Pediatric rheumatology

Longitudinal assessment of disease activity and muscle strength in juvenile dermatomyositis: a multicentre registry study

Clin Exp RheumatolPublished Prospective multicentre registry cohort study

Authors' conclusion

DAS20 scores improved gradually during follow up, MMT8 improved significantly during the first 6 months and CMAS, CHAQ-DI and parents VAS scores had no significant improvement with persistent functional impairment over 2-years.

Study context

Population
New-onset juvenile dermatomyositis (JDM) patients enrolled in a registry at 18 hospitals, 2015–201861 (64%) females
Participants
96 patients
Outcome
Disease activity (DAS20, skin and musculoskeletal subscales), muscle strength (Manual Muscle Test (MMT8), CMAS) and function (CHAQ-DI, VAS wellbeing)
Follow-up
Baseline, 6, 12, 18 and 24 months
Methodological details18 hospitals, repeated measures (Poisson model, Wald post-test)
  • 18 hospitals
  • Repeated measures (Poisson model, Wald post-test)

Main finding

As reported in the abstract

Measured values over follow-up (baseline, then 6-month intervals) · diagnosis to 24 months
Disease Activity Score (DAS20) (0-20), mean ±SD at diagnosis and 6-month intervals
7.8±5, 6.3 ±4.8, 5±4, 4.9 ±5 and 0.5 ±2.3; significant difference from baseline (p<0.01)
Skin DAS subscale, mean ±SD
2.8±3.3, 1.8±2.9, 1,1±2.2, 0.6±1.8, 0.4±1.5
MMT (0-80), mean ±SD
62.6±20.4, 70.2±13.5, 73.3±11, 75.7±7.9 and 74.8±7.8; significant difference from baseline up to 6 months (p=0.016)
Childhood Myositis Assessment Scale (CMAS) (0-53), mean ±SD
29.5±11.4, 33.1±8.3, 34.2±5.8, 34±6 and 33.3±5.4
CHAQ-DI (0-3), mean ±SD
1±0.9, 0.6±0.7, 0.8±0.8, 1±0.8 and 1±0.3
Parents Visual Analog Scale (VAS), mean ±SD
4.1±2.5, 2±2.1; 1.3±2.8, 4.1±3.1, 1.7±2.2

Observational data: associations, not effects.

Further findings

OutcomeValues
Onset calcinosis · diagnosisJDM 13%

Original publication

Longitudinal assessment of disease activity and muscle strength in juvenile dermatomyositis: a multicentre registry study. Clin Exp Rheumatol. Published 9 March 2024. doi:10.55563/clinexprheumatol/yrb7m2

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