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Defining Criteria for Disease Activity States in Systemic Juvenile Idiopathic Arthritis Based on the Systemic
Silvia Rosina1, Ana I Rebollo-Giménez1, Letizia Tarantola2
1IRCCS Istituto Giannina Gaslini, Genoa, Italy.
Insights
New cutoff values for the systemic Juvenile Arthritis Disease Activity Score 10 (sJADAS10) effectively differentiate disease activity levels in children with systemic juvenile idiopathic arthritis, aiding clinical practice.
Area of Science:
- Pediatric Rheumatology
- Clinical Assessment
- Disease Activity Measurement
Background:
- Systemic juvenile idiopathic arthritis (sJIA) requires accurate disease activity assessment.
- The systemic Juvenile Arthritis Disease Activity Score 10 (sJADAS10) is a key metric.
- Establishing validated cutoffs is crucial for consistent interpretation.
Purpose of the Study:
- To develop and validate sJADAS10 cutoff values.
- To define thresholds for inactive disease (ID), minimal (MDA), moderate (MoDA), and high disease activity (HDA).
- To base these cutoffs on pediatric rheumatologist subjective assessments.
Main Methods:
- Utilized a cohort of 400 sJIA patients across 30 centers in 11 countries.
- Applied six statistical methods including ROC analysis and percentile calculations.
- Defined cutoffs using 60% of patients and validated them with the remaining 40%.
Main Results:
- Identified sJADAS10 cutoffs: ID/MDA ≤2.9, MDA/MoDA ≤10, MoDA/HDA >20.6.
- Demonstrated strong discrimination across pain levels and morning stiffness.
- Showed significant differentiation based on parental assessment of disease status and treatment satisfaction.
Conclusions:
- The developed sJADAS10 cutoffs exhibit robust metrologic properties.
- These validated cutoffs are suitable for both clinical trials and routine pediatric rheumatology practice.
- The findings support standardized assessment of sJIA activity.
Objective:
Our objective was to develop and validate cutoff values in the systemic Juvenile Arthritis Disease Activity Score 10 (sJADAS10) that distinguish the states of inactive disease (ID), minimal disease activity (MDA), moderate disease activity (MoDA), and high disease activity (HDA) in children with systemic juvenile idiopathic arthritis, based on subjective disease state assessment by the treating pediatric rheumatologist.
Methods:
The cutoff definition cohort was composed of 400 patients enrolled at 30 pediatric rheumatology centers in 11 countries. Using the subjective physician rating as an external criterion, six methods were applied to identify the cutoffs: mapping, calculation of percentiles of cumulative score distribution, the Youden index, 90% specificity, maximum agreement, and receiver operating characteristic curve analysis. Sixty percent of the patients were assigned to the definition cohort, and 40% were assigned to the validation cohort. Cutoff validation was conducted by assessing discriminative ability.
Results:
The sJADAS10 cutoffs that separated ID from MDA, MDA from MoDA, and MoDA from HDA were ≤2.9, ≤10, and >20.6, respectively. The cutoffs discriminated strongly among different levels of pain, between patients with and without morning stiffness, and among patients whose parents judged their disease status as remission or persistent activity or flare or were satisfied or not satisfied with current illness outcome.
Conclusion:
The sJADAS cutoffs revealed good metrologic properties in both definition and validation cohorts and are therefore suitable for use in clinical trials and routine practice.
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