Search

ObjectiveMethodResultsConclusionTo assess the agreement between child- and parent-reported orofacial symptoms in the Danish version of the patient questionnaire Assessment of Orofacial Symptoms in Juvenile Idiopathic Arthritis.This cross-sectional study was conducted at Aarhus University in March 2023. Eligible candidates were consecutive subjects with juvenile idiopathic arthritis (JIA) and temporomandibular joint involvement accompanied by a parental proxy for examination in the Craniofacial Clinic. After obtaining written informed consent, the questionnaire was completed individually and separately by the child and the parent without any communication between them. The level of agreement was analysed using Cohen’s (weighted) kappa for nominal and ordinal outcome variables (orofacial pain frequency, pain location, jaw function, orofacial symptoms, and changes since last visit) and the intraclass correlation coefficient for linear outcome variables (orofacial pain intensity and functional disability of the jaw).The 34 included dyads had an overall ‘poor’ to ‘moderate’ child–proxy reporting agreement on the questionnaire for the assessment of JIA-related orofacial symptoms. After dividing the children into two age groups, < 13 and ≥ 13 years old, we found substantial agreement on pain frequency and moderate to excellent agreement on pain intensity for the older group. The child–proxy agreement for children aged < 13 years was slight on pain frequency and poor to moderate on pain intensity.The child–proxy reporting agreement on JIA-related orofacial symptoms is inconsistent. We suggest collecting information from both children and parents, especially when assessing orofacial pain and symptoms in children < 13 years of age. [ABSTRACT FROM AUTHOR]

