Comparison of data from the juvenile dermatomyositis national (UK & Ireland) cohort biomarker study and repository for idiopathic inflammatory myopathies with a survey of current practice throughout the UK and Ireland

Results Fifteen of 16 Paediatric Rheumatology centres responded to the survey. For initial treatment of patients with mild disease 13/15 centres report using a combination of steroids and methotrexate and 8/15 would also use IV methylprednisolone. For moderately severe disease all centres use steroids and methotrexate and 11/15 also use IV methylprednisolone. With severe weakness, ulceration or systemic disease all centres use IV methylprednisolone as initial treatment, 13/15 use methotrexate and 9/15 use cyclophosphamide. Prospective data were analysed from 98 patients in the UK JDM cohort study. A combination of steroids and methotrexate was used in the first year of treatment in 78% of patients with mild disease (PGA 03.3cm), 89% of patients with moderately severe disease (PGA 3.4-6.7cm) and 100% of patients with severe disease (PGA 6.8-10cm). Cyclophosphamide was used within the first year of diagnosis in 16% of patients with mild disease at presentation, 19% of patients with moderately severe disease and 38% of those with severe disease (p=0.05). There was no significant association between severity of disease at diagnosis and the use of intravenous steroids.