Unmet Needs in Spondyloarthritis: Pathogenesis, Clinical Trial Design, and Nonpharmacologic Therapy.

A program focused on pathogenesis, clinical trial design, and nonpharmacologic mind-body therapy for of spondyloarthritis (SpA) was presented at the Spondylitis Association of America Unmet Needs Conference IV. SpA pathogenesis is incompletely understood but involves a complex set of drivers, including genetics, biomechanical stress, and microbial factors. Affected tissues may include axial and peripheral joints, entheses, skin, uvea, and intestines. The specific role of key cytokines like interleukin (IL)-23, IL-17, and tumor necrosis factor in the phases of this inflammatory process remains unclear. New insights into pathogenesis will continue to generate targets for novel therapeutics. How to optimally evaluate those therapeutics in clinical trials, and for the various manifestations of SpA, remains less clear. Future trials need better generalizability, robust subgroup analyses to assess differential responses for distinct disease manifestations, a focus on comparative efficacy, and outcomes relevant to the clinician and the patient. Additionally, study designs need to leverage available technology to facilitate subject participation in trials. In view of the interplay between biologic, physical, and psychological aspects of disease, there is increasing attention to nonpharmacologic agents, with the aim of maximizing long-term health-related quality of life through the control of symptoms and inflammation. Recent studies provide encouraging evidence that mind-body interventions such as tai chi, qigong, yoga, and meditation have benefits for patients with SpA, particularly those with pain. The advances in our understanding of pathogenesis, novel therapeutics, and nonpharmacologic interventions have revolutionized the management of SpA, but numerous questions around optimal management remain.

Prologue: Spondyloarthritis Unmet Research Needs Conference IV.

The Spondylitis Association of America (SAA) and the National Institute of Arthritis, Musculoskeletal and Skin Diseases (NIAMS) convened a conference on the campus of the National Institutes of Health (NIH) on September 28 and 29, 2023, to identify unmet needs in spondyloarthritis (SpA) research. The conference featured presentations by experts in areas of disease endotypes, pain, innovative imaging in SpA, health disparities in rheumatic diseases, and therapeutics. Members of the conference planning committee moderated the sessions and led the development of manuscripts summarizing recommendations to address unmet research needs. Early career investigators were invited to submit abstracts, which were presented at a networking session during the conference. Here, we highlight each of the sessions comprising the conference in the form of manuscripts published together as a conference summary.

Rheum for Improvement? Delayed Diagnosis of Juvenile Idiopathic Arthritis: A Narrative Review.

Juvenile idiopathic arthritis (JIA) is the most common rheumatic disease of childhood and a disease for which we have safe and effective therapies. Early diagnosis of JIA enables timely initiation of therapy and improves long-term disease outcomes. However, many patients with JIA experience prolonged diagnostic delays and have a turbulent course to diagnosis. In this narrative review, we explore the importance of early diagnosis in JIA, what is known about time to diagnosis and diagnostic trajectory, and factors that contribute to delayed diagnosis. We also discuss next steps to improve time to diagnosis for these vulnerable patients.

Classification Criteria for Axial Disease in Youth with Juvenile Spondyloarthritis.

OBJECTIVES: To develop and validate classification criteria for axial disease in youth with juvenile spondyloarthritis (SpA; AxJSpA). METHODS: This international initiative consisted of four phases: 1) Item generation; 2) Item reduction; 3) Criteria development; and 4) Validation of the AxJSpA criteria by an independent team of experts in an internationally representative Validation cohort. RESULTS: These criteria are intended to be used on youth with a physician diagnosis of juvenile SpA and for whom axial disease is suspected. Item generation consisted of a systematic literature review and a free-listing exercise using input from international physicians and collectively resulted in 108 items. After the item reduction exercise and expert panel input, 37 items remained for further consideration. The final AxJSpA criteria domains included: imaging: active inflammation, imaging: structural lesions, pain chronicity, pain pattern, pain location, stiffness, and genetics. The most heavily weighted domains were active inflammation and structural lesions on imaging. Imaging typical of sacroiliitis was deemed necessary, but not sufficient, to classify a youth with AxJSpA. The threshold for classification of AxJSpA was a score of ≥55 (out of 100). When tested in the validation data set, the final criteria had a specificity of 97.5% (95% CI: 91.4-99.7), sensitivity of 64.3% (95% CI: 54.9-73.1) and Area Under the Receiver Operating Characteristic (AUROC) curve of 0.81 (95% CI: 0.76-0.86). CONCLUSIONS: The new AxJSpA classification criteria require an entry criterion, physician diagnosis of juvenile SpA, and include seven weighted domains. The AxJSpA classification criteria are validated and designed to identify participants for research studies.

