Juvenile Arthritis Research Articles

Protocols in pediatric rheumatology (PROKIND): treat-to-target in polyarticular juvenile idiopathic arthritis

The aims of the PROKIND protocols are improvement and harmonization of the diagnostics, monitoring, treatment decision and prognosis. This article reports the results of a prospective treat-to-target observational study of patients with polyarticular juvenile idiopathic arthritis (JIA) during the first year of treatment. Disease activity was assessed with the 10-joint juvenile arthritis disease activity score (JADAS-10), functional limitation with the childhood health assessment questionnaire disability index (CHAQ-DI) and with information on overall well-being, on pain, on fatigue and on global estimation of disease activity. Overall, 129 patients with polyarticular JIA (rheumatoid factor, RF, positive (+) polyarthritis n = 22, RF negative (-) polyarthritis n = 133 from 23pediatric rheumatology institutions in Germany and Austria were recruited. Patients with initial treatment with methotrexate formed cohort1, patients with additional repeated intravenous corticosteroid pulse therapy formed cohort2 and patients with concomitant intra-articular corticosteroid administration in at least 5joints formed cohort3. The mean JADAS10 showed adecrease in disease activity from 16.4 ± 6.1 to 2.8 ± 3.6 and the decrease in the CHAQ-DI from 1.0 ± 0.8 to 0.3 ± 0.5 showed the improvement in functional capacity. Similarly, improvements in quality of life, pain and fatigue were demonstrable. A JADAS inactive disease was achieved by 18.1% at month3, 47.7% at month6 and66.7% at month12. In cohort 1 a JADAS remission was achieved by 72.4%, by 50% in cohort 2 and by 69.2% in cohort 3. An escalation to treatment with biologics was necessary in 38% of patients in cohort 1, 60% in cohort 2 and 46% in cohort 3. Using atreat-to-target approach a dramatic improvement in disease activity, functional capacity and quality of life in polyarticular JIA could be achieved. Even after 12months an inactive disease was achieved in the majority of cases.

Advances and Challenges in the Classification of Childhood Arthritis

The most appropriate classification of childhood arthritis remains controversial. Several efforts have been made over the years to devise classification systems that identify homogeneous subgroups within the disease spectrum. Although widely used, the International League of Associations for Rheumatology (ILAR) classification has shown major limitations as it was found to have failed its primary goal of identifying homogeneous disease categories. Furthermore, its use of the count of affected joints and of the presence of psoriatic features to define individual disease subsets has been criticized. A novel classification system has been proposed by the Pediatric Rheumatology International Trials Organization (PRINTO) through expert consensus. The preliminary scheme is currently being scrutinized by means a large-scale data collection aimed to formulate an evidence-based classification, whose results will likely be available in 2024. The development of a clinicobiologic classification has been tried in a proof-of-concept study by integrating meaningful biologic and clinical characteristics, including levels of proinflammatory cytokines and measures of disease activity, that defined indicators or composite variables capable of identifying homogeneous patient subgroups by cluster analysis. The current advance in biotechnology, especially genomics, proteomics and transcriptomics, may pave the way to the future identification of well-defined clusters of patients that will inform a biology-based and data-driven classification system. However, any attempt to defining biologic subtypes should be combined with precise clinical and prognostic data in order to devise a rational classification that facilitates the progress towards personalized management of children with JIA. Furthermore, the observed variability in the prevalence of disease subtypes across geographic areas and ethnic groups must be taken into account to develop a classification that is applicable on a global scale.

