Dermatomyositis (DM) are rare and heterogeneous systemic autoimmune diseases, characterized by the association of muscle inflammation, skin inflammation and vasculopathy. DM concern both adults and children. DM can be life-threatening (interstitial lung disease, infectious complications) and responsible of significant functional disability (muscle weakness). Age of onset appear to be an independent prognostic factor. Juvenile-onset DM is characterized by a higher frequency of calcinosis, skin ulceration and digestive vasculitis. In adults, interstitial lung disease and cancer are more frequent with higher mortality. Data concerning the comparison of the initial severity between juvenile and adult-onset DM are limited. The main objective is to compare global severity between juvenile DM and adult-onset DM at initial diagnosis. Secondary objectives are: * to compare organ-specific severity between juvenile DM and adult-onset DM at diagnosis. * to compare damage during follow-up and at last follow-up between juvenile DM and adult-onset DM. * to compare activity at the last follow-up between juvenile DM and adult-onset DM. * to compare iatrogenic complications between juvenile DM and adult-onset DM.
Study Type
OBSERVATIONAL
Enrollment
200
evaluation of clinical severity
Chu Nancy
Nancy, France
RECRUITINGnumber of patients with global severity
presence of at least one criteria among: severe muscle disease (Childhood Myositis Assessment Scale \- CMAS - score \< 15, and/or Manual Muscle Testing 8 - MMT8 - score \< 30, and/or Medical Research Council - MRC - muscle testing \< 3, and/or dysphagia and/or swallowing difficulties), symptomatic interstitial lung disease (ILD), digestive vasculitis (digestive bleeding and/or vasculitis on CT-scan), myocarditis on cardiac MRI, severe skin ulcerations, intensive care unit admission
Time frame: baseline (J0)
number of patients with muscular severity
presence of at least one of the following criteria: CMAS score \< 15, MMT8 score \< 30, MRC muscle testing \< 3, dysphagia, swallowing difficulties
Time frame: baseline (J0)
number of patients with pulmonary severity
presence of symptomatic ILD
Time frame: baseline (J0)
number of patients with digestive severity
presence of digestive vasculitis: digestive bleeding and/or vasculitis on CT-scan
Time frame: baseline (J0)
number of patients with cutaneous severity
presence of severe skin ulcerations
Time frame: baseline (J0)
myositis damage index (MDI) score
myositis damage index (MDI) extent of damage score: from 0 (better outcome) to 38 (worse outcome)
Time frame: 2 years of follow-up, at 5 years of follow-up and at last follow-up
number of patients with remission at last follow-up
absence of disease activity without any immunosuppressive/immunomodulatory treatment for at least 2 years
Time frame: up to 10 years
number of patients with disease activity at last follow-up
presence of at least one of the following criteria: elevated creatinine kinase (CK) level and/or recent muscle testing deterioration and/or muscle inflammation on MRI, and/or skin manifestations and/or progressive ILD
Time frame: up to 10 years
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