Association of Infant Breastfeeding and Juvenile Spondyloarthritis: A Case-Control Study.

OBJECTIVE: Given the multifactorial pathogenesis of juvenile spondyloarthritis (JSpA) and evidence of a protective effect in phenotypically similar diseases, we aimed to test whether breastfeeding is associated with the development and disease activity of JSpA. METHODS: This single-center retrospective case-control study included children with JSpA and age- and sex-matched controls with a 1:1 ratio. Univariable and multivariable conditional logistic regression modeling for matched pairs was used to test the association of infant factors with the development of JSpA, including infant nutrition and form of delivery. Linear regression was used to assess the association of JSpA disease activity (JSpA Disease Activity Index with 6 elements [JSpADA6]) at presentation with breastfeeding exposure, form of delivery, and antibiotic exposure. RESULTS: For the 195 case-control matched pairs, the mean age was 13.0 years and 47.7% were female. For breastfeeding, 88.7% of controls and 69.2% of JSpA cases were exposed to breastfeeding of any duration, respectively (P < 0.001). In the multivariable model, exclusive breastfeeding > 6 months was independently and significantly associated with a lower chance of JSpA development (odds ratio 0.47, 95% CI 0.30-0.72; P < 0.001). The median JSpADA6 was not significantly associated with breastfeeding for > 6 months. However, vaginal delivery was significantly associated with a lower JSpADA6 (B = -0.65, 95% CI -1.13 to -0.17; P = 0.008). CONCLUSION: This study suggests that infant factors that affect the microbiome may be associated with the occurrence and disease activity of JSpA at presentation.

Clinical features, treatment, and outcomes of celiac-associated arthritis: a retrospective cohort study.

BACKGROUND: Although arthritis is recognized as an extra-intestinal manifestation of celiac disease, little is known about the clinical course and outcomes of pediatric celiac-associated arthritis. This study describes clinical characteristics, treatments, and outcomes of children with celiac-associated arthritis. METHODS: This was a retrospective cohort study of children with celiac disease seen in pediatric rheumatology clinic between 2004 and 2021 for joint complaints. Data was abstracted from electronic health records. Patient demographics and clinical manifestations were evaluated using standard descriptive statistics. Physician- and patient-reported outcomes were evaluated at the index visit, 6-month follow-up, and last recorded visit, and were compared using Wilcoxon signed-rank tests. RESULTS: Twenty-nine patients with celiac disease were evaluated for joint complaints, and 13 were diagnosed with arthritis. Their mean age was 8.9 years (SD 5.9), and 61.5% were female. Celiac disease diagnosis was made before arthritis diagnosis in only 2 cases (15.4%). Initial testing that led to the celiac disease diagnosis was obtained by the rheumatologist in 6 cases (46.2%). Only 8 patients (61.5%) had concomitant GI symptoms, and of these, 3 patients had BMI z-scores <-1.64 and 1 had impaired linear growth. Arthritis presentation was most often oligoarticular (76.9%) and asymmetric (84.6%). Most cases required systemic therapy (n = 11, 84.6%) with DMARDs, biologics, or both. Of the 10 patients who required systemic therapy and reported compliance with the gluten-free diet, 3 (30%) were able to stop systemic medications. Two of 3 patients who cleared celiac serologies came off systemic medications. Statistically significant improvement was noted in the number of joints involved (p = 0.02) and physician global assessment of disease activity (p = 0.03) between the index and final visit. CONCLUSIONS: Rheumatologists play an important role in the identification of celiac disease, as arthritis was the presenting symptom in most cases and was not always associated with GI symptoms or poor growth. The arthritis was most often oligoarticular and asymmetric. Most children required systemic therapy. The gluten-free diet may not be sufficient to manage arthritis, but antibody clearance may be an indicator of higher likelihood of disease control off medications. Outcomes are promising with a combination of diet and medical therapy.