MRI ankle in juvenile idiopathic arthritis and its comparison with clinical disease activity scores

Objectives: Juvenile idiopathic arthritis (JIA) is a common cause of morbidity and consequent disability among youth up to 16 years of age. Our study aims to image the ankle joints of JIA patients and correlate the findings with the clinical disease activity scores. Juvenile Arthritis Disease Activity Score (JADAS-27) is used by pediatricians to assess disease activity in JIA patients. There are four factors that determine this score – Global assessment of disease activity by the physician as well as patient/parent, joint count with active disease, and erythrocyte sedimentation rate (ESR). However, these clinical scores are dependent on the clinician’s experience and observation and, therefore, subjective. Material and Methods: Thirty patients of either sex with a clinical diagnosis of JIA as per the International League of Association for Rheumatology definition with clinically involved ankle joints were included in the study. Chronic arthritis due to other causes, such as infection, trauma, and so on, was excluded. All clinically diagnosed cases of JIA were subjected to magnetic resonance imaging (MRI) evaluation on Siemens Skyra 3 Tesla High-Resolution MR system using a dedicated coil for the ankle joint. Six sub-joints for each patient are evaluated, namely - The tibiotalar joint, subtalar joint, calcaneocuboid joint, calcaneonavicular joint, naviculocuneiform joint, and tarsometatarsal joint. Image analysis and scoring shall be done for each patient at each joint as per the Juvenile Arthritis MRI SCORE, consisting of the following parameters-synovial hypertrophy score, cartilage lesion score, bone erosion score, and bone marrow change score. Each of these scores is calculated individually based on the percentage of involved bone volume. All individual scores were summed to get the total MRI Score. The data collected were then statistically analyzed. Results: A positive correlation was sought between the JADAS-27 score and the total MRI score with the P-value being 0.03. A positive correlation was also found between the total MRI score and the ESR value of the patient (P - 0.02). Conclusion: MRI findings can be a potential marker of disease activity in JIA patients and these findings could also predict the prognosis accurately in these patients. The findings are objective and comparable to the aforementioned clinical scores.

Evaluation of psychosocial aspects in patients with juvenile idiopathic arthritis

The relationship between psychological factors and the overall health of patients with Juvenile Idiopathic Arthritis (JIA) is evident, yet the specific impact of the presence of Temporomandibular Joint (TMJ) signs or symptoms is not fully understood. This article seeks to elucidate the current knowledge of the relationship between TMJ signs and symptoms, psychosocial factors, and broader clinical manifestations in JIA patients. To investigate this relationship further, the present article will also present the results of a study including a total of 73 JIA patients, consisting of 56 females and 17 males (mean age 12.2 ± 2.8 years old; range 6–16). These patients were examined according to the diagnostic criteria for temporomandibular disorders (DC/TMD). Self-reported questionnaires were used to investigate the psychosocial aspects of stress, anxiety, depression, and catastrophizing. General quality of life and disease activity were assessed with the JAMAR questionnaire (Juvenile Arthritis Multidimensional Assessment Report). For inferential statistics, the Pearson r coefficient, linear regression models and T test for unpaired data were used. The level of significance was P<0.05. Increased general disease activity and significant impacts on quality of life were associated with increased psychological distress. The four psychosocial factors examined, including anxiety, stress, catastrophizing, and depression, all had a negative impact on the quality of life, symptomatology, and disease activity. Specifically, anxiety and catastrophizing influenced the relationship between the presence of symptoms and the reported quality of life where higher levels of anxiety and catastrophizing increased the impact of the symptoms on the quality of life. Finally, the presence of TMJ signs and/or symptoms did not exacerbate the psychosocial domains but affected the functional aspect of the patient.

