Pediatric rheumatology

Predictors of persistent active disease and the longitudinal development of organ damage, patient-reported outcomes and autoantibodies in long-term juvenile dermatomyositis

RMD OpenPublished Longitudinal cohort study

Authors' conclusion

After a median of 21.7 years from symptom onset, the majority of JDM patients still had active disease. Higher organ damage and disease activity at the initial visit predicted persistent active disease at follow-up.

Study context

Population
Patients with juvenile dermatomyositis (JDM) after long-term follow-up65% female
Participants
40 patients
Outcome
Persistent active disease (updated PRINTO criteria) and its predictors
Follow-up
Visit 2
Methodological detailstwo time points (visits 1 and 2)
  • Two time points (visits 1 and 2)

Main finding

As reported in the abstract

Active disease (PRINTO)

Active disease (PRINTO)

53%

Observational data: associations, not effects.

Further findings

Impaired physical function (CHAQ/HAQ>0) · visit 2

Impaired physical function (CHAQ/HAQ>0)
40%

Organ damage (MDItotal≥1) · visit 2

Organ damage (MDItotal≥1)
95%

Myositis specific or associated antibodies · visit 2

Myositis specific or associated antibodies
33%

Disease activity (Juvenile DermatoMyositis Activity Index (JDMAI)) · visit 2

Low
24%
Moderate
8%
High
3%

Organ damage and disease activity, visit 1 vs visit 2

No significant differences between visits

Median disease duration from symptom onset

Visit 1 (years)
15.1 (2.0-34.6)
Visit 2 (years)
21.7 (7.6-42.7)

Original publication

Predictors of persistent active disease and the longitudinal development of organ damage, patient-reported outcomes and autoantibodies in long-term juvenile dermatomyositis. RMD Open. Published 5 June 2025. doi:10.1136/rmdopen-2025-005598

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