When to stop medication in juvenile idiopathic arthritis.

PURPOSE OF REVIEW: Disease-modifying antirheumatic drugs (DMARDs) have dramatically improved patient outcomes in juvenile idiopathic arthritis (JIA). However, these medications may also result in physical, psychologic, and economic burden, which must be balanced with risk of flare off treatment. Although some children remain in remission after medication discontinuation, evidence is sparse for if, when, and how medications should be de-escalated once achieving clinically inactive disease (CID). We review the data on medication discontinuation and the role of serologic and imaging biomarkers in JIA. RECENT FINDINGS: The literature uniformly supports early biologic DMARD initiation, although the optimal timing and strategy for medication withdrawal in patients with sustained CID remains unclear. In this review, we present the current data on flare frequency and time to flare, clinical factors associated with flare, and recapture data for each JIA category. We also summarize the current knowledge on the role of imaging and serologic biomarkers in guiding these treatment decisions. SUMMARY: JIA is a heterogenous disease for which prospective clinical trials are needed to address the question of when, how, and in whom to withdraw medication. Research investigating the roles of serologic and imaging biomarkers may help improve the ability to ascertain which children can successfully de-escalate medications.

Opioid use in children with inflammatory bowel disease-related arthritis.

OBJECTIVES: Concomitant arthritis may increase risk of chronic opioid use in youngsters with IBD. We aimed to assess trends and clinical features associated with opioid use in children with IBD-related arthritis. METHODS: Adolescents under 18 years of age with IBD-related arthritis, at least 1 year of continuous enrolment, and at least 1 pharmacy claim in the Truven Health MarketScan Claims and Encounter Database were included. Subjects were identified using previously validated algorithms consisting of ICD codes, pharmacy claims and procedure codes. The primary outcome was chronic opioid exposure. Temporal trends in opioid exposure were tested using the Cuzick-Wilcoxon test. The association of chronic opioid use and baseline covariates in the IBD and IBD-arthritis cohorts were examined using multivariable logistic regression models. RESULTS: 14,943 adolescents with IBD, 480 of whom had arthritis, were included. Chronic opioid use was non-trivial in youngsters with IBD-related arthritis, higher than that of total IBD cohort (12.3% vs. 5%) and remained stable over the years of study. Using multivariable regression, joint pain and arthritis were significantly associated with chronic opioid exposure in young people with IBD. Among IBD-related arthritis patients older age, public insurance, gastrointestinal surgery, hospitalisation and psychiatric comorbidities were significantly associated with chronic opioid use. CONCLUSIONS: Chronic opioid use in adolescents with IBD-related arthritis was higher than that of total IBD cohort but stable over the years of study. Future study is needed to explore ways to optimise non-narcotic pain management strategies and ensuring appropriate use of opioids when necessary.

Imaging in pediatric spondyloarthritis.

PURPOSE OF REVIEW: Imaging is used in the diagnosis of peripheral and axial disease in juvenile spondyloarthritis (JSpA). Imaging of the joints and entheses in children and adolescents can be challenging for those unfamiliar with the appearance of the maturing skeleton. These differences are key for rheumatologists and radiologists to be aware of. RECENT FINDINGS: In youth, skeletal variation during maturation makes the identification of arthritis, enthesitis, and sacroiliitis difficult. A great effort has been put forward to define imaging characteristics seen in healthy children in order to more accurately identify disease. Additionally, there are novel imaging modalities on the horizon that are promising to further differentiate normal physiologic changes versus disease. SUMMARY: This review describes the current state of imaging, limitations, and future imaging modalities in youth, with key attention to differences in imaging interpretation of the peripheral joints, entheses, and sacroiliac joint in youth and adults.

Consensus-Driven Definition for Unequivocal Sacroiliitis on Radiographs in Juvenile Spondyloarthritis.