Mesenteric Lymphadenopathy: a Rare Case of Rosai-Dorfman Disease

Introduction: &#x0D; Rosai-Dorfman Disease (RDD) is rare with approximately 100 new cases annually in the United States and a mean age of 20.6 years [1]. RDD is characterized by massive lymphadenopathy and sinus histiocytosis [1]. Bilateral cervical lymphadenopathy is the typical presentation, however extra nodal sites have been noted [1]. This case discusses manifestations of a rare disease state. &#x0D; Case Presentation: &#x0D; A 19 year old male with a history of eczema and juvenile rheumatoid arthritis presented to the emergency room with four days of diffuse abdominal pain localized to the left upper quadrant, radiation to the right lateral ribs with nausea and diarrhea. He reported an unintentional weight loss of 30 pounds in the last three months. Physical exam revealed generalized abdominal tenderness and rebound. Computed tomography of the abdomen and pelvis (CTAP) along with routine labs were ordered. CTAP showed retroperitoneal lymphadenopathy up to two centimeters in the short axis (figure 1) and mesenteric adenopathy with a 15 millimeter lymph node in the right lower quadrant. Histopathology reported necrotizing granulomatous lymphadenitis with benign sinus histiocytes. Referral to rheumatology was made and treatment was initiated with prednisone 20 milligrams daily with plans for repeat abdominal imaging to evaluate for reduction of adenopathy. &#x0D; Discussion: &#x0D; RDD coexists with immunologic disease in 10% of cases.3 It has been associated with systemic lupus erythematous, idiopathic juvenile arthritis, autoimmune hemolytic anemia,24 and one case of RAS-associated autoimmune leukoproliferative disease [1]. The prognosis for RDD is indolent, 50% of patients experiencing resolution, one third with residual asymptomatic adenopathy and 17% with persistent symptomatology [2]. Our case highlights our patient’s associated history of juvenile rheumatoid arthritis now presenting with histological findings of RDD. A methodical approach to assessing patients with diffuse abdominal pain and ensured collaboration amongst different medical specialists will ensure favorable treatment outcomes.

Community awareness of childhood arthritis in the UK.

The aim was to measure the level of community awareness in the UK that children and young people can develop arthritis. An online survey of a representative quota sample of 2044 adults aged 16-75years in the UK was conducted between 10 and 13 February 2023 by Ipsos UK, a leading social and market research organization, with participants giving informed consent before taking part. Participants were asked which age band they thought is the earliest that someone can get arthritis. They were also asked whether a series of statements about arthritis were true or false, in addition to a series of demographic questions. Data were weighted to the known population proportions for adults aged 16-75years in the UK. Overall, 40% of respondents indicated they believed the earliest that someone could get arthritis was <16 years of age. This was higher amongst respondents with arthritis themselves or if they knew someone with arthritis. Only 19% of respondents were aware that children <5years of age can get arthritis. This varied by gender and ethnicity (males and minority ethnic groups were less likely to be aware). Minority ethnic groups were also more likely to believe a series of incorrect assumptions to be true. Awareness that children and young people can get arthritis is low, and it is lower amongst minority ethnic groups. Further research to understand this is important, to enable targeted interventions and awareness-raising resources to be developed and applied as appropriate.

The role of ultrasound diagnostics in the management of patients with juvenile psoriatic arthritis

Psoriatic arthritis (PsA) is a chronic inflammatory disease of the peripheral joints, entheses, and spine joints, affecting 10–25% of patients with psoriasis. Early diagnosis of the disease is challenging due to the possible delayed skin and joint symptoms and signs. Before the advent of ultrasound examination, the diagnosis of joint diseases was limited only to the radiological method. The ultrasound diagnosis of joint diseases in children is not established and needs a more comprehensive study.&#x0D; Materials and methods. The study included 50 PsA patients aged 3 to 17 years. We performed an ultrasound examination of 178 various joints. Knee joints were involved in 46 children, hip joints in 24, ankle joints in 34, and interphalangeal joints in 10. The mean age at onset was 7.8 years (range 1.5 to 14 years). The mean duration of the disease was 4.7 years.&#x0D; Results. Homogeneous joint effusion was the most common finding in patients with juvenile PsA (JPsA) – 83%. The effusion heterogeneity (17%) was due to hyperechoic inclusions (fibrin) in anechoic content. Diffuse synovium thickening was found in 91% of the joints. In 12.3% of joints, cartilage thinning was observed (in patients with a disease duration of 5 years or more), with fuzzy contours in some cases and normal echostructure in others.&#x0D; Conclusion. Periarticular structures, most often ligaments and tendons, are also involved in JPsA. The inflammation progresses steadily, with the rate depending on disease activity. Joint ultrasound is a reliable and highly informative method of JPsA diagnosing. It can identify a wide range of joint abnormalities; moreover, the severity of ultrasound abnormalities correlates with the disease severity, enabling using this method for monitoring disease-modifying therapy and as a screening tool for detecting the early signs of arthritis in patients with psoriasis. Joint ultrasound helps to detect early abnormalities.