Objectives: To develop consensus-based recommendations for physician's global assessment of disease activity (PhGA) scoring and to standardise definitions of disease activity., Methods: An international task force of 34 members was assembled, and recommendations were developed in 3 phases: (1) 2 preliminary surveys of paediatric rheumatologists and a literature review; (2) 14 videoconference meetings, informed by multicriteria decision analysis and formal anonymous voting; and (3) a 2-day in-person consensus conference using structured nominal group technique discussions and formal voting. The threshold for achieving consensus was ≥78% of voting task force members. Agreement with the final statements was rated using a numerical rating scale from 0, strongly disagree, to 10, strongly agree., Results: Eighteen points to consider were agreed upon. All statements achieved consensus (≥78%), with a level of agreement ≥9.2. Points included the definition of disease activity in juvenile idiopathic arthritis (JIA), factors to assess in nonsystemic JIA and systemic JIA, consideration of available imaging and laboratory tests, the role of extra-articular manifestations, the evaluation of treatment, and the timing of PhGA scoring., Conclusions: The task force developed consensus-based recommendations when scoring the PhGA in nonsystemic and systemic JIA. These recommendations will lead to more reliable scoring of disease activity in patients with JIA., Competing Interests: Competing interests None of the authors received any support from commercial entities for the work reported in the current submitted manuscript. VR and AC reports that the project was supported by the 2022 Paediatric Rheumatology European Association (PReS)/Paediatric Rheumatology International Trials Organisation (PRINTO) grant, The Systemic JIA Foundation, the University Hospital of North Norway, the University of Tromsø, The Arctic University of Norway, and Grete Harbitz legacy against rheumatic diseases, Norway. All the authors of the paper attending the PhGA consensus conference in Tromsø, Norway, received reimbursement of travel expenses covered by the PRes/PRINTO grant, and the Systemic JIA Foundation. The accomodation in Tromsø and meeting facilities expenses were covered by funding from Grete Harbitz legacy, the University Hospital of North Norway and the University of Tromsø - The Arctic University of Norway. CB has received consulting fees from Swedish Orphan Biovitrum AB (SOBI) and Novartis. AC has an investigator initiated grant from Pfizer. AC has received payment or honoraria for lectures, presentations, speakers bureaus, manuscript writing or educational events from Pfizer. BF has received grants from Canadian Institutes of Health Research (CIHR) and the institution. BF has received royalties or licenses from Hemophilia Joint Health Score (HJHS) and the institution. BF has received consulting fees from Pfizer, Cabaletta, and Data Safety and Monitoring Board (DSMB). BF reports participation on a data safety monitoring board or advisory board for Pfizer. JG reports that the project was funded by a competitive grant as stated in the manuscript. JG is the co-lead of the American College of Rheumatology Disease Criteria Subcommittee. DBH reports grant funding from Childhood Arthritis and Rheumatology Research Alliance-Arthritis Foundation and Danisco USA, Inc. DBH reports receiving payment or honoraria for lectures, presentations, speakers bureaus, manuscript writing or educational events from American College of Rheumatology and honorarium for participating in JIA treatment guidelines. DBH reports support for attending meetings and/or travel from Childhood Arthritis and Rheumatology Research Alliance and American College of Rheumatology. DBH reports leadership or fiduciary role in other board, society, committee or advocacy group, paid or unpaid from Childhood Arthritis and Rheumatology Research Alliance and membership of the steering committee. RML reports royalties and license from UpToDate and Elsevier. RML reports consulting fees from SOBI, Novartis, Sanofi, Akros Pharma, and Eli Lilly Canada. RML reports Participation on a Data Safety Monitoring Board or Advisory Board for Novartis, Eli Lilly, Sanofi, and Children's Hospital of Philadelphia. FM reports consulting fees from SOBI. FM reports payment or honoraria for lectures, presentations, speakers bureaus, manuscript writing or educational events from SOBI and Novartis. FM reports support for attending meetings and/or travel from SOBI. FM reports participation on a data safety monitoring board or advisory board for SOBI. EMM reports grants or contracts from Pfizer and Agency for Healthcare Research and Quality. EMM reports payment or honoraria for lectures, presentations, speakers bureaus, manuscript writing or educational events from American College of Rheumatology. EMM reports support for attending meetings and/or travel from American College of Rheumatology and Systemic JIA Foundation. EMM reports leadership or fiduciary role in other board, society, committee or advocacy group, paid or unpaid, for the Pediatric Rheumatology Care and Outcomes Improvement Network. JM reports payment or honoraria for lectures, presentations, speakers bureaus, manuscript writing or educational events for Pfizer excite meeting Sept 2023 Melbourne speaker. CN reports PReS as support for the present manuscript. CN reports support for attending meetings and/or travel from PReS. AR reports payment or honoraria for lectures, presentations, speakers bureaus, manuscript writing or educational events from AbbVie, Alexion, Novartis, Pfizer, SOBI, and Galapagos. AR is the President of the Pediatric Rheumatology European Society. JFS reports receiving a small Research grant from Pfizer. JFS reports Payment or honoraria for lectures, presentations, speakers bureaus, manuscript writing or educational events from Pfizer. MTw reports receiving travel support to attend the Consensus meeting. MTw is a committee chair of Childhood Arthritis and Rheumatology Research Alliance (CARRA) JIA. PV reports Support for attending meetings and/or travel from Pfizer, Novartis, and Orion. PV reports Participation on a Data Safety Monitoring Board or Advisory Board for Novartis. SJV reports grants from Dutch Arthritis Society Grant: InSyST (systemic JIA), Dutch Arthritis Society Grant: Longitudinal Research Support line 10 (LLP10), and PReS PRINTO Grant: Metaphor project, co-PI. SJV reports consulting fees from SOBI and Novartis. SJV reports Payment or honoraria for lectures, presentations, speakers bureaus, manuscript writing or educational events from SOBI and Novartis. SJV reports Patents planned, issued or pending (PCT/EP2023/066764). SJV reports being Chair Kawasaki Disease Coronary Artery Aneurysm Prevention (KD CAAP) Trial Steering Committee, Co-principal investigator for Canada-Netherlands Personalized Medicine Network in Childhood Arthritis and Rheumatic Diseases (UCAN CAN-DU), and Chair Clinical Guideline Committee The European Reference Network-Rare Immunological Disorders (ERN-RITA). JV reports Payment or honoraria for lectures, presentations, speakers bureaus, manuscript writing or educational events from Novartis and Eli Lily. JV reports Support for attending meetings and/or travel from Abbvie and Novartis. JV is a member of the PRES executive committee. PW reports grants from Patient-Centered Outcomes Research Institute, National Institutes of Health (NIH), National Institute of Arthritis and Muskuloskeletal and Skin Diseases (NIAMS) (1R01AR074098), and Spondylitis Association of America. PW reports Royalties or licenses from UpToDate. PW reports Consulting fees from Pfizer and Cerecor. PW is a Site investigator for Pfizer and Abbvie Clinical Trials. PW is an advisory board member of Lily, Biogen, and Novartis. PW reports payment or honoraria for lectures, presentations, speakers bureaus, manuscript writing or educational events from Rheum Now Speaker (2022) and Spondyloarthritis Research and Treatment Network. The following authors have no conflicts of interest to disclose: AA, SA-H, MB, EB-D, MG, DJL, TL, SM-M, EN, NP, EP, CSM, RS, MTa., (Copyright © 2025 European Alliance of Associations for Rheumatology (EULAR). Published by Elsevier B.V. All rights reserved.)