OBJECTIVE: Radiography is still used worldwide for the detection of sacroiliitis in juvenile spondyloarthritis (JSpA), despite its low sensitivity and reliability. We aimed to define unequivocal evidence of sacroiliitis on pelvic radiography in skeletally immature youth for use in classification criteria when magnetic resonance imaging (MRI) is unavailable. METHODS: Subjects were a retrospective cohort of juvenile patients with spondyloarthritis with a radiograph and MRI as part of a diagnostic evaluation for axial disease. Six musculoskeletal imaging experts underwent an iterative consensus process to define unequivocal sacroiliitis on radiography in skeletally immature youth. Radiographs were graded using the modified New York (mNY) criteria and the unequivocal sacroiliitis criteria. Interrater agreement was assessed with the Fleiss [Formula: see text] statistic. Specificity, area under the receiver operator characteristic curve (AUROC), and sensitivity of the 2 measures were tested using 2 MRI reference standards. RESULTS: A total of 112 subjects, with a median age of 14.9 (range 6.7-20.1) years, were included. The Fleiss [Formula: see text] was fair for the mNY criteria (0.54, 95% CI 0.42-0.67) and the unequivocal sacroiliitis criteria (0.58, 95% CI 0.46-0.69). The unequivocal sacroiliitis criteria achieved > 90% specificity using both MRI reference standards. Sensitivity (59.26 and 57.14 vs 44.83 and 43.33) and AUROC (0.76 and 0.76 vs 0.71 and 0.71) were higher, for both reference standards, for the unequivocal sacroiliitis in youth definition than for the mNY criteria, respectively. CONCLUSION: In this study, we propose the first consensus-derived definition to our knowledge of unequivocal sacroiliitis by radiography in skeletally immature youth. This definition achieved excellent specificity and had higher AUROC and sensitivity values than the mNY criteria using both MRI reference standards. This definition has applicability to the JSpA axial disease classification imaging criterion when MRI is unavailable.

Improving Outcomes of Pediatric Lupus Care Delivery With Provider Goal-Setting Activities and Multidisciplinary Care Models.

OBJECTIVE: The present study was undertaken to evaluate high-quality care delivery in the context of provider goal-setting activities and a multidisciplinary care model using an electronic health record (EHR)-enabled pediatric lupus registry. We then determined associations between care quality and prednisone use among youth with systemic lupus erythematosus (SLE). METHODS: We implemented standardized EHR documentation tools to autopopulate a SLE registry. We compared pediatric Lupus Care Index (pLCI) performance (range 0.0-1.0; 1.0 representing perfect metric adherence) and timely follow-up 1) before versus during provider goal-setting activities and population management, and 2) in a multidisciplinary lupus nephritis versus rheumatology clinic. We estimated associations between pLCI and subsequent prednisone use adjusted for time, current medication, disease activity, clinical features, and social determinants of health. RESULTS: We analyzed 830 visits by 110 patients (median 7 visits per patient [interquartile range 4-10]) over 3.5 years. The provider-directed activity was associated with improved pLCI performance (adjusted ß 0.05 [95% confidence interval (95% CI) 0.01, 0.09]; mean 0.74 versus 0.69). Patients with nephritis in multidisciplinary clinic had higher pLCI scores (adjusted ß 0.06 [95% CI 0.02, 0.10]) and likelihood of timely follow-up than those in rheumatology (adjusted relative risk [RR] 1.27 [95% CI 1.02, 1.57]). A pLCI score of ≥0.50 was associated with 0.72-fold lower adjusted risk of subsequent prednisone use (95% CI 0.53, 0.93). Minoritized race, public insurance, and living in areas with greater social vulnerability were not associated with reduced care quality or follow-up, but public insurance was associated with higher risk of prednisone use. CONCLUSION: Greater attention to quality metrics is associated with better outcomes in childhood SLE. Multidisciplinary care models with population management may additionally facilitate equitable care delivery.

Determination of Relative Weightings for Sacroiliac Joint Pathologies in the OMERACT Juvenile Arthritis Magnetic Resonance Imaging Sacroiliac Joint Score.