The Impact of a Physiotherapy Tele-Rehabilitation Program on the Quality of Care for Children with Juvenile Idiopathic Arthritis.

To investigate the applicability and impact of a physiotherapy tele-rehabilitation program (TRP) on children with Juvenile Idiopathic Arthritis (JIA) and their families. Thirty JIA patients, applying an individualized home-exercise program (HEP), were randomly divided in the tele-rehabilitation (TRG, n=15) and control group (CG, n=15). Each TRG patient participated in a 30-minute tele-session, under a paediatric physiotherapist's supervision, twice a week, for 12 weeks. Before and after the TRP (T1 and T2, respectively), all participants and a parent/guardian completed the Juvenile Arthritis Multidimensional Assessment Report (JAMAR) questionnaire and a questionnaire regarding the HEP implementation and compliance. Residual disease was estimated at T1 and T2. At T2, TRG patients/parents completed a questionnaire evaluating the TRP. One month after T2, a reassessment of compliance with the HEP was performed. The patients' median age was 12.8 (8-16) years. At T2, the TRG patients performed the HEP significantly more frequently (p=0.023), for a longer time (p=0.034) and with less urging (p=0.004), compared to T1. Moreover, they exhibited significantly increased compliance with HEP (p=0.001), better functionality (p=0.008), better quality of life (p=0.007) and less pain (p=0.017). The CG patients showed no significant changes. Residual disease improved in both groups (TRG:p=0.002, CG:p=0.018), but more in the TRG (p=0.045). TRP's applicability and total benefit were rated as excellent by patients/parents. Finally, one month after T2, compliance with the HEP was still greater than at T1(p=0.001). An interactive physiotherapy TRP can be implemented effectively for JIA patients, providing an additional tool for their rehabilitation.

The dual role of body mass index on Juvenile Idiopathic Arthritis course: a pediatric experience.

Despite the well-known role of obesity as risk factor for Juvenile Idiopathic Arthritis (JIA) severity, emerging but limited evidence suggested a similar role for underweight. We investigated the role of body mass index (BMI) across its full spectrum in a cohort of children with JIA.We retrospectively studied 113 children with JIA classified according to the International League of Association for Rheumatology (ILAR) criteria attending our Rheumatology Clinic. The patients underwent a comprehensive evaluation including both clinical and biochemical assessments. According to BMI Z-score, the cohort was divided into five groups as underweight, normal weight, overweight (OW), obesity (OB), and severe OB. Disease activity was calculated by Juvenile Arthritis Disease Activity Score 10 (JADAS-10) joint reduced count and relapses were defined according to Wallace criteria.The mean age of the cohort was 7.43 ± 4.03years. The prevalence of underweight, normal weight, OW, OB, and severe OB was 7.2%, 54.1%, 10.8%, 17.1%, and 10.8%, respectively. Significant higher ferritin levels and erythrocyte sedimentation rate values were found in patients with severe OB and underweight compared to subjects belonging to normal weight, OW, and OB groups. A greater JADAS-10 score was observed in underweight patients and in those with severe OB than other groups. The relapse rate was higher in patients with severe OB and underweight compared to other groups. Conclusions: Both underweight and OB might negatively affect JIA course. Weight control is fundamental in children with JIA to avoid a more unfavourable course of the disease. What is Known: • Obesity represents a well-known risk factor for JIA severity. • The role of underweight in children with JIA is still poorly explored. What is New: • As observed in children with obesity, underweight young patients with JIA seem to experience a more severe JIA course. • Healthy lifestyle promotion in children with JIA is a crucial step in the management of the disease.

