Evaluation of Severity in Juvenile and Adult-onset Dermatomyositis

Dermatomyositis Clinical Trial

— JADE  

Official title:

Evaluation of Severity in Juvenile Dermatomyositis and Adult-onset Dermatomyositis: a National Multicentric Retrospective Study

Clinical Trial Details — Status: Not yet recruiting

Administrative data

• NCT number: NCT06004817
• Other study ID #: 2023PI150
• Status: Not yet recruiting
• Start date: October 1, 2023
• Est. completion date: September 30, 2024

Study information

• Verified date: August 2023
• Source: Central Hospital, Nancy, France
• Contact: Paul Decker, MD
• Phone: +33383157240
• Email: p.decker[@]chru-nancy.fr
• Is FDA regulated: No
• Study type: Observational

Clinical Trial Summary

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.

Recruitment information / eligibility

• Status: Not yet recruiting
• Enrollment: 200
• Est. completion date: September 30, 2024
• Est. primary completion date: September 30, 2024
• Gender: All
• Age group: N/A and older

Eligibility

Inclusion Criteria:

• Patient with dermatomyositis according to 2017 American College of Rheumatology (ACR)/ European Alliance of Associations for Rheumatology (EULAR) classification

Exclusion Criteria:

• Patient with cancer-associated dermatomyositis (within 3 years before or after diagnosis of dermatomyositis)

• Patient with antisynthetase syndrome

Related Conditions & MeSH terms

• Dermatomyositis

Intervention

Other:
• evaluation of clinical severity
evaluation of clinical severity

Locations

Country	Name	City	State
n/a

Sponsors (1)

Lead Sponsor	Collaborator
Central Hospital, Nancy, France

Outcome

Type	Measure	Description	Time frame	Safety issue
Primary	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)
Secondary	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)
Secondary	number of patients with pulmonary severity	presence of symptomatic ILD	baseline (J0)
Secondary	number of patients with digestive severity	presence of digestive vasculitis: digestive bleeding and/or vasculitis on CT-scan	baseline (J0)
Secondary	number of patients with cutaneous severity	presence of severe skin ulcerations	baseline (J0)
Secondary	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
Secondary	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
Secondary	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