Proposal for the development of an international minimal data collection for juvenile dermatomyositis (JDM)

Abstract

Background/Purpose: Several groups collect prospective data on patients with juvenile idiopathic inflammatory myopathies (IIM), including the UK JDM Cohort Biomarker Study &Repository, CARRA, and Euromyositis. Datasets are partially overlapping. A consensus minimal data collection would facilitate comparison and communication between groups. We propose a new international minimal data collection, potentially valuable in trials and clinical contexts, to be collected by clinicians for all JDM patients, respecting data protection and ownership. Methods: Variables collected within the UK JDM Cohort/Biomarker Study, CARRA, Euromyositis and a multi-national study of 27 centres in Europe/Latin America1 were compared. Those common to at least 2 datasets were considered for inclusion, based on agreement between collaborators. Results: To date, the group have identified 19 variables common to all data collections (UK JDM cohort, CARRA, Euromyositis and the inception cohort by Ravelli et al1), 16 variables common to 3 datasets and 24 variables common to 2 datasets. Collaborators have discussed each variable to determine which ones are most relevant to clinical practice and useful in research. Thereby, a minimal data collection has been proposed that includes demographic data, diagnostic data, clinical features, major organ involvement, measures of disease activity / damage (as defined by IMACS / PRINTO), health assessment questionnaires, investigations and treatment. The data collection is thought to be achievable by clinicians within their normal practice. Work is in progress to define each variable, including optional detailed collection of activity / damage indices within specialist / research environments. Conclusion: An international minimal data collection for use in trials would allow greater understanding of disease course / prognosis, enhance international collaboration between groups, and facilitate linking to biobanks. The dataset requires testing through existing collaborations (IMACS, PRINTO etc). Collaboration with adult groups (eg. via Euromyositis) may allow harmonised data collection from paediatric to adult services, providing valuable outcome data for this rare disease.