Modified Juvenile Spondyloarthritis Disease Activity Index in the Childhood Arthritis and Rheumatology Research Alliance (CARRA) Registry

Abstract

ObjectiveTo validate the Juvenile Spondyloarthritis Disease Activity (JSpADA) index, and modified versions thereof, in a North American cohort of patients with enthesitis-related arthritis (ERA).MethodsWe utilized the Childhood Arthritis and Rheumatology Research Alliance (CARRA) Registry database ERA cohort to validate the JSpADA and its modifications (JSpADA6-no Schober, no CRP/ESR; JSpADA7- no Schober; and JSpADA7- no CRP/ESR) using OMERACT principles of face validity, discriminative validity, and responsiveness to change.ResultsThere were 51 subjects (64 visits) with complete JSpADA data with a mean age of 13.7 years and disease duration of 30.9 months. Subjects were predominantly white (84.3%) with half of them, male (56.9%), and HLA-B27 positive (50%). The JSpADA showed high correlation with the cJADAS-10 (r=0.81), moderate-to-high correlation with physician global (r=0.69), and low-to-fair correlation with CHAQ (0.22). The modifications of JSpADA (JSpADA7- no Schober, JSpADA7-no CRP/ESR, and JSpADA6 - no Schober, no CRP/ESR) performed similarly with high correlation with cJADAS-10 (r=0.81, 0.79, and 0.80, respectively), moderateto- high correlation with physician global (r=0.65, 0.67, 0.64), and low-to-fair correlation with CHAQ (r= 0.35, 0.34, 0.39). All modified versions of JSpADA had good responsiveness to change. All versions of JSpADA had excellent discriminative validity.ConclusionWe propose the term "modified JSpADA" for the modification of JSpADA with 6 elements (JSpADA6 - no Schober, no CRP/ESR). This shorter disease activity index may improve implementation of JSpADA in both clinical practice and research trials.

Back to the Future: Is the Schober Test Dispensable in Juvenile Spondyloarthritis?

“…In summary, the current study 9 shows that modifying the JSpADA with exclusion of the mSchober test and inflammatory marker requirement did not impair its performance in a population of children with SpA, while increasing the number of children available for evaluation. Although CRP remains an important disease activity biomarker, the value of the mSchober test in children is uncertain, especially in the setting of a clinical trial.…”

“…The current edition of The Journal of Rheumatology includes an assessment of the JSpADA in which either or both of the most frequently missed items, measurement of back mobility and inflammatory markers (ie, C-reactive protein [CRP], erythrocyte sedimentation rate [ESR]), are excluded. 9 To do this, Srinivasalu and colleagues reviewed 1961 visits of patients with SpA captured in the Childhood Arthritis and Rheumatology Research Alliance (CARRA) Registry, a multicenter observational pediatric rheumatology disease registry inclusive of, although not limited to, all of the juvenile idiopathic arthritis categories. 10 They collected data on patients with SpA with complete data for the entire JSpADA, as well as patients missing only the modified Schober (mSchober) test result ( JSpADA7 -no Schober), patients missing only an inflammatory marker ( JSpADA7 -no CRP/ ESR), and patients lacking both a mSchober and an inflammatory marker ( JSpADA6).…”

Drug therapy in juvenile spondyloarthritis

“…Most of the studies use JADAS or ACR response as outcome measures, which do not capture all the disease components of JSpA such as enthesitis or axial disease. In 2014, Weiss et al [39,40 ▪ ] introduced a new measure called the Juvenile Spondyloarthritis Disease Activity Index (JSpADA), which in its original and modified form have been shown to be validated measures of disease activity specific to JSpA. Their use in clinical trials to determine response to therapy in this patient population is encouraged as was done in a post hoc analysis in the JUNIPERA trial [41].…”

Challenges in treating juvenile idiopathic arthritis

“…Since patients with Juvenile Spondyloarthritis (JSpA) (typically those with the JIA categories of ERA or psoriatic JIA) have a disease with features that are unique among patients with JIA, such as axial disease, a special monitoring tool, known as the Juvenile Spondyloarthritis Disease Activity Index (JSpADA) was developed and retrospectively validated by Weiss et al in 2014 [32]. A recent modification of the JSpADA with 6 instead of 8 items was validated in the CARRA Registry [33 ▪ ]. The hope is that this simpler, modified JSpADA may improve the uptake of the JSpADA in both clinical practice and research trials [33 ▪ ].…”

“…A recent modification of the JSpADA with 6 instead of 8 items was validated in the CARRA Registry [33 ▪ ]. The hope is that this simpler, modified JSpADA may improve the uptake of the JSpADA in both clinical practice and research trials [33 ▪ ].…”