2017 European League Against Rheumatism/American College of Rheumatology Classification Criteria for Adult and Juvenile Idiopathic Inflammatory Myopathies and Their Major Subgroups

the International Myositis Classification Criteria Project Consortium, the Euromyositis Register, and the Juvenile Dermatomyositis Cohort Biomarker Study and Repository (UK and Ireland)

doi:10.1002/art.40320

2017 European League Against Rheumatism/American College of Rheumatology Classification Criteria for Adult and Juvenile Idiopathic Inflammatory Myopathies and Their Major Subgroups

the International Myositis Classification Criteria Project Consortium, the Euromyositis Register, and the Juvenile Dermatomyositis Cohort Biomarker Study and Repository (UK and Ireland)

Research output: Contribution to journal › Article › peer-review

510 Citations (Scopus)

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.

Original language	English
Pages (from-to)	2271-2282
Number of pages	12
Journal	Arthritis and Rheumatology
Volume	69
Issue number	12
DOIs	https://doi.org/10.1002/art.40320
Publication status	Published - Dec 2017
Externally published	Yes

Bibliographical note

Funding Information:
Supported by the European League Against Rheumatism, the American College of Rheumatology, The Myositis Association, and in part by the NIH (Intramural Research Program and the National Institute of Environmental Health Sciences), the European Science Foundation for the Euromyositis Register, the Swedish Research Council (grant K2014-52X-14045-14-3), and the regional agreement on medical training and clinical research between the Stockholm County Council and the Karolinska Institutet. The project also received support (not financial support/funding) from different associations: the American Academy of Neurology, the Childhood Arthritis and Rheumatology Research Alliance (CARRA; CARRA Inc. is funded by the National Institute of Arthritis and Musculoskeletal and Skin Diseases, NIH), Friends of CARRA, the Arthritis Foundation, the European Neuromuscular Centre, the International Myositis Assessment and Clinical Studies Group, the Muscle Study Group, the Rheumatologic Dermatology Society, the Pediatric Rheumatology European Society network for juvenile dermatomysitis, and the Pediatric Rheumatology International Trials Organization. Drs. Chinoy and Cooper’s work in myositis is supported in part by Arthritis Research UK (grant 18474) and the Medical Research Council (grant MR/N003322/1). Dr. Vencovsky’s work in myositis is supported by the Ministry of Health, Czech Republic (Project for Conceptual Development of Research Organization grant 00023728).

Funding Information:
We thank Elin Forslund for assistance with data registration. We thank Dr. Andrew Mammen and Dr. Mike Ward for critical reading of the manuscript. We are grateful for contribution of clinical data from investigators and for participants contributing with valuable input at IMCCP meetings.

Funding Information:
Dr. Singh has received consulting fees from Savient, Regen-eron, Merz, Iroko, Bioiberica, Crealta/Horizon, Allergan, WebMD, UBM (less than $10,000 each), and Takeda (more than $10,000) and research grants from Takeda and Savient; he also 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, and is a member of the executive committee of OMERACT, an organization that develops outcome measures in rheumatology and receives arms-length funding from 36 companies.

Publisher Copyright:
© 2017, American College of Rheumatology

ASJC Scopus Subject Areas

Immunology and Allergy
Rheumatology
Immunology

PubMed: MeSH publication types

Journal Article
Research Support, N.I.H., Intramural
Research Support, Non-U.S. Gov't

Access to Document

10.1002/art.40320

Cite this

the International Myositis Classification Criteria Project Consortium, the Euromyositis Register, and the Juvenile Dermatomyositis Cohort Biomarker Study and Repository (UK and Ireland) (2017). 2017 European League Against Rheumatism/American College of Rheumatology Classification Criteria for Adult and Juvenile Idiopathic Inflammatory Myopathies and Their Major Subgroups. Arthritis and Rheumatology, 69(12), 2271-2282. https://doi.org/10.1002/art.40320

2017 European League Against Rheumatism/American College of Rheumatology Classification Criteria for Adult and Juvenile Idiopathic Inflammatory Myopathies and Their Major Subgroups. / the International Myositis Classification Criteria Project Consortium, the Euromyositis Register, and the Juvenile Dermatomyositis Cohort Biomarker Study and Repository (UK and Ireland).
In: Arthritis and Rheumatology, Vol. 69, No. 12, 12.2017, p. 2271-2282.

