TY - JOUR T1 - 2016 American College of Rheumatology/European League Against Rheumatism Criteria for Minimal, Moderate, and Major Clinical Response in Juvenile Dermatomyositis JF - Annals of the Rheumatic Diseases JO - Ann Rheum Dis SP - 782 LP - 791 DO - 10.1136/annrheumdis-2017-211401 VL - 76 IS - 5 AU - Lisa G Rider AU - Rohit Aggarwal AU - Angela Pistorio AU - Nastaran Bayat AU - Brian Erman AU - Brian M Feldman AU - Adam M Huber AU - Rolando Cimaz AU - Rubén J Cuttica AU - Sheila Knupp de Oliveira AU - Carol B Lindsley AU - Clarissa A Pilkington AU - Marilynn Punaro AU - Angelo Ravelli AU - Ann M Reed AU - Kelly Rouster-Stevens AU - Annet van Royen-Kerkhof AU - Frank Dressler AU - Claudia Saad Magalhaes AU - Tamás Constantin AU - Joyce E Davidson AU - Bo Magnusson AU - Ricardo Russo AU - Luca Villa AU - Mariangela Rinaldi AU - Howard Rockette AU - Peter A Lachenbruch AU - Frederick W Miller AU - Jiri Vencovsky AU - Nicolino Ruperto AU - for the International Myositis Assessment and Clinical Studies Group and the Paediatric Rheumatology International Trials Organisation Y1 - 2017/05/01 UR - http://ard.bmj.com/content/76/5/782.abstract N2 - 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. ER -