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Systemic juvenile idiopathic arthritis (sJIA) and adult-onset Still’s disease (AOSD) are considered the same disease, but a common approach for diagnosis and management is still missing.MethodsIn May 2022, EULAR and PReS endorsed a proposal for a joint task force (TF) to develop recommendations for the diagnosis and management of sJIA and AOSD. The TF agreed during a first meeting to address four topics: similarity between sJIA and AOSD, diagnostic biomarkers, therapeutic targets and strategies and complications including macrophage activation syndrome (MAS). Systematic literature reviews were conducted accordingly.ResultsThe TF based their recommendations on four overarching principles, highlighting notably that sJIA and AOSD are one disease, to be designated by one name, Still’s disease.Fourteen specific recommendations were issued. Two therapeutic targets were defined: clinically inactive disease (CID) and remission, that is, CID maintained for at least 6 months. The optimal therapeutic strategy relies on early use of interleukin (IL-1 or IL-6 inhibitors associated to short duration glucocorticoid (GC). MAS treatment should rely on high-dose GCs, IL-1 inhibitors, ciclosporin and interferon-γ inhibitors. A specific concern rose recently with cases of severe lung disease in children with Still’s disease, for which T cell directed immunosuppressant are suggested. The recommendations emphasised the key role of expert centres for difficult-to-treat patients. All overarching principles and recommendations were agreed by over 80% of the TF experts with a high level of agreement.ConclusionThese recommendations are the first consensus for the diagnosis and management of children and adults with Still’s disease.

By improving core strength and stability, children with JIA can experience increased endurance, reduced pain, and improved posture. No study was found that investigated the effectiveness of 3-Dimension (3D) exercises in JIA. This study aimed to compare the effectiveness of structured 3D exercises with that of a conventional exercise program specially planned for children with JIA who have scoliosis. This was a prospective, randomized-controlled study. Structured 3D scoliosis exercises for Group 1 (n: 25) and conventional exercises for the Group 2 (n: 25) were applied for 24 weeks. The angle of trunk rotation (ATR) by scoliometer, scoliosis angle by Cobb angle, pain by a numerical rating scale (NRS), respiratory functions by spirometry, and perception of cosmetic deformity by The Walter Reed visual assessment scale (WRVAS) were evaluated. ATR, Cobb angle, and pain in Group I showed significant improvement compared to Group II. While the sub-parameters of WRVAS and increased significantly in both groups, the improvement in Group I was found to be greater between the groups. While FVC (%) and FEV1 (%) results within the group were significant in both groups. 3D exercises and conventional exercises are an effective and feasible method in the treatment of scoliosis in these children. Despite the curative effect of both methods, 3D exercises have been proven in this study to be more effective on Cobb angle, ATR, WRAS, and respiratory parameters.

Background Genuine uncertainty on superiority of one intervention over the other is called equipoise. Physician-investigators in randomized controlled trials (RCT) need equipoise at least in studies with more than minimal risks. Ideally, this equipoise is also present in patient-participants. In pediatrics, data on equipoise are lacking. We hypothesize that 1) lack of equipoise at enrolment among parents may reduce recruitment; 2) lack of equipoise during participation may reduce retention in patients assigned to a less favoured treatment-strategy. Methods We compared preferences of parents/patients at enrolment, documented by a questionnaire (phase 1), with preferences developed during follow-up by an interview-study (phase 2) to investigate equipoise of child-participants and parents in the BeSt-for-Kids-study (NTR 1574). This trial in new-onset Juvenile Idiopathic Arthritis-patients consists of three strategies. One strategy comprises initial treatment with a biological disease-modifying-antirheumatic-drug (DMARD), currently not standard-of-care. Semi-structured interviews were conducted with 23 parents and 7 patients, median 11 months after enrolment. Results Initially most parents and children were not in equipoise. Parents/patients who refused participation, regularly declined due to specific preferences. Many participating families preferred the biological-first-strategy. They participated to have a chance for this initial treatment, and would even consider stopping trial-participation when not randomized for it. Their conviction of superiority of the biological-first strategy was based on knowledge from internet and close relations. According to four parents, the physician-investigator preferred the biological-first-strategy, but the majority ( n  = 19) stated that she had no preferred strategy. In phase 2, preferences tended to change to the treatment actually received. Conclusions Lack of equipoise during enrolment did not reduce study recruitment, mainly due to the fact that preferred treatment was only available within the study. Still, when developing a trial it is important to evaluate whether the physicians’ research question is in line with preferences of the patient-group. By exploring so-called ‘informed patient-group’-equipoise, successful recruitment may be enhanced and bias avoided. In our study, lack of equipoise during trial-participation did not reduce retention in those assigned to a less favoured option. We observed a change for preference towards treatment actually received, possibly explained by comparable outcomes in all three arms.