This study aims to determine the relative weights (point value) of items of the juvenile idiopathic arthritis magnetic resonance imaging-sacroiliac joint scoring system (JAMRIS-SIJ). An adaptive multicriteria decision analysis was performed using the 1000Minds web application to determine the relative weights of the items in the JAMRIS-SIJ inflammation and damage domains. Experts in imaging and rheumatology independently completed a conjoint analysis survey (CAS) to determine the point value of the measurement items of the JAMRIS-SIJ. Each CAS survey question asked the expert to compare two hypothetical patient profiles, which were otherwise similar but different at two items at a time, and to select which item showed a more severe stage of inflammation or osteochondral damage. In addition, experts ranked 14 JAMRIS-SIJ grade only or image + grade patient vignettes while blinded to the CAS-derived weights. The validity of the weighted JAMRIS-SIJ was tested by comparing the expert CAS-weighted score and the image + grade ranking method. Seventeen experts completed the CAS (11 radiologists and 6 rheumatologists). Considering the point value for inflammation domain items, osteitis (24.7%) and bone marrow edema (24.3%) had higher group-averaged percentage weights compared to inflammation in erosion cavity (16.9%), joint space enhancement (13.1%), joint space fluid (9.1%), capsulitis (7.3%), and enthesitis (4.6%). Similarly, concerning the damage domain, ankylosis (41.3%) and erosion (25.1%) showed higher group-averaged weights compared to backfill (13.9%), sclerosis (10.7%), and fat metaplasia lesion (9.1%). The Spearman correlation coefficients of the CAS-weighted vignette order and unweighted JAMRIS-SIJ grade only order vignettes for all experts were 0.79 for inflammation and 0.80 for damage. The correlations of image vignettes among imaging experts to CAS were 0.75 for inflammation and 0.90 for damage. The multicriteria decision analysis identified differences in relative weights among the JAMRIS-SIJ measurement items. The determination of the relative weights provided expert-driven score scaling and face validity for the JAMRIS-SIJ, enabling the future evaluation of its longitudinal construct validity.

Biologic Abatement and Capturing Kids' Outcomes and Flare Frequency in Juvenile Spondyloarthritis (BACK-OFF JSpA): study protocol for a randomized pragmatic trial.

BACKGROUND: The effectiveness of biologic therapies, primarily tumor necrosis factor inhibitors (TNFi), for children with spondyloarthritis (SpA) has made inactive disease a realistic patient outcome. However, biologic therapies are costly, primarily delivered by subcutaneous or intravenous route, and have non-trivial side effects. Many patients and families want to know if biologic medications can be discontinued after inactive disease is achieved. It remains unclear whether medication dose should remain unchanged, tapered (increase the time between doses), or discontinued once when inactive disease is attained. METHODS: The Biologic Abatement and Capturing Kids' Outcomes and Flare Frequency in Juvenile SpA (BACK-OFF JSpA) trial is a multicenter pragmatic trial that will randomize 198 participants ages 8-21 years old with SpA and sustained inactive disease on standard TNFi dosing to (1) continue standard TNFi dosing, (2) fixed longer dosing intervals of TNFi, or (3) stop TNFi. The trial will compare the hazard rate of protocol-defined flare and participants' emotional health among the 3 groups over 12 months. Innovative aspects of this trial are the involvement of patient and parent stakeholders in the design and conduct of the study as well as an electronic health record-based enhanced recruitment strategy. DISCUSSION: This is the first randomized pragmatic trial to assess the efficacy of TNFi de-escalation strategies in children with JSpA with sustained inactive disease. This research will improve the evidence base that patients, caregivers, and rheumatologists use to make shared decisions about continued treatment versus de-escalation of TNFi therapy in this population. TRIAL REGISTRATION: ClinicalTrials.gov NCT04891640. Registered on 18 May 2021.

Development and Validation of a Juvenile Spondyloarthritis Disease Flare Measure: Ascertaining Flare in Patients With Inactive Disease.