Clonal Trafficking of Individual T Cells between Peripheral Blood and Inflamed Synovial Tissue in Patients with Immune-Mediated Refractory Arthritis

Background Rheumatoid arthritis (RA) is the most prevalent form of immune mediated arthritis, which is characterised by immune-mediated destruction of synovial joints, leading to synovitis, acute joint pain, reduced motility, and mortality. Enrichment of CD4+ memory T cell subsets with local clonal expansions in the RA synovial tissue (ST) has been extensively reported in the literature. In addition, emerging evidence suggests that CD8+ T cells with inflammatory phenotypes are also present in increased numbers in the ST. However, little is known about whether these populations represent autoantigen-experienced migratory T cells or locally proliferated populations. Here, with the help of paired single cell T cell receptor (scTCR) and RNA sequencing recognizing individual T cell clones, we aimed to gain insights into the trafficking of T cells between peripheral blood (PB) and inflamed target tissue. Methods We collected ST and PB samples from patients with immune mediated arthritis during surgical synovectomy. The patients had been diagnosed with seronegative juvenile arthritis (Pt1-JIA), seropositive RA (Pt2-SP) and seronegative RA (Pt3-SN), and at the time of sampling they were refractory to treatment. We flow sorted T cells and CD45+ leukocytes from ST and PB and profiled them with 10x Genomics 5´-scRNA+V(D)J-seq. Altogether, we captured 68,610 cells passing quality control thresholds. We integrated the data from all individual samples using scVI and visualised the subpopulation structure using UMAP dimensionality reduction as implemented in the Seurat package. Results Altogether we analysed 34134 CD4+ T cells and 23385 CD8+ T cells from PB and ST. In concordance with previous reports, we observed strong transcriptomic differences between the tissue of origin for both CD4+ and CD8+ T cells. We pooled all CD4+ and CD8+ T cells separately and further grouped all cells with identical CDR3b aa sequences into unique clones. In CD8+ T cells, a high number of clones (n=101, pertaining to 7409 cells) was shared between PB and ST. 8 of these clones were enriched to the ST as compared to PB. Interestingly, Pt2-SP harboured a large clone in the ST consisting of 27% of the CD8+ T cells (CDR3b sequence - CASRGGTSITDTQYF). This clone was also detected in PB with a much smaller frequency of only 2% and in a phenotypically different cluster, indicating local proliferation of specific T cell clones in the synovium. By performing a DE gene analysis between cells with the same TCR but between different tissues of origin, we found that the top upregulated genes in ST were CCL4 and CREM suggesting more activated and inflamed phenotype in ST. In the CD4+ compartment, we found only 12 intersecting TCR clones pertaining to 637 cells. Majority of these clones were coming from PB (458 cells from PB and 179 from ST) and were hyperexpanded in PB as compared to ST. No matches for common viral or bacterial epitopes in the existing VDJ database for any of the intersecting clones was found. We then pooled all T cell clones that were enriched in the ST and found 113 clones (p &amp;lt; 0.05, Fisher's exact test). DE gene analysis showed that ST enriched clones have a more activated profile. Pathway enrichment analysis with hypergeometric testing against the GO Biological Process database identified T cell activation and positive regulation of cell adhesion as the top hits. Using GLIPH2, we used publicly available data from a cohort of JIA patients to check TCR level similarity between the two cohorts. We found common motifs between these two cohorts in some of the hyperexpanded clones from PB and ST, and interestingly, TCRs which were predicted to target similar antigens, were enriched to both expanded and intersecting clones. By performing DE gene analysis, we found that predicted TCRs with similarity were more cytotoxic ( GZMH, GNLY, NKG7, GZMB, GZMA) and they overexpressed the TRBV7-6 gene. Conclusions Our results suggest that the cytokine signalling environment in the ST leads to a widespread activation and inflammatory signature within the T cell compartment. This shift in the phenotype occurs within clonal families and is not due to the selective recruitment of activated cells to inflamed tissue. Additional investigations may elucidate the cellular interactions that promote expansion of specific disease promoting clones in ST and provide a more detailed understanding of the spatiotemporal aspects of this process.