Background Juvenile idiopathic arthritis (JIA) is a common chronic inflammatory disorder in children, leading to significant physical and psychosocial challenges. Current treatments often fall short, prompting interest in complementary therapies. This study aims to evaluate the effectiveness of electroacupuncture (EA) on clinical outcomes in children with JIA, focusing on its impact on the pyroptosis pathway and autophagy function. Methods A randomized controlled clinical trial was conducted from 7 November 2023 to 12 January 2025. A total of 106 participants were recruited and randomly assigned to receive EA or sham acupuncture (SA) for 8 weeks. Clinical assessments, including functional ability, pain scores, and quality of life, were measured at baseline, week 4, week 8, month 3, and month 6. Serum levels of pyroptosis-related proteins and autophagy markers were analyzed to elucidate the underlying mechanisms. Results The EA group showed significant improvements in functional ability and pain scores compared to the SA group at both week 4 and week 8. Notable reductions in serum levels of pyroptosis markers (caspase-1, GSDMD, NLRP3) and pro-inflammatory cytokines (IL-1β, IL-18) were observed in the EA group. Additionally, autophagy markers (LC3, Becline1) significantly decreased after EA treatment, suggesting enhanced autophagic activity. Conclusion This study demonstrates that electroacupuncture is a safe and effective adjunctive treatment for improving function and reducing pain in children with JIA. The observed biological changes indicate potential anti-inflammatory effects, supporting EA’s role in comprehensive JIA management. Future research should explore long-term outcomes and mechanisms for these findings further. Key Points • The study demonstrates that electroacupuncture (EA) significantly improves functional ability and pain scores in children with juvenile idiopathic arthritis (JIA) compared to sham acupuncture. • EA treatment led to notable reductions in serum levels of pyroptosis-related proteins and pro-inflammatory cytokines, indicating its potential role in modulating inflammatory pathways. • The research observed significant changes in autophagy markers post-EA treatment, suggesting that EA may enhance autophagic function in children with JIA. • EA is presented as a safe adjunctive therapy for JIA management, with implications for further research into its long-term effects and underlying mechanisms.

Objective: This study intended to examine the effects of Pilates exercise on pain, cardiorespiratory fitness, functional ability, and quality of life in children with polyarticular juvenile idiopathic arthritis. Methods: Forty children with polyarticular JIA aged 10–14 years old were randomly allocated into two groups: the control group (n = 20) received conventional physical therapy (CPT), and the experimental group (n = 20) received clinical Pilates exercises combined with CPT. Patients in both groups received their program three times/week for 3 months. Pain, cardiorespiratory fitness, functional ability, and quality of life were assessed through the visual analogue scale, cardiopulmonary exercise test, 6 min walk test, and PedsQL scale, respectively, just before and after treatment. Results: Pain (p = 0.001), cardiorespiratory markers (all p < 0.05), functional ability (p = 0.002), and overall quality of life (p = 0.007) improved significantly in the experimental groups compared to the control group. Conclusion: Incorporating Pilates exercises into CPT is likely more effective for decreasing pain intensity, improving cardiorespiratory fitness, augmenting functional ability, and promoting quality of life in children with JIA than CPT alone.

