Pediatric rheumatology

Defining criteria for disease activity states in juvenile dermatomyositis based on the Juvenile Dermatomyositis Activity Index

RMD OpenPublished Validation study

Authors' conclusion

Both JDMAI1 and JDMAI2 cut-offs revealed good metrologic properties in validation analyses and are, therefore, suited for application in clinical practice and research.

Study context

Population
Children with juvenile dermatomyositis (JDM): 139 patients from an RCT for cut-off definition; two validation cohortsvalidation: 488 JDM patients
Participants
139 children
Comparison
JDMAI1 vs JDMAI2
Outcome
Juvenile DermatoMyositis Activity Index (JDMAI) cut-offs for inactive disease, low, moderate and high disease activity
Methodological detailspercentiles of score distribution, ROC curve analysis, validation in two cohorts
  • Percentiles of score distribution
  • ROC curve analysis
  • Validation in two cohorts

Performance

As reported in the abstract

Disease activity state cut-offs
Test or modelInactive disease (ID)Low disease activity (LDA)Moderate disease activity (MDA)High disease activity (HDA)
JDMAI1≤2.4≤6.66.7-11>11
JDMAI2≤5.2≤8.58.6-11.3>11.3

Inactive disease (ID)

JDMAI1≤2.4
JDMAI2≤5.2

Low disease activity (LDA)

JDMAI1≤6.6
JDMAI2≤8.5

Moderate disease activity (MDA)

JDMAI16.7-11
JDMAI28.6-11.3

High disease activity (HDA)

JDMAI1>11
JDMAI2>11.3

Further findings

Discriminative ability of cut-offs

Cut-offs discriminated strongly among physician- and parent-defined activity states, parent satisfaction, pain, fatigue, physical functional impairment and physical well-being

Original publication

Defining criteria for disease activity states in juvenile dermatomyositis based on the Juvenile Dermatomyositis Activity Index. RMD Open. Published 2 February 2024. doi:10.1136/rmdopen-2023-003093

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