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 Arnardottir2, Hector Chinoy11, Robert G Cooper12, Katalin Dankó13, Mazen M Dimachkie7, Brian M Feldman14, Ignacio Garcia-De La Torre15, Patrick Gordon16, Taichi Hayashi17, James D Katz18, Hitoshi Kohsaka19, Peter A Lachenbruch20, Bianca A Lang21, Yuhui Li22, Chester V Oddis10, Marzena Olesinska23, Ann M Reed24, Lidia Rutkowska-Sak25, Helga Sanner26, Albert Selva-O'Callaghan27, Yeong-Wook Song28, Jiri Vencovsky29, Steven R Ytterberg30, Frederick W Miller31, Lisa G Rider31. 1. Karolinska University Hospital and Karolinska Institutet, Stockholm, Sweden. 2. Karolinska Institutet, Stockholm, Sweden. 3. Philadelphia VA Medical Center and Hospital of the University of Pennsylvania, Philadelphia. 4. Great Ormond Street Hospital for Children NHS Trust, London, UK. 5. Academic Medical Centre, Amsterdam, The Netherlands. 6. Brigham and Women's Hospital, Harvard Medical School, Boston, Massachusetts. 7. University of Kansas Medical Center, Kansas City. 8. Brigham and Women's Hospital and Boston VA Healthcare, Boston, Massachusetts. 9. Mayo Clinic College of Medicine, Rochester, Minnesota, and University of Alabama and Birmingham VA Medical Center, Birmingham, Alabama. 10. University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania. 11. Central Manchester University Hospitals NHS Foundation Trust, University of Manchester, Manchester, UK. 12. University of Liverpool, Liverpool, UK. 13. University of Debrecen, Debrecen, Hungary. 14. University of Toronto and The Hospital for Sick Children, Toronto, Ontario, Canada. 15. Hospital General de Occidente, Secretaría de Salud and University of Guadalajara, Guadalajara, Mexico. 16. King's College Hospital NHS Foundation Trust, London, UK. 17. University of Tsukuba, Tsukuba, Japan. 18. National Institute of Arthritis and Musculoskeletal and Skin Diseases, NIH, Bethesda, Maryland. 19. Tokyo Medical and Dental University, Tokyo, Japan. 20. Oregon State University, Corvallis. 21. IWK Health Centre and Dalhousie University, Halifax, Nova Scotia, Canada. 22. People's Hospital of Beijing University, Beijing, China. 23. National Institute of Geriatrics, Rheumatology and Rehabilitation, Warsaw, Poland. 24. Duke University, Durham, North Carolina. 25. Institute of Rheumatology, Warsaw, Poland. 26. Oslo University Hospital-Rikshospitalet, Oslo, Norway. 27. Vall d'Hebron General Hospital, Barcelona, Spain. 28. Seoul National University College of Medicine, Seoul, Republic of Korea. 29. Charles University, Prague, Czech Republic. 30. Mayo Clinic College of Medicine, Rochester, Minnesota. 31. National Institute of Environmental Health Sciences, NIH, Bethesda, Maryland.
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. 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.
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.
Authors: Jessica E Hoogendijk; Anthony A Amato; Bryan R Lecky; Ernest H Choy; Ingrid E Lundberg; Michael R Rose; Jiri Vencovsky; Marianne de Visser; Richard A Hughes Journal: Neuromuscul Disord Date: 2004-05 Impact factor: 4.296
Authors: M F G van der Meulen; I M Bronner; J E Hoogendijk; H Burger; W J van Venrooij; A E Voskuyl; H J Dinant; W H J P Linssen; J H J Wokke; M de Visser Journal: Neurology Date: 2003-08-12 Impact factor: 9.910
Authors: Anastasiya Muntyanu; Michelle Le; Zainab Ridha; Elizabeth O'Brien; Ivan V Litvinov; Philippe Lefrançois; Elena Netchiporouk Journal: J Cell Commun Signal Date: 2021-08-03 Impact factor: 5.782