Research output: Contribution to journal › Article › peer-review

the International Myositis Classification Criteria Project Consortium, the Euromyositis Register, and the Juvenile Dermatomyositis Cohort Biomarker Study and Repository (UK and Ireland) 2017, '2017 European League Against Rheumatism/American College of Rheumatology Classification Criteria for Adult and Juvenile Idiopathic Inflammatory Myopathies and Their Major Subgroups', Arthritis and Rheumatology, vol. 69, no. 12, pp. 2271-2282. https://doi.org/10.1002/art.40320

the International Myositis Classification Criteria Project Consortium, the Euromyositis Register, and the Juvenile Dermatomyositis Cohort Biomarker Study and Repository (UK and Ireland). 2017 European League Against Rheumatism/American College of Rheumatology Classification Criteria for Adult and Juvenile Idiopathic Inflammatory Myopathies and Their Major Subgroups. Arthritis and Rheumatology. 2017 Dec;69(12):2271-2282. doi: 10.1002/art.40320

the International Myositis Classification Criteria Project Consortium, the Euromyositis Register, and the Juvenile Dermatomyositis Cohort Biomarker Study and Repository (UK and Ireland). / 2017 European League Against Rheumatism/American College of Rheumatology Classification Criteria for Adult and Juvenile Idiopathic Inflammatory Myopathies and Their Major Subgroups. In: Arthritis and Rheumatology. 2017 ; Vol. 69, No. 12. pp. 2271-2282.

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title = "2017 European League Against Rheumatism/American College of Rheumatology Classification Criteria for Adult and Juvenile Idiopathic Inflammatory Myopathies and Their Major Subgroups",

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.",

author = "{the International Myositis Classification Criteria Project Consortium, the Euromyositis Register, and the Juvenile Dermatomyositis Cohort Biomarker Study and Repository (UK and Ireland)} and Lundberg, {Ingrid E.} and Anna Tj{\"a}rnlund and Matteo Bottai and Werth, {Victoria P.} and Clarissa Pilkington and {de Visser}, Marianne and Lars Alfredsson and Amato, {Anthony A.} and Barohn, {Richard J.} and Liang, {Matthew H.} and Singh, {Jasvinder A.} and Rohit Aggarwal and Snjolaug Arnardottir and Hector Chinoy and Cooper, {Robert G.} and Katalin Dank{\'o} and Dimachkie, {Mazen M.} and Feldman, {Brian M.} and {Garcia-De La Torre}, Ignacio and Patrick Gordon and Taichi Hayashi and Katz, {James D.} and Hitoshi Kohsaka and Lachenbruch, {Peter A.} and Lang, {Bianca A.} and Yuhui Li and Oddis, {Chester V.} and Marzena Olesinska and Reed, {Ann M.} and Lidia Rutkowska-Sak and Helga Sanner and Albert Selva-O'Callaghan and Song, {Yeong Wook} and Jiri Vencovsky and Ytterberg, {Steven R.} and Miller, {Frederick W.} and Rider, {Lisa G.}",

note = "Funding Information: Supported by the European League Against Rheumatism, the American College of Rheumatology, The Myositis Association, and in part by the NIH (Intramural Research Program and the National Institute of Environmental Health Sciences), the European Science Foundation for the Euromyositis Register, the Swedish Research Council (grant K2014-52X-14045-14-3), and the regional agreement on medical training and clinical research between the Stockholm County Council and the Karolinska Institutet. The project also received support (not financial support/funding) from different associations: the American Academy of Neurology, the Childhood Arthritis and Rheumatology Research Alliance (CARRA; CARRA Inc. is funded by the National Institute of Arthritis and Musculoskeletal and Skin Diseases, NIH), Friends of CARRA, the Arthritis Foundation, the European Neuromuscular Centre, the International Myositis Assessment and Clinical Studies Group, the Muscle Study Group, the Rheumatologic Dermatology Society, the Pediatric Rheumatology European Society network for juvenile dermatomysitis, and the Pediatric Rheumatology International Trials Organization. Drs. Chinoy and Cooper{\textquoteright}s work in myositis is supported in part by Arthritis Research UK (grant 18474) and the Medical Research Council (grant MR/N003322/1). Dr. Vencovsky{\textquoteright}s work in myositis is supported by the Ministry of Health, Czech Republic (Project for Conceptual Development of Research Organization grant 00023728). Funding Information: We thank Elin Forslund for assistance with data registration. We thank Dr. Andrew Mammen and Dr. Mike Ward for critical reading of the manuscript. We are grateful for contribution of clinical data from investigators and for participants contributing with valuable input at IMCCP meetings. Funding Information: Dr. Singh has received consulting fees from Savient, Regen-eron, Merz, Iroko, Bioiberica, Crealta/Horizon, Allergan, WebMD, UBM (less than $10,000 each), and Takeda (more than $10,000) and research grants from Takeda and Savient; he also 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, and is a member of the executive committee of OMERACT, an organization that develops outcome measures in rheumatology and receives arms-length funding from 36 companies. Publisher Copyright: {\textcopyright} 2017, American College of Rheumatology",

year = "2017",

month = dec,

doi = "10.1002/art.40320",

language = "English",

volume = "69",

pages = "2271--2282",

journal = "Arthritis and Rheumatology",

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T1 - 2017 European League Against Rheumatism/American College of Rheumatology Classification Criteria for Adult and Juvenile Idiopathic Inflammatory Myopathies and Their Major Subgroups