The objective of this study is to develop evidence-based recommendations for the diagnosis and management of enthesitis-related arthritis (ERA) and juvenile psoriatic arthritis (JPsA) in the African context. The recommendations for ERA and JPsA were combined into a single document. The steering committee and task force identified 15 key questions and formulated 35 research questions. A comprehensive literature review, utilizing Medline and a manual search for African local data, was conducted to gather evidence. Following this synthesis, the task force developed draft recommendations and engaged in a Delphi process with an expert panel, including 17 African and three international experts, to reach a consensus and ensure alignment with global standards. The final recommendations were assigned a level of evidence and subsequently approved by the task force members, the expert panel, and the PAFLAR Board. Fifteen recommendations on the diagnosis and management of ERA and JPsA were developed, covering the role of the pediatric rheumatologist in multiple aspects of disease management, including diagnosis, monitoring of disease and extra-articular manifestations, determining treatment strategies, and guiding interventions. The level of evidence supporting these recommendations was variable, leading to the identification of a research agenda to address African particularities and answer pending questions. The final recommendations achieved a high level of agreement, with consensus ranging from 90 to 100%. These recommendations represent an important achievement for pediatric rheumatology in Africa, being the first of their kind, tailored specifically to the region. Developed through a rigorous methodology and collaboration between international and African experts, they aim to standardize care and address the unique challenges faced in African setting.

In a randomized trial, children with uveitis associated with juvenile idiopathic arthritis who were taking a stable dose of methotrexate were significantly less likely to have treatment failure but more likely to have adverse events with adalimumab than with placebo. Juvenile idiopathic arthritis (JIA) is the most common rheumatic disease in children. Children with JIA are at risk for inflammation of the uvea (uveitis). Uveitis develops in approximately 12 to 38% of patients with JIA within 7 years after the onset of arthritis. 1 , 2 Despite current screening and therapeutic options, visual impairment in both eyes may develop in up to 15% of children with JIA-associated uveitis, and they may be certified as legally blind. 3 , 4 Experimental models of autoimmune uveitis have shown that tumor necrosis factor α (TNF-α) plays a pivotal role in the pathogenesis, 5 findings that have been borne . . .

BackgroundTreatment options in patients with enthesitis-related arthritis (ERA) and juvenile psoriatic arthritis (JPsA) are currently limited. This trial aimed to demonstrate the efficacy and safety of secukinumab in patients with active ERA and JPsA with inadequate response to conventional therapy.MethodsIn this randomised, double-blind, placebo-controlled, treatment-withdrawal, phase 3 trial, biologic-naïve patients (aged 2 to <18 years) with active disease were treated with open-label subcutaneous secukinumab (75/150 mg in patients <50/≥50 kg) in treatment period (TP) 1 up to week 12, and juvenile idiopathic arthritis (JIA) American College of Rheumatology 30 responders at week 12 were randomised 1:1 to secukinumab or placebo up to 100 weeks. Patients who flared in TP2 immediately entered open-label secukinumab TP3 that lasted up to week 104. Primary endpoint was time to disease flare in TP2.ResultsA total of 86 patients (median age, 14 years) entered open-label secukinumab in TP1. In TP2, responders (ERA, 44/52; JPsA, 31/34) received secukinumab or placebo. The study met its primary end point and demonstrated a statistically significant longer time to disease flare in TP2 for ERA and JPsA with secukinumab versus placebo (27% vs 55%, HR, 0.28; 95% CI 0.13 to 0.63; p<0.001). Exposure-adjusted incidence rates (per 100 patient-years (PY), 95% CI) for total patients were 290.7/100 PY (230.2 to 362.3) for adverse events and 8.2/100 PY (4.1 to 14.6) for serious adverse events in the overall JIA population.ConclusionsSecukinumab demonstrated significantly longer time to disease flare than placebo in children with ERA and JPsA with a consistent safety profile with the adult indications of psoriatic arthritis and axial spondyloarthritis.Trial registration number NCT03031782.

