Comparison of disease severity and activity between juvenile- and adult-onset dermatomyositis: a multicentre cohort study
Authors' conclusion
Severity profiles differ by age of DM onset. Autoantibodies are key indicators of severity at diagnosis in both juvenile DM and adult DM.
Study context
- Population
- Patients with DM fulfilling the 2017 ACR/EULAR criteria and tested for myositis-specific antibodies
- Participants
- 201 patients
- Exposure
- Juvenile-onset DM vs adult-onset DM
- Outcome
- Overall severity at diagnosis (≥1 of: severe muscle impairment, symptomatic ILD, GI vasculitis, myocarditis, severe skin ulceration or ICU admission) and disease activity during follow-up
Main finding
As reported in the abstract
Severe disease at diagnosis
- Severe disease at diagnosis present in 44% of cases
- Symptomatic interstitial lung disease (ILD) more common in adults
- Gastrointestinal (GI) vasculitis more common in children
- No difference in severe muscle disease
Observational data: associations, not effects.
Further findings
Factors associated with severe disease at diagnosis in jDM (subgroup)
| OR (CI) | P value | ||
|---|---|---|---|
| Anti-NXP2 Abs | 6.29 (2.00–23.07) | P = 0.003 | |
| Joint involvement | 3.44 (1.33–11.47) | P = 0.03 | |
| 0.050.21520Odds ratio (log scale) |
Anti-Mi2 antibodies (Abs) and severe disease at diagnosis in aDM (subgroup)
| OR (CI) | P value | ||
|---|---|---|---|
| Anti-Mi2 AbsLower likelihood of severe disease | 0.12 (0.02–0.42) | P = 0.003 | |
| 0.050.21520Odds ratio (log scale) |
Clinically inactive disease or remission · last follow-up
- No significant difference between jDM and aDM
Disease features
- Female predominance less pronounced and GI involvement more frequent in jDM
- Anti-NXP2 Abs more frequent in jDM
Safety
- Severe infections occurred in 25% of patients
Parts of this Brief are withheld: the automatic checks could not confirm them against the abstract.
Original publication
Comparison of disease severity and activity between juvenile- and adult-onset dermatomyositis: a multicentre cohort study. Rheumatology (Oxford). Published 29 January 2026. doi:10.
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