Evaluation of Severity in Juvenile and Adult-onset Dermatomyositis

NCT06004817 | Recruiting

• 1 other identifier: 2023PI150
• Study Type: observational
• Target: 200
• Locations: 1 country
• Sites: 1

Brief 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.

Conditions

Dermatomyositis

Outcome Measures

Primary Outcomes (1)

• 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 Outcomes (7)

• number of patients with muscular severity

  baseline (J0)

• number of patients with pulmonary severity

  baseline (J0)

• number of patients with digestive severity

  baseline (J0)

• number of patients with cutaneous severity

  baseline (J0)

• myositis damage index (MDI) score

  2 years of follow-up, at 5 years of follow-up and at last follow-up

Study Arms (2)

Juvenile dermatomyositis

Dermatomyositis before 18 years-old

Other: evaluation of clinical severity

Adult-onset dermatomyositis

Dermatomyositis from 18 years-old

Other: evaluation of clinical severity

Interventions

evaluation of clinical severity OTHER

evaluation of clinical severity

Adult-onset dermatomyositis; Juvenile dermatomyositis

Eligibility Criteria

• Sex: all
• Age Groups: Child (0-17), Adult (18-64), Older Adult (65+)
• Sampling Method: Probability Sample

Study Population

Patients followed in pediatric and adult units during January 2010 and December 2022

You may qualify if:

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

You may not qualify if:

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

Sponsors & Collaborators

• Central Hospital, Nancy, France: lead

Study Sites (1)

Chu Nancy

Nancy, France

RECRUITING

MeSH Terms

Conditions

Dermatomyositis

Condition Hierarchy (Ancestors)

Polymyositis; Myositis; Muscular Diseases; Musculoskeletal Diseases; Neuromuscular Diseases; Nervous System Diseases; Connective Tissue Diseases; Skin and Connective Tissue Diseases; Skin Diseases

Study Officials

• Paul Decker, MD

  CHU NANCY

  PRINCIPAL INVESTIGATOR

Central Study Contacts

Paul Decker, MD

CONTACT

+33383157240; p.decker@chru-nancy.fr

Study Design

• Study Type: observational
• Observational Model: COHORT
• Time Perspective: RETROSPECTIVE
• Sponsor Type: OTHER
• Responsible Party: PRINCIPAL INVESTIGATOR
• PI Title: Principal Investigator

Study Record Dates

• First Submitted: August 8, 2023
• First Posted: August 22, 2023
• Study Start: October 1, 2023
• Primary Completion: September 30, 2024
• Study Completion: September 30, 2024
• Last Updated: August 27, 2024

Record last verified: 2024-08

Data Sharing

• IPD Sharing: Will not share

Locations

FR (1)