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Inter-reader reliability of a new scoring system for evaluating joint inflammation and enthesitis in whole body MRI (WBMRI) in juvenile idiopathic arthritis was tested. The scoring system grades 732 item-region combinations of bone marrow and soft tissue changes for commonly involved joints and entheseal sites. Five radiologists rated 17 WBMRI scans through an online rating platform. Item-wise reliability was calculated for 117 items with non-zero scores in >10 % of readings. Interquartile ranges of the five-reader Kappa reliability coefficients were 0.58-0.73 (range: 0.36-0.88) for the joints, 0.65-0.81 (range: 0.39-0.95) for the entheses, and 0.62-0.75 (range: 0.60-0.76) for chronic nonbacterial osteomyelitis-like lesions., Competing Interests: Declaration of competing interest Dr. Andrea Doria has had the following relationships unrelated to the conduct of this study: Chair and Co-Chair of the International Myositis Assessment & Clinical Studies Group (not for profit) and the OMERACT SIG in MRI in JIA (not for profit), respectively, board member of the OMERACT Technical Advisory Group (not for profit), and PI of research grants from Novo Nordisk, the Terry Fox Foundation, the PSI Foundation, the Society of Pediatric Radiology, and the Garron Family Cancer Centre. Drs. Jonathan Akikusa and Marion van Rossum are also Co-Chairs of OMERACT SIG in MRI in JIA (not for profit). The remaining authors did not express any conflicts of interest related to current study., (Copyright © 2024. Published by Elsevier Inc.)

The treatment of a patient with juvenile idiopathic arthritis (JIA) is best monitored with standardized and validated tools to measure joint changes over time. Treatment approaches are best indicated if the clinicians are aware of the structural status of the joint at a given time, especially in anatomically deep joints for which clinical assessment is limited. Magnetic resonance imaging (MRI) is of utmost importance for assessment of deep joints and extra-articular soft tissue of the entire body for which ultrasound may be suboptimal. Because the distinction between pathologic and physiologic joint changes on MRI is key for proper diagnosis and treatment of patients with arthropathies, a comprehensive standardized approach is needed to effectively measure outcomes of growing joints of children with JIA. Such an approach is essential for both clinical assessment and to conduct clinical trials in patients with JIA treated in different centers around the world. To meet this need, several international imaging collaborative research groups have been developing MRI scales over the past years, including the MRI in JIA (JAMRI) special interest group within the Outcome Measures in Rheumatology (OMERACT) research network. This manuscript reviews the efforts of the OMERACT JAMRI working group to generate and validate pediatric MRI scoring systems for different joints in children with JIA that can have ubiquitous utilization anywhere in the world. In particular, it describes the different steps of development and validation of an MRI scale using the TMJ as a model., (© 2023. The Author(s) under exclusive licence to Japan Radiological Society.)

Objective: To determine the relative importance weights of items and grades of a newly developed additive outcome measure called the juvenile idiopathic arthritis (JIA) magnetic resonance imaging (MRI) scoring system for the temporomandibular joint (TMJ) (JAMRIS-TMJ)., Methods: An adaptive partial-profile, discrete choice experiment (DCE) survey using the 1000Minds platform was independently completed by members of an expert group consisting of radiologists and non-radiologist clinicians to determine the group-averaged relative weights for the JAMRIS-TMJ. Subsequently, an image-based vignette ranking exercise was done, during which experts individually rank ordered 14 patient vignettes for disease severity while blinded to the weights and unrestricted to JAMRIS-TMJ assessment criteria. Validity of the weighted JAMRIS-TMJ was tested by comparing the consensus-graded, DCE-weighted JAMRIS-TMJ score of the vignettes with their unrestricted image-based ranks provided by the experts., Results: Nineteen experts completed the DCE survey, and 21 completed the vignette ranking exercise. Synovial thickening and joint enhancement showed higher weights per raw score compared to bone marrow items and effusion in the inflammatory domain, while erosions and condylar flattening showed nonlinear and higher weights compared to disk abnormalities in the damage domain. The weighted JAMRIS-TMJ score of the vignettes correlated highly with the ranks from the unrestricted comparison method, with median Spearman's ρ of 0.92 (interquartile range [IQR] 0.87-0.95) for the inflammation and 0.93 (IQR 0.90-0.94) for the damage domain., Conclusion: A DCE survey was used to quantify the importance weights of the items and grades of the JAMRIS-TMJ. The weighted score showed high convergent validity with an unrestricted, holistic vignette ranking method., (© 2021, American College of Rheumatology.)

Objectives: Whole body-MRI is helpful in directing diagnostic and treatment approaches, and as a research outcome measure. We describe our initial consensus-driven phase towards developing a whole body-MRI scoring system for juvenile idiopathic arthritis., Methods: An iterative approach using three rounds of anonymous Delphi surveys followed by a consensus meeting was used to draft the structure of the whole body-MRI scoring system, including the relevant anatomic joints and entheses for assessment, diagnostic item selection, definition and grading, and selection of appropriate MRI planes and sequences. The surveys were completed independently by an international expert group consisting of pediatric radiologists and rheumatologists., Results: Twenty-two experts participated in at least one of three rounds of Delphi surveys and a concluding consensus meeting. A first iteration scoring system was developed which ultimately included the assessment of 100 peripheral, 23 chest, and 76 axial joints, and 64 entheses, with 2-4 diagnostic items graded in each of the items, using binary (presence/absence) and 2-3-level ordinal scores. Recommendations on anatomic MRI planes and sequences were specified as the minimally necessary imaging protocol for the scoring system., Conclusion: A novel whole body-MRI scoring system for juvenile idiopathic arthritis was developed by consensus among members of MRI in JIA OMERACT working group. Further iterative refinements, reliability testing, and responsiveness are warranted in upcoming studies., (Copyright © 2021 Elsevier Inc. All rights reserved.)

