«Портрет» пациента с ювенильным артритом: результаты Всероссийского когортного ретроспективного исследования

AB0020 The Role of Macrophage Migration Inhibitory Factor Gene Polymorphism in Susceptibility to Juvenile Arthritis

Background:There is a need for scales to assess the quality of life among children diagnosed with Juvenile Idiopathic Arthritis (JIA), incorporating comprehensive health data [1]. In pursuit of this objective,...

The effectiveness of Janus kinase inhibitors in immunoinflammatory rheumatic, autoimmune non-rheumatic and autoinflammatory diseases is due to the involvement of cytokines in the pathogenesis of their development, the activation of which is mediated by the JAK-STAT signaling pathway in the cell nucleus. Its activation enhances the transcriptional response of genes associated with the development of autoimmune diseases and genes of proinflammatory cytokines. Janus kinase inhibitors such as tofacitinib, upadacitinib, ruxolitinib and baricitinib are currently available for pediatric patients with rheumatic and non-rheumatic diseases, but data on their efficacy and safety, as well as the list of indications for use are limited. In this literature review, we attempted to summarize the world experience of baricitinib use in children suffering from rheumatic diseases. Summarizing the results of previous studies, we can conclude that the use of baricitinib in children with juvenile arthritis is effective and safe, but further clinical trials in children with diffuse connective tissue diseases are needed. Key words: diffuse connective tissue diseases, systemic lupus erythematosus, baricitinib, juvenile idiopathic arthritis, Janus kinases

THU0502 Which is the Clinical Relevance of Ultrasound Examination in the Assessment of Established Juvenile Idiopathic Arthritis?

To assess prevalence of allergic diseases among schoolchildren in the Russian Federation (RF) in the age groups of 11 and 15 years.The data obtained during the screening of schoolchildren (which...

THU0475-HPR A waitlist controlled trial of the efficacy of a psychological treatment program for children with juvenile idiopathic arthritis and their parents

BackgroundPhysical activity (PA) patterns in children with juvenile idiopathic arthritis (JIA) over time are not well described. The aim of this study was to describe associations of physical activity (PA) with disease activity, function, pain, and psychosocial stress in the 2 years following diagnosis in an inception cohort of children with juvenile idiopathic arthritis (JIA).MethodsIn 82 children with newly diagnosed JIA, PA levels, prospectively determined at enrollment, 12 and 24 months using the Physical Activity Questionnaire for Children (PAQ-C) and Adolescents (PAQ-A) raw scores, were evaluated in relation to disease activity as reflected by arthritis activity (Juvenile Arthritis Disease Activity Score (JADAS-71)), function, pain, and psychosocial stresses using a linear mixed model approach. Results in the JIA cohort were compared to normative Pediatric Bone Mineral Accrual Study data derived from healthy children using z-scores.ResultsAt enrollment, PA z-score levels of study participants were lower than those in the normative population (median z-score − 0.356; p = 0.005). At enrollment, PA raw scores were negatively associated with the psychosocial domain of the Juvenile Arthritis Quality of Life Questionnaire (r = − 0.251; p = 0.023). There was a significant decline in PAQ-C/A raw scores from baseline (median and IQR: 2.6, 1.4–3.1) to 24 months (median and IQR: 2.1, 1.4–2.7; p = 0.003). The linear mixed-effect model showed that PAQ-C/A raw scores in children with JIA decreased as age, disease duration, and ESR increased. The PAQ-C/A raw scores of the participants was also negatively influenced by an increase in disease activity as measured by the JADAS-71 (p < 0.001).ConclusionCanadian children with newly diagnosed JIA have lower PA levels than healthy children. The decline in PA levels over time was associated with disease activity and higher disease-specific psychosocial stress.

SAT0506 Towards the Development of an Ultrasound Composite Disease Activity Score for Juvenile Idiopathic Arthritis

Selective IgA deficiency is the most frequent primary immunodeficiency and is associated with an increased rate of respiratory infections in susceptible patients. Juvenile idiopathic arthritis is an autoimmune disease in children affecting the joints and is frequently treated with mild to moderately active immunosuppressive medications. We describe a pediatric patient with juvenile idiopathic arthritis who developed a selective IgA deficiency at age 4 years, with well-documented prior IgA levels within the normal range for that age. This girl was previously diagnosed with arthritis at age 2 years; she was treated with methotrexate starting at age 24 months, and etanercept for 18 months starting at age 32 months. The development of IgA deficiency occurred after discontinuation of etanercept treat ment. She did not exhibit an increased rate of infections or other clinical signs of immunodeficiency. A work-up for immunodeficiency demonstrated increased levels of transitional B-cells, but no other abnormal findings. Congenital IgA deficiency is well known to be linked to autoimmune diseases. Acquired IgA deficien cy has previously been described in a very small number of pediatric and adult patients, and is usually associated with some form of immune dysregulation or inciting event. Four patients with juvenile arthritis with acquired IgA deficiency of varying levels have been reported, but biologic agents have not been implicated previously.

New or vanishing frontiers: LACC1-associated juvenile arthritis.