Objectives: To identify long-term disease activity trajectories from childhood to adulthood by using the clinical Juvenile Arthritis Disease Activity Score (cJADAS10) in juvenile idiopathic arthritis (JIA). Second, to evaluate the contribution of the cJADAS10 components and explore characteristics associated with active disease at the 18-year follow-up., Methods: Patients with onset of JIA in 1997-2000 were followed for 18 years in the population-based Nordic JIA cohort. We used a discrete mixture model for longitudinal clustering of the cJADAS10 and its components. We assessed factors potentially associated with higher scores on the patient's global assessment of well-being (PaGA) by hierarchical clustering and correlation analysis., Results: Four disease activity trajectories were identified based on the cJADAS10 components among 427 patients. In trajectory-group 2, the PaGA and the physician's global assessment of disease activity (PhGA) increased significantly during the course, but not the active joint count. The increase in the PaGA was significantly higher than the increases in the PhGA and the active joint count (p<0.0001). A similar pattern was found among all the patients with active disease in the total cohort. Patients with higher PaGA scores had unfavourable scores on several other patient-reported outcomes., Conclusions: We have identified groups of patients based on long-term disease activity trajectories. In our study the PaGA was the most important driver of disease activity into adulthood assessed by cJADAS10. We need to better understand how our patients interpret global well-being and implement strategies to achieve inactive disease perceived both by the patient and the physician., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2024. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.)

Involvement of the temporomandibular joint (TMJ) is common in juvenile idiopathic arthritis (JIA). TMJ arthritis can lead to orofacial symptoms, orofacial dysfunction, and dentofacial deformity with negative impact on quality of life. Management involves interdisciplinary collaboration. No current recommendations exist to guide clinical management. We undertook this study to develop consensus-based interdisciplinary recommendations for management of orofacial manifestations of JIA, and to create a future research agenda related to management of TMJ arthritis in children with JIA. Recommendations were developed using online surveying of relevant stakeholders, systematic literature review, evidence-informed generation of recommendations during 2 consensus meetings, and Delphi study iterations involving external experts. The process included disciplines involved in the care of orofacial manifestations of JIA: pediatric rheumatology, radiology, orthodontics, oral and maxillofacial surgery, orofacial pain specialists, and pediatric dentistry. Recommendations were accepted if agreement was >80% during a final Delphi study. Three overarching management principles and 12 recommendations for interdisciplinary management of orofacial manifestations of JIA were outlined. The 12 recommendations pertained to diagnosis (n = 4), treatment of TMJ arthritis (active TMJ inflammation) (n = 2), treatment of TMJ dysfunction and symptoms (n = 3), treatment of arthritis-related dentofacial deformity (n = 2), and other aspects related to JIA (n = 1). Additionally, a future interdisciplinary research agenda was developed. These are the first interdisciplinary recommendations to guide clinical management of TMJ JIA. The 3 overarching principles and 12 recommendations fill an important gap in current clinical practice. They emphasize the importance of an interdisciplinary approach to diagnosis and management of orofacial manifestations of JIA., (© 2022 The Authors. Arthritis & Rheumatology published by Wiley Periodicals LLC on behalf of American College of Rheumatology.)

