title: Developing a provisional, international minimal dataset for Juvenile Dermatomyositis: for use in clinical practice to inform research.
abstract: BACKGROUND
  Juvenile dermatomyositis (JDM) is a rare but severe autoimmune inflammatory myositis of childhood. International collaboration is essential in order to undertake clinical trials, understand the disease and improve long-term outcome. The aim of this study was to propose from existing collaborative initiatives a preliminary minimal dataset for JDM. This will form the basis of the future development of an international consensus-approved minimum core dataset to be used both in clinical care and inform research, allowing integration of data between centres.
  
  
  METHODS
  A working group of internationally-representative JDM experts was formed to develop a provisional minimal dataset. Clinical and laboratory variables contained within current national and international collaborative databases of patients with idiopathic inflammatory myopathies were scrutinised. Judgements were informed by published literature and a more detailed analysis of the Juvenile Dermatomyositis Cohort Biomarker Study and Repository, UK and Ireland.
  
  
  RESULTS
  A provisional minimal JDM dataset has been produced, with an associated glossary of definitions. The provisional minimal dataset will request information at time of patient diagnosis and during on-going prospective follow up. At time of patient diagnosis, information will be requested on patient demographics, diagnostic criteria and treatments given prior to diagnosis. During on-going prospective follow-up, variables will include the presence of active muscle or skin disease, major organ involvement or constitutional symptoms, investigations, treatment, physician global assessments and patient reported outcome measures.
  
  
  CONCLUSIONS
  An internationally agreed minimal dataset has the potential to significantly enhance collaboration, allow effective communication between groups, provide a minimal standard of care and enable analysis of the largest possible number of JDM patients to provide a greater understanding of this disease. This preliminary dataset can now be developed into a consensus-approved minimum core dataset and tested in a wider setting with the aim of achieving international agreement.
authors: Alder Hey Children's NHS Foundation Trust, Eaton Road, Liverpool L12 2AP, UK.
authors: Liza J
authors: LJ
authors: McCann
authors: Rheumatology Unit, UCL Institute of Child Health, University College London, London, UK.
authors: Katie
authors: K
authors: Arnold
authors: Rheumatology Unit, UCL Institute of Child Health, University College London, London, UK ; Great Ormond Street Hospital, London, UK.
authors: Clarissa A
authors: CA
authors: Pilkington
authors: IWK Health Centre and Dalhousie University, 5850 University Avenue, Halifax, NS B3K 6R8, Canada.
authors: Adam M
authors: AM
authors: Huber
authors: Università degli Studi di Genova and Istituto Giannina Gaslini, Largo G. Gaslini 5, 16147 Genoa, Italy.
authors: Angelo
authors: A
authors: Ravelli
authors: Rheumatology Unit, UCL Institute of Child Health, University College London, London, UK.
authors: Laura
authors: L
authors: Beard
authors: Alder Hey Children's NHS Foundation Trust, Eaton Road, Liverpool L12 2AP, UK ; Department of Women's and Children's Health, Institute of Translational Medicine, University of Liverpool, Liverpool, UK.
authors: Michael W
authors: MW
authors: Beresford
authors: Rheumatology Unit, UCL Institute of Child Health, University College London, London, UK ; Centre for Adolescent Rheumatology at University College London, University College London Hospital, London, UK ; Great Ormond Street Hospital, London, UK.
authors: Lucy R
authors: LR
authors: Wedderburn
mesh_terms: D015415:Biomarkers / Q000032:analysis; D002170:Canada; D002648:Child; D003882:Dermatomyositis / Q000145:classification / Q000175:diagnosis / Q000503:physiopathology / Q000628:therapy; D019468:Disease Management; D018450:Disease Progression; D005260:Female; D006801:Humans; D007391:International Cooperation; D007494:Ireland; D007558:Italy; D008297:Male; D017063:Outcome Assessment, Health Care; D062072:Patient Acuity; D006113:United Kingdom
chemical_list: D015415:Biomarkers
keywords: Collaboration; Core set; Dataset; Disease activity; Idiopathic Inflammatory myopathy; International; Juvenile dermatomyositis