Evaluation of Severity in Juvenile and Adult-onset Dermatomyositis (JADE)

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

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 Overview

• Status: Recruiting
• Conditions: Dermatomyositis
• Intervention / Treatment: Other: evaluation of clinical severity

• Study Type: Observational
• Enrollment (Estimated): 200

Contacts and Locations

• Study Contact: Name: Paul DECKER, MD; Phone Number: +33383157240; Email: p.decker@chru-nancy.fr

Study Locations

• France
  • Nancy, France
    • Recruiting
    • CHU Nancy
    • Contact: Paul DECKER, MD

Participation Criteria

Eligibility Criteria

• Ages Eligible for Study: Child; Adult; Older Adult
• Accepts Healthy Volunteers: N/A

• Sampling Method: Probability Sample

Study Population

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

Description

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

Study Plan

How is the study designed?

Number of groups / cohorts

2

Cohorts and Interventions

Group / Cohort	Intervention / Treatment
Juvenile dermatomyositis; Dermatomyositis before 18 years-old	Other: evaluation of clinical severity; evaluation of clinical severity
Adult-onset dermatomyositis; Dermatomyositis from 18 years-old	Other: evaluation of clinical severity; evaluation of clinical severity

What is the study measuring?

Primary Outcome Measures

Outcome Measure	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)

Secondary Outcome Measures

Outcome Measure	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	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

Collaborators and Investigators

• Sponsor: Central Hospital, Nancy, France
• Investigators: Principal Investigator: Paul Decker, MD, CHU Nancy

Study record dates

Study Major Dates

• Study Start (Actual): October 1, 2023
• Primary Completion (Estimated): September 30, 2024
• Study Completion (Estimated): September 30, 2024

Study Registration Dates

• First Submitted: August 8, 2023
• First Submitted That Met QC Criteria: August 16, 2023
• First Posted (Actual): August 22, 2023

Study Record Updates

• Last Update Posted (Actual): August 27, 2024
• Last Update Submitted That Met QC Criteria: August 26, 2024
• Last Verified: August 1, 2024

More Information

Terms related to this study

• Additional Relevant MeSH Terms: Nervous System Diseases; Skin Diseases; Musculoskeletal Diseases; Connective Tissue Diseases; Muscular Diseases; Neuromuscular Diseases; Polymyositis; Myositis; Dermatomyositis
• Other Study ID Numbers: 2023PI150

Plan for Individual participant data (IPD)

• Plan to Share Individual Participant Data (IPD)?: NO

Drug and device information, study documents

• Studies a U.S. FDA-regulated drug product: No
• Studies a U.S. FDA-regulated device product: No