Background: Etanercept (ETN) and adalimumab (ADA) are considered equally effective biologicals in the treatment of arthritis in juvenile idiopathic arthritis (JIA) but no studies have compared their impact on patient-reported well-being. The objective of this study was to determine whether ETN and ADA have a differential effect on patient-reported well-being in non-systemic JIA using real-world data., Methods: Biological-naive patients without a history of uveitis were selected from the international Pharmachild registry. Patients starting ETN were matched to patients starting ADA based on propensity score and outcomes were collected at time of therapy initiation and 3-12 months afterwards. Primary outcome at follow-up was the improvement in Juvenile Arthritis Multidimensional Assessment Report (JAMAR) visual analogue scale (VAS) well-being score from baseline. Secondary outcomes at follow-up were decrease in active joint count, adverse events and uveitis events. Outcomes were analyzed using linear and logistic mixed effects models., Results: Out of 158 eligible patients, 45 ETN starters and 45 ADA starters could be propensity score matched resulting in similar VAS well-being scores at baseline. At follow-up, the median improvement in VAS well-being was 2 (interquartile range (IQR): 0.0 - 4.0) and scores were significantly better (P = 0.01) for ETN starters (median 0.0, IQR: 0.0 - 1.0) compared to ADA starters (median 1.0, IQR: 0.0 - 3.5). The estimated mean difference in VAS well-being improvement from baseline for ETN versus ADA was 0.89 (95% CI: -0.01 - 1.78; P = 0.06). The estimated mean difference in active joint count decrease was -0.36 (95% CI: -1.02 - 0.30; P = 0.28) and odds ratio for adverse events was 0.48 (95% CI: 0.16 -1.44; P = 0.19). One uveitis event was observed in the ETN group., Conclusions: Both ETN and ADA improve well-being in non-systemic JIA. Our data might indicate a trend towards a slightly stronger effect for ETN, but larger studies are needed to confirm this given the lack of statistical significance., (© 2022. The Author(s).)

Objective: To describe the appearances of bone marrow in the appendicular skeleton on fat-suppressed T2-weighted sequences as assessed by whole-body MRI in healthy and asymptomatic children and adolescents., Material and Methods: Following ethical approval, we assessed the bone marrow of the extremities on water-only Dixon T2-weighted images as part of a whole-body MRI in 196 healthy and asymptomatic children aged 5-19 years. Based on a newly devised and validated scoring system, we graded intensity (0-2 scale) and extension (1-4 scale) of focal high signal bone marrow areas, and divided them into minor or major findings, based on intensity and extension, reflecting their potential conspicuousness in a clinical setting., Results: In the upper extremity, we registered 366 areas with increased signal whereof 79 were major findings. In the lower extremities there were 675 areas of increased signal of which 340 were major findings. Hundred-and-fifteen (58.79%) individuals had at least one major finding, mainly located in the hand and proximal humerus, and the feet and knees. We found no differences according to gender, reported hours of sports activity, handedness, or age group, except for more minor findings in the upper extremities amongst 15-18-year-olds as compared to those aged 5-8 years., Conclusion: Focal areas of high signal intensity on whole-body MRI, T2-weighted fat suppressed images that, in a clinical setting could cause concern, were seen in more than half of healthy, asymptomatic children and adolescents. Awareness of this is important when interpreting whole-body MRI in this age group, particularly in the assessment of clinically silent lesions., (Copyright © 2022 The Author(s). Published by Elsevier B.V. All rights reserved.)

Objective: To describe risk factors for IBD development in a cohort of children with JIA., Methods: JIA patients who developed IBD were identified from the international Pharmachild register. Characteristics were compared between IBD and non-IBD patients and predictors of IBD were determined using multivariable logistic regression analysis. Incidence rates of IBD events on different DMARDs were calculated, and differences between therapies were expressed as relative risks (RR)., Results: Out of 8942 patients, 48 (0.54% ) developed IBD. These were more often male (47.9% vs 32.0%) and HLA-B27 positive (38.2% vs 21.0%) and older at JIA onset (median 8.94 vs 5.33 years) than patients without IBD development. They also had more often a family history of autoimmune disease (42.6% vs 24.4%) and enthesitis-related arthritis (39.6% vs 10.8%). The strongest predictors of IBD on multivariable analysis were enthesitis-related arthritis [odds ratio (OR): 3.68, 95% CI: 1.41, 9.40] and a family history of autoimmune disease (OR: 2.27, 95% CI: 1.12, 4.54). Compared with methotrexate monotherapy, the incidence of IBD on etanercept monotherapy (RR: 7.69, 95% CI: 1.99, 29.74), etanercept with methotrexate (RR: 5.70, 95% CI: 1.42, 22.77) and infliximab (RR: 7.61, 95% CI: 1.27, 45.57) therapy was significantly higher. Incidence on adalimumab was not significantly different (RR: 1.45, 95% CI: 0.15, 13.89)., Conclusion: IBD in JIA was associated with enthesitis-related arthritis and a family history of autoimmune disease. An increased IBD incidence was observed for etanercept therapy regardless of concomitant methotrexate use., (© The Author(s) 2021. Published by Oxford University Press on behalf of the British Society for Rheumatology.)

