2016 American College of Rheumatology/European League Against Rheumatism Criteria for Minimal, Moderate, and Major Clinical Response in Juvenile Dermatomyositis

Abstract

Objective Develop response criteria for adult dermatomyositis (DM) and polymyositis (PM). Methods Expert surveys, logistic regression, and conjoint analysis were used to develop 287 definitions using core set measures (CSM). Myositis experts rated greater improvement among multiple pair-wise scenarios in conjoint analysis surveys, where different levels of improvement in two CSM were presented. The PAPRIKA (Potentially All Pairwise Rankings of All Possible Alternatives) method determined relative weights of CSM and conjoint analysis definitions. Performance characteristics of definitions were evaluated on patient profiles using expert consensus (gold standard) and were validated using a clinical trial. Nominal group technique was used for consensus. Results Consensus was reached for a conjoint analysis–based continuous model using absolute percentage change in CSMs (physician, patient, and extra-muscular global activity, muscle strength, health assessment questionnaire and muscle enzymes). A Total Improvement Score (0–100), determined by summing scores in each CSM, was based on the improvement and relative weight of each CSM. Thresholds for minimal, moderate, and major improvement were ≥20, ≥40, and ≥60 points in the Total Improvement Score. The same criteria were chosen for juvenile DM with different improvement thresholds. Sensitivity and specificity in DM/PM patient cohorts were 85% and 92%, 90% and 96%, and 90% and 96% for minimal, moderate, and major improvement, respectively. Definitions were validated in trial analysis for differentiating the physician rating of improvement (P<0.001). Conclusion The response criteria for adult DM/PM was the conjoint analysis model based on absolute percentage change in six CSMs, with thresholds for minimal, moderate, and major improvement.

Current Classification and Management of Inflammatory Myopathies

“…The extra muscular disease activity is an important component of the core set since it reflects tissue damage of joints, lung, skin, heart, or the gastrointestinal system [ 168 ]. Recently, the core set measures were transformed into a numeric scale and weighted depending on their impact on the final score; this composite score was officially accredited as “response criteria” by the American College of Rheumatology (ACR) and the European League Against Rheumatology (EULAR) for adult myositis [ 169, 170 ] as well as for juvenile myositis [ 171, 172 ]. Manual muscle testing of a group of muscles (MMT-6 or MMT-8) is an important component of this longitudinal assessment in myositis and should be performed by the physician at each clinical visit.…”

Update on outcome assessment in myositis

“…They were developed using large patient datasets; novel conjoint analysis methodology 17–21 ; clinical trial validation; and, finally, consensus among adult and pediatric myositis experts who specialize in rheumatology, neurology, and dermatology. They are now approved as final myositis response criteria by the ACR and EULAR 22–25 . These criteria are the same for adult and juvenile patients, but with different thresholds for the varying degrees of response.…”

Efficacy and Safety of Rituximab in Korean Patients with Refractory Inflammatory Myopathies

“…With the brief response criteria in this study, the data regarding extra-muscular lesions were simply handled by ‘physician's global assessment’, and further studies should apply a standardized assessment protocol for extra-muscular involvement such as the 2016 ACR-EULAR response criteria. 44 …”