Abstract

BackgroundThe aim was to develop prediction rules that may guide early treatment decisions based on baseline clinical predictors of long-term unfavorable outcome in juvenile idiopathic arthritis (JIA).MethodsIn the Nordic JIA cohort, we assessed baseline disease characteristics as predictors of the following outcomes 8 years after disease onset. Non-achievement of remission off medication according to the preliminary Wallace criteria, functional disability assessed by Childhood Health Assessment Questionnaire (CHAQ) and Physical Summary Score (PhS) of the Child Health Questionnaire, and articular damage assessed by the Juvenile Arthritis Damage Index-Articular (JADI-A). Multivariable models were constructed, and cross-validations were performed by repeated partitioning of the cohort into training sets for developing prediction models and validation sets to test predictive ability.ResultsThe total cohort constituted 423 children. Remission status was available in 410 children: 244 (59.5%) of these did not achieve remission off medication at the final study visit. Functional disability was present in 111/340 (32.7%) children assessed by CHAQ and 40/199 (20.1%) by PhS, and joint damage was found in 29/216 (13.4%). Model performance was acceptable for making predictions of long-term outcome. In validation sets, the area under the curves (AUCs) in the receiver operating characteristic (ROC) curves were 0.78 (IQR 0.72–0.82) for non-achievement of remission off medication, 0.73 (IQR 0.67–0.76) for functional disability assessed by CHAQ, 0.74 (IQR 0.65–0.80) for functional disability assessed by PhS, and 0.73 (IQR 0.63–0.76) for joint damage using JADI-A.ConclusionThe feasibility of making long-term predictions of JIA outcome based on early clinical assessment is demonstrated. The prediction models have acceptable precision and require only readily available baseline variables. Further testing in other cohorts is warranted.

Highlights

  • The aim was to develop prediction rules that may guide early treatment decisions based on baseline clinical predictors of long-term unfavorable outcome in juvenile idiopathic arthritis (JIA)

  • In the Nordic JIA cohort, we studied prediction of four established and validated outcomes, and aimed to construct prediction models that may aid decision on early aggressive treatment

  • Disease onset was defined as the time of presentation of symptoms of active arthritis, and the JIA categories were determined according to the International League of Associations for Rheumatology (ILAR) criteria [14]

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Summary

Introduction

The aim was to develop prediction rules that may guide early treatment decisions based on baseline clinical predictors of long-term unfavorable outcome in juvenile idiopathic arthritis (JIA). Juvenile idiopathic arthritis (JIA) is a heterogeneous childhood disease, with chronic joint inflammation as the common feature. The JIA categories differ by the number of joints affected, and the presence of extra-articular involvement [1]. Disease course and prognosis differ between JIA categories, but there is large variability within each. Prediction of the disease course for the individual child can facilitate tailored treatment. There is increasing evidence for the concept of “the window of therapeutic opportunity” in JIA, where early aggressive treatment with biologic agents and/or other disease-modifying anti-rheumatic drugs (DMARDs) may modify the disease course and improve long-term prognosis [7,8,9]. It is Rypdal et al Arthritis Research & Therapy (2018) 20:91 essential to avoid unnecessary, costly, and potentially toxic treatment in children with a favorable prognosis

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