OBJECTIVE: Our objective was to develop and validate a composite disease flare definition for juvenile spondyloarthritis (SpA) that would closely approximate the clinical decision made to reinitiate or not reinitiate systemic therapy after therapy de-escalation. METHODS: Retrospective chart reviews of children with SpA who underwent systemic therapy de-escalation of biologic or conventional disease-modifying antirheumatic drugs were used to develop and validate the flare outcome. Data on independent cohorts for development (1 center) and validation (4 centers) were collected from large tertiary health care systems. Core measure thresholds and candidate disease flare outcomes were assessed using sensitivity, specificity, positive predictive values (PPVs) and negative predictive values (NPVs), and the receiver operating characteristic (ROC) area under the curve (AUC), with physician assessment of active disease plus re-initiation of standard dose of systemic therapy as the reference standard. RESULTS: Of the candidate definitions, clinically meaningful worsening in ≥3 of the following 5 core measures performed best: caregiver/patient assessment of well-being; physician assessment of disease activity; caregiver/patient assessment of pain, physical function, and active joint count. The ROC AUC was 0.91, PPV 87.5%, NPV 98.1%, sensitivity 82.4%, and specificity 98.7%. Cronbach's α was 0.81, signifying internal consistency, and factor analysis demonstrated that the outcome measured 1 construct. The Juvenile SpA Flare measure had face validity according to 21 surveyed pediatric rheumatologists. Juvenile SpA Flare had an ROC AUC of 0.85, a PPV of 92.3%, and an NPV of 96.8% in the validation cohort. CONCLUSION: There is initial support for the validity of the Juvenile SpA Flare measure as a tool to identify disease flare in juvenile SpA patients de-escalating therapy, and the measure is potentially applicable in clinical practice, observational studies, and therapeutic trials.

Stakeholder outcome prioritization in the Biologic Abatement and Capturing Kids' Outcomes and Flare Frequency in Juvenile Spondyloarthritis (BACK-OFF JSpA) trial.

BACKGROUND: The Biologic Abatement and Capturing Kids' Outcomes and Flare Frequency in Juvenile Spondyloarthritis (BACK-OFF JSpA) study is a randomized, pragmatic trial investigating different tumour necrosis factor inhibitor de-escalation strategies for children with sustained inactive disease. In this project, we elicited concept rankings that aided in the selection of the patient-reported outcome (PRO) measures that should be examined as part of the BACK-OFF JSpA trial. METHODS: We conducted a discrete choice experiment to evaluate individuals' preferences regarding PROs. Stakeholders assessed a discrete list of 21 outcome concepts, each of which had a Patient-Reported Outcome Measurement Information System (PROMIS) measure associated with it. PROMIS measures are self- or proxy-reported instruments that are universally applicable to the general population and all chronic conditions. Stakeholders were required to make choices instead of expressing the strength of a preference. RESULTS: Fourteen caregivers, 12 patients (9-22 years old), 16 rheumatologists and three executives from health insurance companies completed the exercise, which took approximately 10 min. The discrete choice experiment resulted in an estimate of the relative importance of each outcome and rank. All stakeholder groups agreed that the primary PRO should be 'Pain Interference', a measure that evaluates the effect of pain on a child's everyday activities, including its impact on social, emotional, mental and physical functioning. Patients and caregivers were mostly aligned in their top priorities, with patients valuing physical health (50% of the top 10) whereas caregivers were more interested in mental health (60% of the top 10). Rheumatologists and health insurance executives were most interested in physical health outcomes, which were ranked 80% and 60% of their top 10 PROs, respectively. Overall, the patients had the most diverse set of prioritized outcomes, including at least one of each category in their top 10 rank order of importance. Patients were also the only stakeholders to prioritize 'social' health. CONCLUSIONS: Patients and caregivers were mostly aligned in their outcome priority rankings. The rank-order list directly informed the creation of a profile of PRO measures for our upcoming trial. PATIENT OR PUBLIC CONTRIBUTION: Stakeholder partners helped with acquisition of data and lead parent partners helped interpret data.

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

OBJECTIVE: To validate the Juvenile Spondyloarthritis Disease Activity Index (JSpADA), and modified versions thereof, in a North American cohort of patients with enthesitis-related arthritis (ERA). METHODS: We utilized the Childhood Arthritis and Rheumatology Research Alliance Registry database ERA cohort to validate the JSpADA and its modifications (JSpADA6-no Schober, no C-reactive protein [CRP]/erythrocyte sedimentation rate [ESR]; JSpADA7-no Schober; and JSpADA7-no CRP/ESR) using the Outcome Measures in Rheumatology principles of face validity, discriminative validity, and responsiveness to change. RESULTS: There 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%), and 56.9% were male and 50% were HLA-B27 positive. The JSpADA showed high correlation with the clinical 10-joint Juvenile Arthritis Disease Activity Score (cJADAS10; r = 0.81), moderate-to-high correlation with physician global assessment (PGA; r = 0.69), and low-to-fair correlation with Childhood Health Assessment Questionnaire (CHAQ; r = 0.22). The modifications of the JSpADA (JSpADA7-no Schober; JSpADA7-no CRP/ESR; and JSpADA6-no Schober, no CRP/ESR) performed similarly with high correlation with cJADAS10 (r = 0.81, 0.79, and 0.80, respectively), moderate-to-high correlation with PGA (r = 0.65, 0.67, 0.64, respectively), and low-to-fair correlation with CHAQ (r = 0.35, 0.34, 0.39, respectively). All modified versions of JSpADA had good responsiveness to change. All versions of JSpADA had excellent discriminative validity. CONCLUSION: We 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.