AU - the International Myositis Classification Criteria Project Consortium, the Euromyositis Register, and the Juvenile Dermatomyositis Cohort Biomarker Study and Repository (UK and Ireland)

AU - Lundberg, Ingrid E.

AU - Tjärnlund, Anna

AU - Bottai, Matteo

AU - Werth, Victoria P.

AU - Pilkington, Clarissa

AU - de Visser, Marianne

AU - Alfredsson, Lars

AU - Amato, Anthony A.

AU - Barohn, Richard J.

AU - Liang, Matthew H.

AU - Singh, Jasvinder A.

AU - Aggarwal, Rohit

AU - Arnardottir, Snjolaug

AU - Chinoy, Hector

AU - Cooper, Robert G.

AU - Dankó, Katalin

AU - Dimachkie, Mazen M.

AU - Feldman, Brian M.

AU - Garcia-De La Torre, Ignacio

AU - Gordon, Patrick

AU - Hayashi, Taichi

AU - Katz, James D.

AU - Kohsaka, Hitoshi

AU - Lachenbruch, Peter A.

AU - Lang, Bianca A.

AU - Li, Yuhui

AU - Oddis, Chester V.

AU - Olesinska, Marzena

AU - Reed, Ann M.

AU - Rutkowska-Sak, Lidia

AU - Sanner, Helga

AU - Selva-O'Callaghan, Albert

AU - Song, Yeong Wook

AU - Vencovsky, Jiri

AU - Ytterberg, Steven R.

AU - Miller, Frederick W.

AU - Rider, Lisa G.

N1 - Funding Information: Supported by the European League Against Rheumatism, the American College of Rheumatology, The Myositis Association, and in part by the NIH (Intramural Research Program and the National Institute of Environmental Health Sciences), the European Science Foundation for the Euromyositis Register, the Swedish Research Council (grant K2014-52X-14045-14-3), and the regional agreement on medical training and clinical research between the Stockholm County Council and the Karolinska Institutet. The project also received support (not financial support/funding) from different associations: the American Academy of Neurology, the Childhood Arthritis and Rheumatology Research Alliance (CARRA; CARRA Inc. is funded by the National Institute of Arthritis and Musculoskeletal and Skin Diseases, NIH), Friends of CARRA, the Arthritis Foundation, the European Neuromuscular Centre, the International Myositis Assessment and Clinical Studies Group, the Muscle Study Group, the Rheumatologic Dermatology Society, the Pediatric Rheumatology European Society network for juvenile dermatomysitis, and the Pediatric Rheumatology International Trials Organization. Drs. Chinoy and Cooper’s work in myositis is supported in part by Arthritis Research UK (grant 18474) and the Medical Research Council (grant MR/N003322/1). Dr. Vencovsky’s work in myositis is supported by the Ministry of Health, Czech Republic (Project for Conceptual Development of Research Organization grant 00023728). Funding Information: We thank Elin Forslund for assistance with data registration. We thank Dr. Andrew Mammen and Dr. Mike Ward for critical reading of the manuscript. We are grateful for contribution of clinical data from investigators and for participants contributing with valuable input at IMCCP meetings. Funding Information: Dr. Singh has received consulting fees from Savient, Regen-eron, Merz, Iroko, Bioiberica, Crealta/Horizon, Allergan, WebMD, UBM (less than $10,000 each), and Takeda (more than $10,000) and research grants from Takeda and Savient; he also 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, and is a member of the executive committee of OMERACT, an organization that develops outcome measures in rheumatology and receives arms-length funding from 36 companies. Publisher Copyright: © 2017, American College of Rheumatology

PY - 2017/12

Y1 - 2017/12

N2 - 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.

AB - 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.

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2017 European League Against Rheumatism/American College of Rheumatology Classification Criteria for Adult and Juvenile Idiopathic Inflammatory Myopathies and Their Major Subgroups

Abstract

Bibliographical note

ASJC Scopus Subject Areas

PubMed: MeSH publication types

Access to Document

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