Dissemination of a Pediatric Musculoskeletal POCUS Scoring System via Virtual Education: A Proof-of-Concept Study

Point of care pediatric musculoskeletal POCUS scanning and scoring protocols for childhood arthritis have emerged in recent years. However, pediatric musculoskeletal POCUS curricula in rheumatology fellowship programs are limited due to availability of trained faculty and resources. This proof-of-concept study investigated the effectiveness of educational methods for a pediatric musculoskeletal POCUS scoring protocol among fellows and physicians of differing subspecialties. Educational methods assessed included recorded videos and virtual review sessions. Effectiveness was assessed by calculating interrater reliability for the musculoskeletal POCUS scoring systems using the intra-class correlation coefficient (ICC). Following training sessions, participants then underwent scoring exercise(s) until the goal of an excellent ICC ≥ 0.75 was reached. Four participants completed two rounds of virtual education, review, and scoring sessions. Excellent interrater reliability was achieved for most views. This proof-of-concept study demonstrated virtual education covering advanced concepts of pediatric musculoskeletal POCUS provides a knowledge base for physicians from different subspecialties and various experience.

Monogenic variants in Laccase domain-containing1 (LACC1) as the cause of juvenile arthritis

Monogenic mutations in laccase domain-containing 1 (LACC1) are associated with clinical pictures that mimic severe courses of polyarticular or systemic juvenile idiopathic arthritis. The diseases are characterized by an early onset during the first year of life, afamilial clustering and ahigh inflammatory activity. The courses are mostly difficult to influence and often lead to sequelae. In this article four cases from two families are presented in which the homozygous mutation p.T276fs* in LACC1 was detected. The children initially suffered from polyarticular or systemic forms of juvenile arthritis. Of the patients two are currently being treated with tocilizumab and methotrexate and one female patient without a basis treatment is currently only receiving local repeated intra-articular steroids. A fourth female patient underwent an allogeneic bone marrow transplantation due to arelapse of an acute lymphatic leukemia. Since then, no further inflammatory symptoms have occurred. The cases presented are compared with the other 50courses published to date. In addition, recent studies investigating the influence of LACC1 mutations, particularly on macrophage function, are summarized.

Diagnostic Accuracy Study of the Pediatric-Specific Ultrasound Scoring System for the Knee Joint in Children With Juvenile Idiopathic Arthritis.

We undertook this study to validate the Pediatric Arthritis Ultrasound Scoring System for the knee joint (PAUSS-knee) in children with juvenile idiopathic arthritis (JIA). Children with JIA were enrolled to prospectively receive a musculoskeletal ultrasound (MSUS) examination of the knee and a physical examination to determine presence/absence of clinical arthritis. MSUS images were scored using the PAUSS-knee, a semiquantitative MSUS scoring system (0-3, normal to severe) for B-mode and power Doppler mode. In addition to MSUS, a subset of participants also received magnetic resonance imaging (MRI) of the knee, which was scored according to the combined Juvenile Arthritis MRI Scoring (JAMRIS) system. Spearman's correlations (rs ) were used to calculate associations between variables. Test characteristics of the PAUSS-knee were calculated with MRI as the reference standard. Inflammatory biomarkers were assessed in synovial fluid from involved knees. Eighty children with JIA contributed 112 MSUSs and 25 MRIs of the knee. Of the knees, 41% (n=46) had clinical evidence of arthritis. The B-mode PAUSS-knee score moderately correlated with clinically determined arthritis (rs = 0.54, P < 0.001) and strongly correlated with the JAMRIS score (rs = 0.75, P < 0.001). Compared with MRI, the area under the curve for the B-mode PAUSS-knee was 0.92. For a cutoff of >1, the B-mode PAUSS-knee had a sensitivity of 83% and specificity of 82%. Biomarker analysis indicates that interleukin-2R levels correlate with PAUSS score. Our data indicate that the PAUSS-knee has excellent accuracy for the diagnosis of arthritis when compared with MRI. The PAUSS-knee has the potential to effectively inform JIA medical decision-making in real time.

The role of CD14 and CSF1R in osteoarthritis and gastritis.

