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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:
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| Label | Type | Description | Intervention Names |
|---|---|---|---|
| Juvenile dermatomyositis | Dermatomyositis before 18 years-old |
| |
| Adult-onset dermatomyositis | Dermatomyositis from 18 years-old |
|
| Name | Type | Description | Arm Group Labels | Other Names |
|---|---|---|---|---|
| evaluation of clinical severity | Other | evaluation of clinical severity |
|
| Measure | Description | Time Frame |
|---|---|---|
| number 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 | baseline (J0) |
| Measure | Description | Time Frame |
|---|---|---|
| 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 | baseline (J0) |
| number of patients with pulmonary severity |
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Inclusion Criteria:
Exclusion Criteria:
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Patients followed in pediatric and adult units during January 2010 and December 2022
| Name | Role | Phone | Extension | |
|---|---|---|---|---|
| Paul Decker, MD | Contact | +33383157240 | p.decker@chru-nancy.fr |
| Name | Affiliation | Role |
|---|---|---|
| Paul Decker, MD | Central Hospital, Nancy, France | Principal Investigator |
| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| Chu Nancy | Recruiting | Nancy | France |
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| ID | Term |
|---|---|
| D003882 | Dermatomyositis |
| ID | Term |
|---|---|
| D017285 | Polymyositis |
| D009220 | Myositis |
| D009135 | Muscular Diseases |
| D009140 | Musculoskeletal Diseases |
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presence of symptomatic ILD
| baseline (J0) |
| number of patients with digestive severity | presence of digestive vasculitis: digestive bleeding and/or vasculitis on CT-scan | baseline (J0) |
| number of patients with cutaneous severity | presence of severe skin ulcerations | baseline (J0) |
| myositis damage index (MDI) score | myositis damage index (MDI) extent of damage score: from 0 (better outcome) to 38 (worse outcome) | 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 | 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 | up to 10 years |
| D009468 |
| Neuromuscular Diseases |
| D009422 | Nervous System Diseases |
| D003240 | Connective Tissue Diseases |
| D017437 | Skin and Connective Tissue Diseases |
| D012871 | Skin Diseases |