Objective: To propose multidisciplinary, consensus-based, standardization of operational terminology and method of assessment for temporomandibular joint (TMJ) involvement in juvenile idiopathic arthritis (JIA)., Methods: Using a sequential expert group-defined terminology and methods-of-assessment approach by (1) establishment of task force, (2) item generation, (3) working group consensus, (4) external expert content validity testing, and (5) multidisciplinary group of experts final Delphi survey consensus., Results: Seven standardized operational terms were defined: TMJ arthritis, TMJ involvement, TMJ arthritis management, dentofacial deformity, TMJ deformity, TMJ symptoms, and TMJ dysfunction. CONCLUSION: Definition of 7 operational standardized terms provides an optimal platform for communication across healthcare providers involved in JIA-TMJ arthritis management.

The Juvenile Arthritis Multidimensional Assessment Report (JAMAR) is a new parent/patient-reported outcome measure that enables a thorough assessment of the disease status in children with juvenile idiopathic arthritis (JIA). We report the results of the cross-cultural adaptation and validation of the parent and patient versions of the JAMAR in the Norwegian language. The reading comprehension of the questionnaire was tested in 10 JIA parents and patients. Each participating centre was asked to collect demographic and clinical data and the JAMAR in 100 consecutive JIA patients or all consecutive patients seen in a 6-month period and to administer the JAMAR to 100 healthy children and their parents. The statistical validation phase explored descriptive statistics and the psychometric issues of the JAMAR: the 3 Likert assumptions, floor/ceiling effects, internal consistency, Cronbach's alpha, interscale correlations, test-retest reliability, and construct validity (convergent and discriminant validity). A total of 301 JIA patients (3.3% systemic, 41.2% oligoarticular, 25.9% RF negative polyarthritis, and 29.6% other categories) and 74 healthy children were enrolled in three centres. The JAMAR components discriminated well healthy subjects from JIA patients. All JAMAR components revealed good psychometric performances. In conclusion, the Norwegian version of the JAMAR is a valid tool for the assessment of children with JIA and is suitable for use both in routine clinical practice and clinical research.

Objectives: To describe the characteristics, outcome and predictive factors of juvenile mixed connective tissue disease (JMCTD) in a nationwide cohort of patients., Methods: We examined 55 patients with JMCTD after a mean disease duration of 16.2 years (SD 10.0). Patients were registered according to Kasukawa's criteria. Remission criteria were defined according to those for juvenile idiopathic arthritis, plus absence of cytopenia, myositis, progressive sclerodactyly, lung and oesophageal manifestations. Organ damage was assessed with the Systemic Lupus International Collaborating Clinics (SLICC)/American College of Rheumatology (ACR) Damage Index and the Juvenile Arthritis Damage Index (JADI). Medical records were reviewed for early predictors for outcome, which were assessed by multivariate logistic regression analyses., Results: Three patients developed systemic lupus erythematosus (SLE). Fifty-two patients had continuous JMCTD; the most common manifestations were: Raynaud (100%), arthritis (94%), puffy hands (77%) and pulmonary manifestations (58%). SLE-like, systemic sclerosis (SSc)-like and polymyositis (PM)-like findings were found in 98%, 77% and 48%, respectively. Over time, SLE-like and PM-like manifestations decreased, and SSc-like findings increased. At follow-up, 35 patients (67%) had active disease and 17 (33%) were in remission. In 34 patients (65%), SLICC or JADI≥1 assessments indicated organ damage. Active disease was associated with higher anti-ribonucleoprotein antibody titres at follow-up and positive rheumatoid factor (RF) at diagnosis and follow-up., Conclusions: Most patients with JMCTD had active disease and organ damage after a mean follow-up of 16.2 years. Active disease was associated with higher anti-ribonucleoprotein antibody levels and positive RF. The presence of RF at diagnosis predicted persistent disease activity., (Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://www.bmj.com/company/products-services/rights-and-licensing/.)