Osteoarthritis (OA) is a non-inflammatory degenerative joint disease that mainly involves articular cartilage damage and involves the whole joint tissue. Gastritis is a common stomach disorder, typically referring to inflammation or lesions of the gastric mucosa. However, the relationship between CD14 and colony stimulating factor-1 receptor (CSF1R) and these 2 diseases is not yet clear. OA datasets GSE46750, GSE82107 and gastritis datasets GSE54043 profiles were downloaded from gene expression omnibus databases generated by GPL10558 and GPL570.The R package limma was used to screen differentially expressed genes (DEGs). Weighted gene co-expression network analysis was performed. The construction and analysis of protein-protein interaction network, functional enrichment analysis, gene set enrichment analysis and comparative toxicogenomics database analysis were performed. TargetScan was used to screen miRNAs regulating central DEGs. A total of 568 DEGs were identified. According to the gene ontology (GO) and biological processes analysis, they were mainly enriched in ATP metabolism negative regulation, toll-like receptor TLR1:TLR2 signaling pathway, and intracellular transport. The enrichment terms for OA and gastritis were similar to the GO and Kyoto encyclopedia of gene and genome enrichment terms of DEGs, mainly enriched in ATP metabolism negative regulation, secretion granules, transmembrane receptor protein kinase activity, cytokine-cytokine receptor interaction, Toll-like receptor signaling pathway, MAPK signaling pathway, and TGF-β signaling pathway. In the Metascape enrichment projects, GO enrichment projects showed functions related to cell-cell receptor interaction, cell secretion, and growth. Two core genes were identified through the construction and analysis of the protein-protein interaction network. The core genes (CD14 and CSF1R) exhibited high expression in OA and gastritis samples and low expression in normal samples. Comparative toxicogenomics database analysis revealed associations between core genes (CD14 and CSF1R) and diseases such as OA, osteoporosis, gastritis, juvenile arthritis, diarrhea, and inflammation. CD14 and CSF1R are highly expressed in OA and gastritis, making them potential therapeutic targets for both diseases.

Comparison of Biopsychosocial Characteristics of Children with Juvenile Idiopathic Arthritis According to Common Disease Subtypes.

In this study, we assessed the functional and biopsychosocial characteristics of juvenile idiopathic arthritis (JIA) patients according to disease subtypes. Child Health Assessment Questionnaire (CHAQ), Juvenile Arthritis Disease Activity Score-71 (JADAS-71), and Juvenile Arthritis Biopsychosocial Questionnaire (JAB-Q) scales were administered to 304 JIA patients, and the subscale of JAB-Q was administered to their families. The median age of JIA patients at diagnosis was 7.9 (5.5-13) years (female/male = 1.3). Most patients were under treatment (68.7%) and had inactive disease (69.3%). While there was no significant difference between JADAS-71 scores according to the JIA subtypes, total CHAQ scores in polyarticular JIA patients were higher than in systemic JIA patients (P = .005). Enthesitis-related arthritis (ERA) patients had higher JAB-Q fatigue total scores compared to systemic JJIA patients (P = .001). Juvenile Arthritis Biopsychosocial Questionnaire-child psychosocial status scores were higher in polyarticular JIA patients than oligoarticular and systemic JIA patients (P = .004 and P = .003, respectively), and they had higher JAB-Q child form total scores than systemic JIA patients (P = .006). In addition, systemic JIA patients' parents had higher JAB-Q family total scores compared to oligoarticular JIA patients' parents (P = .03). Our results suggest that polyarticular JIA patients had higher CHAQ, JAB-Q psychosocial status, and child form total scores, and the JAB-Q fatigue score was higher in ERA patients. Also, JAB-Q-parent scores were higher in systemic JIA patients' parents. Biopsychosocial characteristics should be evaluated in both JIA patients and their parents.

The importance ofsmall joint involvement inoligoarticular juvenile idiopathic arthritis.