Sarcopenia is characterized by a loss of skeletal muscle mass, a decrease in muscle strength and/or physical performance, and is one of the main causes for limiting daily activities in the elderly. This is associated with an increased incidence of many adverse events such as dysfunction, falls, frailty, hospitalization, disability, and mortality. Primary (considered as a part of the aging process) and secondary sarcopenia (due to malabsorption, immobility/bed rest, starvation, hypothyroidism, osteoporosis, immune-mediated rheumatic diseases) are united by a chronic inflammatory process of different degrees. Sarcopenia supports one of the most widely accepted theories that low-grade chronic inflammation is important in the pathogenesis of many diseases. For a long time, sarcopenia was considered an age-related disease, but recently it has been reported to be more common in young subjects with autoimmune diseases. In particular, the relationship between sarcopenia and rheumatic diseases such as rheumatoid arthritis has been studied in detail. Although the pathogenesis of sarcopenia in autoimmune diseases is not fully understood, it is believed that a chronic inflammatory process contributes to the development of loss of muscle mass and strength, and is different depending on the underlying disease. The definition of sarcopenia varies between studies, which complicates and limits direct comparisons. Therefore, in this review, we demonstrate various diagnostic criteria for sarcopenia, focusing on its prevalence in patients with rheumatic diseases, including rheumatoid arthritis, juvenile idiopathic arthritis, axial spondylitis, psoriatic arthritis, and systemic sclerosis. We developed a structured search strategy for English language publications in PubMed using the term “sarcopenia” in combination with the following keywords: “inflammation”, “diagnosis”, “criteria”, “muscle mass”, “strength”, “outcomes”, “disability”, “mortality”, “pathophysiology”, “rheumatoid arthritis”, “juvenile arthritis”, “axial spondylitis”, “psoriatic arthritis”, “systemic sclerosis”. We focused on clinical trials, meta-analyses and review articles. Articles published only after 2000 year were included, however, we did not include major contributions published before. The search was completed on October 8, 2022.

Background:In autoimmune diseases that onset during childhood, the gender disparity is typically less pronounced compared to adulthood, likely due to distinct hormonal environments, with estrogen playing a significant role in...

BackgroundFoot and ankle problems are a common but relatively neglected manifestation of juvenile idiopathic arthritis. Studies of medical and non-medical interventions have shown that clinical outcome measures can be improved. However existing data has been drawn from small non-randomised clinical studies of single interventions that appear to under-represent the adult population suffering from juvenile idiopathic arthritis. To date, no evidence of combined therapies or integrated care for juvenile idiopathic arthritis patients with foot and ankle problems exists.Methods/designAn exploratory phase II non-pharmacological randomised controlled trial where patients including young children, adolescents and adults with juvenile idiopathic arthritis and associated foot/ankle problems will be randomised to receive integrated podiatric care via a new foot care programme, or to receive standard podiatry care. Sixty patients (30 in each arm) including children, adolescents and adults diagnosed with juvenile idiopathic arthritis who satisfy the inclusion and exclusion criteria will be recruited from 2 outpatient centres of paediatric and adult rheumatology respectively. Participants will be randomised by process of minimisation using the Minim software package. The primary outcome measure is the foot related impairment measured by the Juvenile Arthritis Disability Index questionnaire's impairment domain at 6 and 12 months, with secondary outcomes including disease activity score, foot deformity score, active/limited foot joint counts, spatio-temporal and plantar-pressure gait parameters, health related quality of life and semi-quantitative ultrasonography score for inflammatory foot lesions. The new foot care programme will comprise rapid assessment and investigation, targeted treatment, with detailed outcome assessment and follow-up at minimum intervals of 3 months. Data will be collected at baseline, 6 months and 12 months from baseline. Intention to treat data analysis will be conducted.A full health economic evaluation will be conducted alongside the trial and will evaluate the cost effectiveness of the intervention. This will consider the cost per improvement in Juvenile Arthritis Disability Index, and cost per quality adjusted life year gained. In addition, a discrete choice experiment will elicit willingness to pay values and a cost benefit analysis will also be undertaken.Trial RegistrationTrial registration number: UKCRN5045

THU0526 LONG-TERM OUTCOME IN JUVENILE IDIOPATHIC ARTHRITIS – A POPULATION-BASED STUDY FROM SWEDEN

The study aims to evaluate the clinical significance of serum levels of tumor necrosis factor alpha (TNF-α) and -308 A/G promoter polymorphism in juvenile idiopathic arthritis (JIA) patients and find any association to the subsets, clinical and laboratory features, disease activity, and damage as well as functional disability. Forty-eight JIA children and 30 controls were included in the present study. Juvenile arthritis disease activity score in 27 joints (JADAS-27) was calculated, juvenile arthritis damage index (JADI) was assessed, and Childhood Health Assessment Questionnaire (CHAQ) measured the functional status. Serum TNF-α was assayed by ELISA and gene (-308) promoter polymorphism was determined by polymerase chain reaction. The 48 JIA children (mean age 11.5±2.8years) were 13 systemic, 17 oligoarticular, and 18 polyarticular onset. The serum TNF-α was significantly higher in patients (90.4±6.3ng/ml) compared to control (3.5±2.6ng/ml) (p<0.0001) with a tendency to be higher in the polyarticular subtype. All controls had TNF-α -308 GG alleles. The frequency of GG genotype tended to be higher in systemic onset compared to oligoarticular and polyarticular subtypes. The serum TNF-α significantly correlated with JADAS-27 (r=0.32, p=0.03) and CHAQ (r=0.37, p=0.01) and negatively with the presence of GG alleles (r=-0.48, p=0.001). The GG alleles were significantly negatively associated with C-reactive protein (r=-0.32, p=0.03) with a tendency to negatively correlate with JADAS-27, CHAQ, and JADI-extrarticular (r=-0.28, p=0.06; r=-0.25, p=0.09 and r=-0.25, p=0.09, respectively). There is evidence of a possible influence of the -308 SNP promoter position on the production of TNF-α, the severity of JIA which may consequently influence the response to anti-TNF-α treatment.