Development and Retrospective Validation of the Juvenile Spondyloarthritis Disease Activity Index

Abstract

Objectives To develop and validate a Juvenile Spondyloarthritis (JSpA) Disease Activity (JSpADA) index for use in clinical practice and research. Methods Using modified Delphi consensus techniques, ten items were selected by participants in the international pediatric rheumatology list-serve, the Childhood Arthritis and Rheumatology Research Alliance, and the list-serve for the Pediatric Section of the American College of Rheumatology. Validation was performed in a retrospective multicenter cohort of 243 children. Results 106 physicians representing 14 countries completed the initial questionnaire. Completion rates for the subsequent questionnaires were 84%, 75%, and 77% of the original respondents. Ten items reached 80% consensus: arthritis, enthesitis, patient pain assessment, inflammatory markers, morning stiffness, clinical sacroiliitis, uveitis, back mobility, and patient and physician assessments of disease activity. Two items were eliminated after item analysis (patient and physician assessments of disease activity). Factor analysis identified 3 primary domains that explain 58% of variance: peripheral disease, axial disease, and uveitis. Cronbach α coefficient was 0.66. The JSpADA had high or moderate correlations with the Juvenile Arthritis disease activity score (r=0.80), patient and physician assessments of disease activity (r=0.70 and 0.66), and the Childhood Health Assessment Questionnaire (r=0.56). The JSpADA discriminated well between subjects with active versus inactive disease (p<0.001) and was responsive to improvement or worsening in disease activity over time (p<0.001). Conclusion Using international input and consensus formation techniques, we developed and validated the first disease activity assessment for JSpA. Future studies should validate the JSpADA index in a prospective multi-center cohort.

The Increased Levels of Fecal Calprotectin in Children With Active Enthesitis Related Arthritis and MRI Signs of Sacroiliitis: The Results of a Single Center Cross-Sectional Exploratory Study in Juvenile Idiopathic Arthritis Patients

“…The novel finding of our study was that fCAL concentration was significantly higher in ERA patients with MRI sign(s) characteristic for the SlJ inflammation, which adds to the growing number of evidences for a clinical association of gut inflammation and axial spondyloarthritis in adult and pediatric patients ( 40 – 43 ). Moreover, to best of our knowledge, our study was the first to correlate the fCAL concentration with somewhat novel disease activity scores, such as JADAS-27 and jSpADA, with the results showing significantly higher fCAL concentration in ERA patients with active disease ( 33 , 34 ). Ultimately, the highest level of fCAL was observed in group of ERA patients with active disease and MRI sign(s) of sacroiliitis, suggesting that gastrointestinal inflammation might be a part of a wider inflammatory process present in patients with ERA.…”

The Increased Levels of Faecal Calprotectin in Children With Active Enthesitis Related Arthritis and Mri Signs of Sacroiliitis: the Results of A Single Centre Cross-Sectional Study in Juvenile Idiopathic Arthritis Patients

“…With this in mind, our study for the rst time gives some clue that the concomitant in ammation of the gut and sacroiliac joints can be present early in the course of SpA. Moreover, to best of our knowledge, our study was the rst to correlate the fCAL concentrations with somewhat novel disease activity scores, such as JADAS and jSpADA (27,28). The nal version of the JSpADA consists of eight items which re ect the status of uveitis, peripheral and axial disease, but as might be expected, none of the items involves the GI status (28).…”

The Juvenile Spondyloarthritis Disease Activity Index Is a Useful Tool in Enthesitis-Related Arthritis

“…The reliability of JSpADA developed for this purpose was validated in a cohort of 597 patients. 26 Recently, Zanwar et al 27 reported that JSpADA was highly correlated with physician and patient global assessment and JADAS7 and that JSpADA is a valid score for measuring disease activity in ERA. In the present study, when the groups with and without inactive disease at 6 months were compared, there was no difference in the baseline JSpADA scores of both groups, whereas the median JSpADA at 3 months was 1.5 in the active group and 0.5 in the inactive group.…”

“…However, both measurement tools lack parameters to assess axial involvement, which is more specific to ERA. The reliability of JSpADA developed for this purpose was validated in a cohort of 597 patients 26 . Recently, Zanwar et al 27 reported that JSpADA was highly correlated with physician and patient global assessment and JADAS7 and that JSpADA is a valid score for measuring disease activity in ERA.…”