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Author Notes:

Address correspondence to: Nicolino Ruperto, MD, MPH, Istituto Giannina Gaslini, Pediatria II, PRINTO, Via G. Gaslini 5, 16147 Genova Italy, Phone: +39-010-382854, Fax: +39-010-3933424, nicolaruperto@gaslini.org, www.printo.it or http://www.printo.it/ pediatric-rheumatology/.

See publication for full list of author contributions.

We thank the following people for invaluable input and feedback on project development and support: Dr. Brian Feldman, American College of Rheumatology; Dr. Daniel Aletaha, European League Against Rheumatism; Drs. Suzette Peng and Sarah Yim, Food and Drug Administration; Drs. Thorsten Vetter and Richard Vesely, European Medicines Agency; Mr. Bob Goldberg and Ms. Theresa Curry, The Myositis Association; Ms. Rhonda McKeever and Ms. Patti Lawler, Cure JM Foundation; and Ms. Irene Oakley, Myositis UK.

We thank Drs. Michael Ward and Steven Pavletic for critical reading of the manuscript.

Paul Hansen owns the 1000Minds software referred to in this article, which he co-invented with Franz Ombler. Jiri Vencovsky's work in myositis supported by the project (Ministry of Health, Czech Republic) for conceptual development of research organization 00023728 (Institute of Rheumatology).


Research Funding:

This work was supported in part by the American College of Rheumatology, the European League Against Rheumatism, the Intramural Research Program of the National Institutes of Health, the National Institute of Environmental Health Sciences, the National Center for Advancing Translational Sciences, and the National Institute of Arthritis and Musculoskeletal and Skin Diseases; Istituto Giannina Gaslini Genova (Italy) and the Paediatric Rheumatology International Trials Organisation (PRINTO); Cure JM Foundation; Myositis UK; and The Myositis Association.


  • Science & Technology
  • Life Sciences & Biomedicine
  • Rheumatology

2016 American College of Rheumatology/European League Against Rheumatism Criteria for Minimal, Moderate, and Major Clinical Response in Juvenile Dermatomyositis An International Myositis Assessment and Clinical Studies Group/Paediatric Rheumatology International Trials Organisation Collaborative Initiative

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Journal Title:

Annals of the Rheumatic Diseases


Volume 76, Number 5


, Pages 782-791

Type of Work:

Article | Post-print: After Peer Review


To develop response criteria for juvenile dermatomyositis (DM). We analysed the performance of 312 definitions that used core set measures from either the International Myositis Assessment and Clinical Studies Group (IMACS) or the Paediatric Rheumatology International Trials Organisation (PRINTO) and were derived from natural history data and a conjoint analysis survey. They were further validated using data from the PRINTO trial of prednisone alone compared to prednisone with methotrexate or cyclosporine and the Rituximab in Myositis (RIM) trial. At a consensus conference, experts considered 14 top candidate criteria based on their performance characteristics and clinical face validity, using nominal group technique. Consensus was reached for a conjoint analysis-based continuous model with a total improvement score of 0-100, using absolute per cent change in core set measures of minimal (≥30), moderate (≥45), and major (≥70) improvement. The same criteria were chosen for adult DM/polymyositis, with differing thresholds for improvement. The sensitivity and specificity were 89% and 91-98% for minimal improvement, 92-94% and 94-99% for moderate improvement, and 91-98% and 85-86% for major improvement, respectively, in juvenile DM patient cohorts using the IMACS and PRINTO core set measures. These criteria were validated in the PRINTO trial for differentiating between treatment arms for minimal and moderate improvement ( p=0.009-0.057) and in the RIM trial for significantly differentiating the physician's rating for improvement (p < 0.006). The response criteria for juvenile DM consisted of a conjoint analysis-based model using a continuous improvement score based on absolute per cent change in core set measures, with thresholds for minimal, moderate, and major improvement.

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