With powerful new therapies available for management of juvenile idiopathic arthritis (JIA), early diagnosis leading to appropriate treatment may prevent long-term structural joint damage. Although magnetic resonance imaging (MRI) is typically used to assess individual body parts, indications for whole body (WB) MRI are increasing. Its utility as a diagnostic and monitoring tool has already been widely investigated in adult rheumatology patients, but less so in pediatric rheumatologic patients. This paper is a comprehensive review of scoring systems and a proposal for the conceptual development of a WB-MRI scoring system for the evaluation of JIA. In this review we identify, summarize, and critically appraise the available literature on the use of WB-MRI in inflammatory arthritis, addressing relevant considerations on components of a classification system that can lead to the development of a future pediatric WB-MRI scoring system for use in children with JIA. We also discuss advantages and challenges of developing such a WB-MRI scoring system for assessment of JIA and outline next steps toward the conceptual development of this scoring system., (Copyright © 2020. Published by Elsevier Inc.)

Juvenile idiopathic arthritis (JIA) is the most common chronic arthropathy in the pediatric population. Although the diagnosis is essentially clinical for many affected joints, MR imaging has become an important tool for the assessment of joints that are difficult to evaluate clinically, such as temporomandibular and sacroiliac joints, and for screening of inflammatory changes in the entire body by whole body MRI (WBMRI) assessment. The utilization of MR imaging is challenging in the pediatric population given the need for discrimination between pathological and physiological changes in the growing skeleton. Several multicentric multidisciplinary organizations have made major efforts over the past decades to standardize, quantify, and validate scoring systems to measure joint changes both cross-sectionally and longitudinally according to rigorous methodological standards. In this paper, we (1) discuss current trends for the diagnosis and management of JIA, (2) review challenges for detecting real pathological changes in growing joints, (3) summarize the current status of standardization of MRI protocols for data acquisition and the quantification of joint pathology in JIA by means of scoring systems, and (4) outline novel MR imaging techniques for the evaluation of anatomy and function of joints in JIA. Optimizing the role of MRI as a robust biomarker and outcome measure remains a priority of future research in this field., (Copyright © 2020. Published by Elsevier Ltd.)

Objective: To develop definitions for the assessment of magnetic resonance imaging (MRI) pathologies of the sacroiliac joints (SIJ) in juvenile idiopathic arthritis., Methods: An Outcome Measures in Rheumatology (OMERACT) consensus-driven methodology consisting of iterative surveys and focus group meetings within an international group of rheumatologists and radiologists., Results: Two domains, inflammation and structural, were identified. Definitions for bone marrow edema, joint space inflammation, capsulitis, and enthesitis were derived for joint inflammation; sclerosis, erosion, fatty lesion, and ankylosis were defined for assessing structural joint changes., Conclusion: Preliminary consensus-driven definitions for inflammation and structural elements have been derived, underpinning the ongoing development of the OMERACT Juvenile Arthritis MRI SIJ scoring system (OMERACT JAMRIS-SIJ).

Objective: Intra- and interreader reliability, construct validity, and responsiveness of the Spondyloarthritis Research Consortium of Canada (SPARCC) magnetic resonance imaging (MRI) scoring system were investigated for scoring sacroiliitis in patients with juvenile spondyloarthritis (JSpA)/enthesitis-related arthritis (ERA) who have received biologic and/or nonbiologic treatment., Methods: Ninety whole-body MRI examinations with dedicated oblique coronal planes of the sacroiliac joints in 46 patients were independently reviewed and scored by 2 pediatric musculoskeletal radiologists, blinded to clinical details, using the SPARCC system. Intra- and interreader reliability was assessed by intraclass correlation coefficients (ICC). Construct validity testing was done by (1) correlating the SPARCC MRI scores of sacroiliitis with clinical disease activity indicators (cross-sectional validity), and (2) correlating the change in the MRI score with the change in clinical indicators before and after treatment (longitudinal validity). Responsiveness of the MRI and clinical indicators was also evaluated, grouped by biologic and nonbiologic treatment., Results: When applied in children with JSpA/ERA, the SPARCC showed almost perfect intra- and interreader reliability (ICC 0.79-1.00). There was poor cross-sectional and longitudinal correlation between clinical assessment indicators and MRI scoring. SPARCC scores showed higher responsiveness to treatment-related change than most clinical outcome measures. Three clinical outcome measures correlated longitudinally with SPARCC score in nonbiologic treatment: active joint count (r = 0.72, p < 0.001), FABER (Flexion, Abduction, External Rotation) test (r = 0.58, p = 0.012), and physician's global assessment (r = 0.61, p = 0.034)., Conclusion: The SPARCC MRI scoring system is a reliable tool with relatively higher responsiveness than clinical indicators and is suitable for objective quantification of sacroiliitis when applied to pediatric patients with JSpA/ERA.