Pediatric rheumatology

Clinical characteristics and prognostic factor in juvenile dermatomyositis: data of the Spanish registry

Pediatr Rheumatol Online JPublished Retrospective cohort study

Main findingResult — go to the resultHazard ratio per month of evolution 0.95

Authors' conclusion

We describe heliotrope rash as a risk factor for developing complications in our cohort of JDM patients

Study context

Population
Juvenile Dermatomyositis (JDM) patients included in the Spanish JDM registry, 17 Spanish paediatric rheumatology centres76 girls (65%)
Participants
116 patients
Outcome
Epidemiological, clinical and laboratory characteristics at diagnosis; prognostic factors for complications and time to disease inactivity
Methodological detailsregistry-based, multicentre, retrospective review
  • Registry-based
  • Multicentre
  • Retrospective review

Main finding

As reported in the abstract

Hazard ratio per month of evolution

0.95

P = 0.02

Girls
76 (65%)
Median age at diagnosis, years (IQR)
7.3 (4.5-10.2)
Pathognomonic skin lesions at onset
All patients
Muscle weakness
86.2%
Median Childhood Muscle Assessment Scale (IQR)
34 (22-47)
Dysphagia
12 (34%)
Calcinosis
29
Macrophage activation syndrome
4
Reached inactivity; median time, months (IQR)
70%; 8.9 (4.5-34.8)
Relapsed after inactivity; median time, months (IQR)
41%; 14.4 (8.6-22.8)

Observational data: associations, not effects.

Further findings

OutcomeHRP value
Prognostic factorsShorter time to treatment associated with better prognosis0.95P = 0.02

Prognostic factors

Heliotrope rash at diagnosis correlates with higher risk of developing complications

Parts of this Brief are withheld: the automatic checks could not confirm them against the abstract.

Original publication

Clinical characteristics and prognostic factor in juvenile dermatomyositis: data of the Spanish registry. Pediatr Rheumatol Online J. Published 22 July 2024. doi:10.1186/s12969-024-00999-9

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