PG, HK, PAL, BAL, YL, FWM, LGR, IEL.

      • Funding This study was financially supported by ACR, EULAR, The Myositis Association (TMA), the Intramural Research Program of the NIH, National Institute of Environmental Health Sciences and the European Science Foundation for the Euromyositis Register. CARRA, Inc. is funded by NIH-NIAMS, Friends of CARRA and the Arthritis Foundation.

      • Disclaimer The views expressed in this article are those of the authors and do not necessarily reflect the position or policy of the Department of Veterans Affairs or the United States government, or the NHS, the National Institute for Health Research or the Department of Health (UK).

      • Competing interests JAS has received research grants from Takeda and Savient and consultant fees from Savient, Takeda, Regeneron, Merz, Bioiberica, Crealta and Allergan. JAS serves as the principal investigator for an investigator-initiated study funded by Horizon pharmaceuticals through a grant to DINORA, Inc., a 501 (c)(3) entity. JAS is a member of the executive of OMERACT, an organisation that develops outcome measures in rheumatology and receives arms-length funding from 36 companies; a member of the American College of Rheumatology’s (ACR) Annual Meeting Planning Committee (AMPC); Chair of the ACR Meet-the-Professor, Workshop and Study Group Subcommittee; and a member of the Veterans Affairs Rheumatology Field Advisory Committee. HC and RGC’s work in myositis is partly funded by grants from Arthritis Research UK (18474) and the Medical Research Council (MR/N003322/1). JV’s work in myositis is supported by the Project (Ministry of Health, Czech Republic) for Conceptual Development of Research Organization 00023728.

      • Ethics approval The study was approved by ethics committees at each participating site.

      • Provenance and peer review Not commissioned; externally peer reviewed.

      • Collaborators Maria Amoruso (Children’s Hospital of Chicago and Northwestern University Feinberg School of Medicine, Chicago, USA), Helena Andersson (Section of Rheumatology, Oslo University Hospital–Rikshospitalet, Oslo, Norway), Nastaran Bayat (Environmental Autoimmunity Group, Clinical Research Branch, National Institute of Environmental Health Sciences, National Institutes of Health, US Department of Health and Human Services, Bethesda, USA), Kavish J Bhansing (Department of Neurology, Radboud University Medical Center, Nijmegen, The Netherlands), Sara Bucher (Department of Neurology, Örebro University, Örebro, Sweden), Richard Champbell (King’s College Hospital, London, UK), Christina Charles-Schoeman (Division of Rheumatology, Department of Medicine, University of California Los Angeles, Los Angeles, USA), Vinay Chaudhry (Department of Neurology, Johns Hopkins University School of Medicine, Baltimore, USA), Lisa Christopher-Stine (Division of Rheumatology, Johns Hopkins University School of Medicine, Baltimore, USA), Lorinda Chung (Department of Dermatology, Stanford University School of Medicine, Stanford, USA; Division of Rheumatology, Palo Alto Veterans Affairs Health Care System, Palo Alto, USA; Department of Medicine, Division of Rheumatology, Stanford University School of Medicine, Stanford, USA), Mary Cronin (Rheumatology Division, Medical College of Wisconsin, Milwaukee, USA), Theresa Curry (The Myositis Association), Kathe Dahlbom (Department of Neurology, Örebro University, Örebro, Sweden), Oliver Distler (Division of Rheumatology, University Hospital Zurich, Zurich, Switzerland), Petros Efthimiou (New York Methodist Hospital, Brooklyn, NY & Weill Cornell Medical College, New York, USA), Baziel GM van Engelen (Department of Neurology, Radboud University Medical Center, Nijmegen, The Netherlands), Abdullah Faiq (Environmental Autoimmunity Group, Clinical Research Branch, National Institute of Environmental Health Sciences, National Institutes of Health, US Department of Health and Human Services, Bethesda, USA), Payam Noroozi Farhadi (Environmental Autoimmunity Group, Clinical Research Branch, National Institute of Environmental Health Sciences, National Institutes of Health, US Department of Health and Human Services, Bethesda, USA), David Fiorentino (Division of Immunology and Rheumatology, Stanford University School of Medicine, Stanford, USA), Gerald Hengstman (Department of Neurology, Catharina Hospital, Eindhoven, the Netherlands), Jessica Hoogendijk (Rudolf Magnus Institute for Neuroscience, Department of Neurology, University Medical Center Utrecht, Netherlands), Adam Huber (IWK Health Centre and Dalhousie University, Halifax, Canada), Hiroshi Kataoka (Department of Medicine II, Hokkaido University Graduate School of Medicine, Hokkaido, Japan), Yasuhiro Katsumata (Institute of Rheumatology, Tokyo Women’s Medical University, Tokyo, Japan), Susan Kim (Boston Children’s Hospital, Pediatric Rheumatology, Boston, USA), Michelle Kong-Rosario (North shore-LIJ Health System, USA), Apostolos Kontzias (Cleveland Clinic, Cleveland, USA), Petra Krol (Department of Pediatrics and Adolescent Medicine, Charles University, 1st Medical School, Prague, Czech Republic), Takashi Kurita (Department of Medicine II, Hokkaido University Graduate School of Medicine, Hokkaido, Japan), Zhan-Guo Li (Department of Rheumatology and Immunology, People’s Hospital of Beijing University, China), Björn Lindvall (Department of Neurology, Örebro University, Örebro, Sweden), Helen Linklater (Department of Rheumatology, King`s College Hospital NHS Foundation Trust, London, UK), Sue Maillard (Department of Rheumatology, Great Ormond Street Hospital for Children NHS Trust, London, UK), Gulnara Mamyrova (Myositis Center, Division of Rheumatology, Department of Medicine, George Washington University, Washington DC, USA), Renato Mantegazza (Neurology IV, Neuroimmunology and Neuromuscular Diseases Unit, Fondazione Istituto Neurologico Carlo Besta, Milan, Italy), Galina S Marder (North shore-LIJ Health System, USA), Suely Kazue Nagahashi Marie (Division of Neurology, Faculdade de Medicina, Universidade de São Paulo, São Paulo, Brasil), Pernille Mathiesen (Paediatric Department, Copenhagen University Hospital Holbaek, Holbaek, Denmark), Clio P Mavragani (Department of Pathophysiology, School of Medicine, University of Athens, Athens, Greece), Neil J McHugh (Royal National Hospital for Rheumatic Diseases, Bath, UK), Mimi Michaels (Department of Neurology, University of Kansas Medical Center, Kansas City, USA), Reem Mohammed (Division of Rheumatology, Department of Paediatrics, University of Toronto and The Hospital for Sick Children, Toronto, Canada), Gabrielle Morgan (Children’s Hospital of Chicago and Northwestern University Feinberg School of Medicine, Chicago, USA), David W Moser (Rheumatology, Cincinnati Children’s Hospital Medical Center, Cincinnati, USA), Haralampos M Moutsopoulos (Department of Pathophysiology, School of Medicine, University of Athens, Athens, Greece).