Background: Juvenile dermatomyositis (JDM) is a rare but severe autoimmune inflammatory myositis of childhood.\nInternational collaboration is essential in order to undertake clinical trials, understand the disease and improve\nlong-term outcome. The aim of this study was to propose from existing collaborative initiatives a preliminary\nminimal dataset for JDM. This will form the basis of the future development of an international consensus-approved\nminimum core dataset to be used both in clinical care and inform research, allowing integration of data between\ncentres.\nMethods: A working group of internationally-representative JDM experts was formed to develop a provisional\nminimal dataset. Clinical and laboratory variables contained within current national and international collaborative\ndatabases of patients with idiopathic inflammatory myopathies were scrutinised. Judgements were informed by\npublished literature and a more detailed analysis of the Juvenile Dermatomyositis Cohort Biomarker Study and\nRepository, UK and Ireland.\nResults: A provisional minimal JDM dataset has been produced, with an associated glossary of definitions. The\nprovisional minimal dataset will request information at time of patient diagnosis and during on-going prospective\nfollow up. At time of patient diagnosis, information will be requested on patient demographics, diagnostic criteria\nand treatments given prior to diagnosis. During on-going prospective follow-up, variables will include the presence\nof active muscle or skin disease, major organ involvement or constitutional symptoms, investigations, treatment,\nphysician global assessments and patient reported outcome measures.\nConclusions: An internationally agreed minimal dataset has the potential to significantly enhance collaboration,\nallow effective communication between groups, provide a minimal standard of care and enable analysis of the\nlargest possible number of JDM patients to provide a greater understanding of this disease. This preliminary dataset\ncan now be developed into a consensus-approved minimum core dataset and tested in a wider setting with the\naim of achieving international agreement.
Loading....