Early-onset juvenile dermatomyositis: A tertiary referral center experience and review of the literature
Authors' conclusion
Compared to older-onset JDM patients, this group had higher relapse rate, more severe muscle biopsy findings, and received intensive immunosuppressive treatment.
Study context
- Population
- Children with juvenile dermatomyositis (JDM) followed between December 2010 and April 2022 at a tertiary referral centreplus 75 literature patients
- Participants
- 9 vs 63
- Exposure
- Early-onset JDM vs older-onset JDM
- Early-onsetn = 9
- Older-onsetn = 63reference
- Outcome
- Clinical features and course, early- vs older-onset
Methodological detailssingle tertiary referral centre, literature review of PubMed/MEDLINE and Scopus
- Single tertiary referral centre
- Literature review of PubMed/MEDLINE and Scopus
Main finding
As reported in the abstract
- No difference in partial and complete remission rates
- Early-onset vs Older-onset: P = 0.005
- Early-onset vs Older-onset: P = 0.006
- Early-onset vs Older-onset: P = 0.049
- Early-onset vs Older-onset: P < 0.001
- Early-onset vs Older-onset: P = 0.001
- Early-onset vs Older-onset: P = 0.001
- Early-onset vs Older-onset: P = 0.011
- Early-onset vs Older-onset: P = 0.016
Observational data: associations, not effects.
Further findings
Muscle biopsy in the diagnostic pathway
- Muscle biopsy, early-onset vs older-onset JDM
- 77.7% versus 50.8%
Literature review of early-onset JDM (32 articles, 75 patients)
- Median diagnostic delay (range), months
- 5 (1-30)
- Calcinosis
- 29.5%
- Muscle biopsy, 23 of 44 patients
- 52.3%
- Second- and third-line treatments, 41 patients
- 64.1%
- Complete remission
- 48.9%
- Relapse
- 75%
- Mortality rate
- 10.2%
Original publication
Early-onset juvenile dermatomyositis: A tertiary referral center experience and review of the literature. Semin Arthritis Rheum. Published 19 November 2022. doi:10.
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