BackgroundJuvenile idiopathic arthritis (JIA) is the most prevalent rheumatic disease in children. The core stability exercises ensure proper muscular strength and balance around the lumbo-pelvic-hip complex.ObjectiveThis study evaluated whether the use of core stability exercises would increase the effectiveness of conventional physical therapy (PT) in enhancing bone mineralization and improving functional capacity in children with polyarticular JIA.MethodsThirty-three children with polyarticular JIA (age; 10–14 years) assigned randomly into two groups: the control group (n = 16) received the conventional PT, and the study group (n = 17) received the core stability exercises in addition to the same conventional PT program. Both core stability and conventional PT exercises continued for 3 months. The measures of bone mineralization and functional ability were investigated by dual-energy X-ray absorptiometry (DXA) device and 6-min walk test (6MWT), respectively, at baseline and immediately post-treatment.ResultsAnalysis of covariance (ANCOVA) revealed significant differences between groups in favor of the study group regarding measures of bone mineralization of lumbar spine and femoral neck regions as P < 0.05, except for volumetric bone mineral density of lumbar spine the P > 0.05. There was a significant difference between the two groups concerning functional capacity measured in 6MWT (P < 0.05), where children in the study group walked 531.71 ± 90.59 m compared with the control group 509.31 ± 73.10 m.ConclusionCore stability exercises are an effective adjunctive therapy to enhance bone health status and improve functional capacity in children with polyarticular JIA.Key Points• In addition to conventional physical therapy, core stability exercises had a definite effect on improving bone health status and quality of life in children with polyarticular juvenile idiopathic arthritis.• Improved bone mineralization and functional capacity due to core stability exercises contain two parts: strengthening training and controlling equilibrium.

Tofacitinib is an oral Janus kinase inhibitor. This trial assessed the efficacy and safety of tofacitinib versus placebo in patients with polyarticular course juvenile idiopathic arthritis (JIA). This double-blind, withdrawal phase 3 trial enrolled patients with polyarticular course JIA (extended oligoarthritis, rheumatoid factor-positive or rheumatoid factor-negative polyarthritis, or systemic JIA without active systemic features) aged 2 years to younger than 18 years, and was done at 64 centres of the Paediatric Rheumatology International Trials Organisation and Pediatric Rheumatology Collaborative Study Group networks in 14 countries. Patients with psoriatic arthritis or enthesitis-related arthritis were enrolled for exploratory endpoints. During part 1 of the study, patients received oral open-label tofacitinib (weight-based doses; 5 mg twice daily or lower) for 18 weeks. Patients achieving at least JIA/American College of Rheumatology 30 response were randomly assigned (1:1) using an Interactive Response Technology system to continue tofacitinib or switch to placebo in part 2 of the study for 26 weeks. The primary endpoint was JIA flare rate by week 44 in part 2 in patients with polyarticular course JIA; the intention-to-treat principle was applied. Safety was evaluated throughout part 1 and part 2 of the study in all patients who received one dose or more of study medication. This trial is registered with ClinicalTrials.gov, NCT02592434. Between June 10, 2016, and May 16, 2019, of 225 patients enrolled, 184 (82%) patients had polyarticular course JIA, 20 (9%) had psoriatic arthritis, and 21 (9%) had enthesitis-related arthritis. 147 (65%) of 225 patients received concomitant methotrexate. In part 2, 142 patients with polyarticular course JIA were assigned to tofacitinib (n=72) or placebo (n=70). Flare rate by week 44 was significantly lower with tofacitinib (21 [29%] of 72 patients) than with placebo (37 [53%] of 70 patients; hazard ratio 0·46, 95% CI 0·27–0·79; p=0·0031). In part 2 of the study, adverse events occurred in 68 (77%) of 88 patients receiving tofacitinib and 63 (74%) of 85 in the placebo group. Serious adverse events occurred in one (1%) and two (2%), respectively. In the entire tofacitinib exposure period, 107 (48%) of 225 patients had infections or infestations. There were no deaths during this study. The results of this pivotal trial show that tofacitinib is an effective treatment in patients with polyarticular course JIA. New oral therapies are particularly relevant for children and adolescents, who might prefer to avoid injections. Pfizer.