Data-Driven Magnetic Resonance Imaging Definitions for Active and Structural Sacroiliac Joint Lesions in Juvenile Spondyloarthritis Typical of Axial Disease: A Cross-Sectional International Study.

OBJECTIVE: We aimed to determine quantitative sacroiliac (SI) joint magnetic resonance imaging (MRI) cutoffs for active and structural lesions that will be incorporated as imaging domains in classification criteria of axial disease in juvenile spondyloarthritis (SpA). METHODS: MRI scans from an international cross-section of juvenile SpA patients were reviewed by 6 musculoskeletal imaging experts blinded to clinical details. Raters globally assessed the presence/absence of lesions typical of axial SpA and performed SI joint quadrant- or joint-based scoring. Sensitivity and specificity of lesion cutoffs were calculated using a rater majority (≥4 of 6 raters) on a global assessment of the presence/absence of active or structural lesions typical of axial SpA with high confidence as the reference standard. Cutoffs were validated in an independent cohort. RESULTS: Imaging from 243 subjects, 61% male, median age 14.9 years, had sequences available for detailed MRI scoring. Optimal cutoffs for defining lesions typical of axial disease in juvenile SpA were: 1) inflammatory lesion: bone marrow edema in ≥3 SI joint quadrants across all SI joint MRI slices (sensitivity 98.6%, specificity 96.5%); 2) structural lesions: erosion in ≥3 quadrants or sclerosis or fat lesion in ≥2 SI joint quadrants or backfill or ankylosis in ≥2 joint halves across all SI joint MRI slices (sensitivity 98.6%, specificity 95.5%). Sensitivity and specificity of the optimal cutoffs in the validation cohort were excellent. CONCLUSION: We propose data-driven cutoffs for active inflammatory and structural lesions on MRI typical of axial disease in juvenile SpA that have high specificity and sensitivity using central imaging global assessment as the reference standard and excellent reliability.

The Feasibility and Acceptability of Resilience Coaching for Adolescent Chronic Musculoskeletal Pain: A Single-Arm Pilot Trial.

Promoting Resilience in Stress Management (PRISM) is a well-established resilience coaching program for youth with chronic illness. It is a one-on-one intervention targeting skills in stress management, goal-setting, cognitive reframing, and meaning-making. We aimed to (i) assess the feasibility and acceptability of PRISM and (ii) explore PRISM's impact on clinical outcomes among youth with chronic musculoskeletal pain (CMP). This was a single-arm pilot trial of PRISM for youth with CMP aged 12-17 years. Patients completed patient-reported outcome measures (PROs) pre- and post- intervention; patients and caregivers provided qualitative feedback. Twenty-seven patients were enrolled (63% enrollment rate); 82% percent were female. The patients' median age was 16 years (IQR: 13-16). The intervention completion rate was 81% (n = 22). The mean satisfaction for PRISM overall was 4.3 (SD 0.9), while the mean acceptability of the intervention measure (AIM) was 4.4 (SD 0.89). Participants reported improved resilience (2.2 [SD 5.1]), functional disability (-3.5 [IQR: -6.0, 1.0]), and psychological distress (-1.0 [-5.0, 2.0]) from baseline to immediately post-treatment; pain intensity, pain catastrophizing, and global health were similar at both time points. Feedback was positive and suggested that a group component may be helpful. PRISM is feasible and acceptable among youth with CMP. Exploratory analyses suggest improvements in clinically relevant outcomes, warranting further investigation.