Objective: To compare the juvenile arthritis disease activity score (JADAS) based on C reactive protein (CRP) (JADAS-CRP) with JADAS based on erythrocyte sedimentation rate (ESR) (JADAS-ESR) and to validate JADAS in a population-based setting., Methods: The CRP and ESR values and the corresponding JADAS scores (JADAS10/27/71) were compared in a longitudinal cohort study of 389 children newly diagnosed with juvenile idiopathic arthritis (JIA) in the Nordic JIA study. The construct validity and the discriminative and predictive ability of JADAS were assessed during a median disease course of 8 years by comparing JADAS with other measures of disease activity and outcome., Results: At the first study visit the correlation between JADAS27-CRP and JADAS27-ESR was r=0.99 whereas the correlation between CRP and ESR was r=0.57. Children with higher JADAS scores had an increased risk of concomitant pain, physical disability and use of disease-modifying antirheumatic drugs (DMARDs). A higher JADAS score at the first study visit also significantly predicted physical disability, damage and no remission off medication at the final study visit, and also use of DMARDs during the disease course. Sensitivity to change, demonstrated as change in JADAS score compared with the American College of Rheumatology paediatric measures of improvement criteria, mostly showed excellent classification ability., Conclusion: The JADAS-CRP and JADAS-ESR correlate closely, show similar test characteristics and are feasible and valid tools for assessing disease activity in JIA.

Objective: To evaluate response to therapy over a 24-month period in a large prospective international cohort of patients with juvenile dermatomyositis (DM)., Methods: The study included 145 patients with recent-onset juvenile DM and 130 juvenile DM patients experiencing disease flare, all of whom were <18 years old. Disease activity parameters and therapeutic approaches in 4 geographic areas were analyzed at baseline and at 6, 12, and 24 months. Response was assessed according to the Pediatric Rheumatology International Trials Organization (PRINTO) juvenile DM response criteria, and data were reported "as observed" and in the intent-to-treat (ITT) population., Results: Patients with recent-onset juvenile DM at baseline had higher baseline disease activity and greater improvement over 24 months when compared to juvenile DM patients experiencing disease flare at baseline. Methotrexate (MTX) or high-dose corticosteroids were administered more frequently to patients with recent-onset juvenile DM, compared to juvenile DM patients experiencing disease flare, who were more likely to receive cyclosporine. Compared to patients from Western and Eastern Europe, a higher proportion of patients from South and Central America and North America received pulse steroids, and the average steroid dosage was higher in the North American and South and Central American patients. The use of MTX was similar in all 4 regions, while cyclosporin A was more frequently used in Western Europe. In the "as observed" analysis, 57.9% of the patients with recent-onset juvenile DM and 36.4% of the patients experiencing disease flare (P<0.001) reached at least a 70% response by PRINTO criteria at 6 months; these proportions had increased at month 24 to 78.4% and 51.2%, respectively (P<0.001). Corresponding results of the ITT analysis were much lower, with only one-third of the patients able to maintain the initial assigned therapy over 24 months., Conclusion: Patients with recent-onset juvenile DM are more likely to achieve significant clinical improvement over 24 months, when compared to patients experiencing flares of juvenile DM. Internationally, various therapeutic approaches are used to treat this disease., (Copyright © 2011 by the American College of Rheumatology.)

Chorea was observed in a 12-year-old girl with primary antiphospholipid syndrome (APS). She developed severe chorea in a few weeks. On immunosuppressive treatment, including high doses of glucocorticoids and cyclophosphamide, she had a rapid clinical recovery. Single photon emission computed tomography (SPECT) of the brain showed decreased circulation in the basal ganglia and in the medial parts of both temporal lobes. One month after treatment, SPECT was completely normalised. APS in children has a variety of clinical manifestations, and should be suspected in cases of unexplained thromboembolic disease or obscure neurological symptoms.