Our study aimed to evaluate the relationship of small joint involvement with demographic, clinical, and laboratory findings and to determine its possible effects on prognosis. This retrospective observational study was conducted in patients diagnosed with oligoarticular juvenile idiopathic arthritis (oJIA) in the paediatric rheumatology department of Ankara City Hospital between April 2009 and September 2022. The relationship between small joint involvement and demographic, clinical, and laboratory findings and prognosis was investigated by statistical methods with the data recorded from the medical records of oJIA patients. Of the 198 patients diagnosed with oJIA, small joint involvement was observed in a total of 20 (10%) patients, 11 (5.5%) at the time of diagnosis, and 9 (4.5%) during the follow-up period. The frequency of small joint involvement in extended oJIA was significantly higher than in persistent oJIA (P = .001). Patients with small joint involvement had significantly higher erythrocyte sedimentation rate and C-reactive protein values at admission (P = .047, P = .038) and Juvenile Arthritis Disease Activity Score at 3, 6, and 12 months (P = .001, P = .001, P = .018). The need for conventional disease-modifying antirheumatic drugs and biologic disease-modifying antirheumatic drugs was significantly higher in patients with small joint involvement (P = .001, P = .001). oJIA patients with small joint involvement may have higher acute phase reactants at diagnosis, a more extended course and active disease in follow-up, and the need for treatment escalation.

Current Validated Clinical and Patient Reported Disease Outcome Measures in Juvenile Idiopathic Arthritis.

Juvenile idiopathic arthritis (JIA) is a common chronic illness in childhood and comprises seven categories based on the International League of Associations for Rheumatology (ILAR) criteria. Accurate assessment and measurement of the clinical, functional, and quality of life outcomes of patients with JIA are paramount for understanding the disease course and formulating effective treatment strategies. Interest in the development and use of outcome measurements specifically focused on rheumatologic conditions has greatly expanded over the last two decades, adding to and improving upon the established disease measures. Furthermore, many of these measures have been validated using the widely accepted Outcome Measures in Rheumatology (OMERACT) core principles of instrument validation, allowing researchers and clinicians to gain confidence in these tools. This review summarizes the current validated disease outcome measures in JIA, including clinical, imaging, patient-reported, and functional outcome measurement tools, and highlights ongoing work that continues to refine and improve upon the available tools. The clinical disease outcome measures discussed in this review include physician global assessment (PhGA), American College of Rheumatology (ACR, Wallace) criteria for clinical inactive disease and clinical remission, juvenile arthritis disease activity scores (JADAS), juvenile spondyloarthritis disease activity index (JSPaDA), juvenile arthritis damage index (JADAI), and the ACR pediatric response scores. The imaging outcome measures discussed include the Dijkstra composite scores, childhood arthritis radiographic score of the hip (CARSH), and Poznanski Score. The patient-reported disease outcome measures discussed include patient global assessment (PtGA), patient-reported outcome measurement information system for JIA (PROMIS), juvenile arthritis parent/child centered disease assessment index (JAPAI, JACAI), juvenile arthritis multidimensional assessment report (JAMAR), and the Pediatric quality of life inventory rheumatology module (PedsQL). The functional outcome tools discussed include the Childhood Health Assessment Questionnaire (CHAQ), juvenile arthritis functionality scale and index (JAFS and JASI), and Juvenile Arthritis Functional Assessment Report and Scale (JAFAS and JAFAR).

Lengthening sleep reduces pain in childhood arthritis: a crossover randomised controlled trial

ObjectivesJuvenile idiopathic arthritis (JIA) is a common chronic childhood disease and chronic pain is a debilitating feature. A strong link has been shown between poor sleep and pain in JIA....

Clinical and immunological features of heart and vascular damage in children with juvenile arthritis with systemic onset

Objectives. To study the clinical and immunological features of cardiac and vascular damage in children with juvenile arthritis with systemic onset. Materials and methods. The study included 30 children with juvenile arthritis with systemic onset who were hospitalized in the cardio-rheumatology department of the Republican Specialized Scientific and Practical Medical Center of Pediatrics. All patients underwent clinical and anamnestic, functional, instrumental and laboratory research methods. Conclusion. Clinical and immunological features of lesions of the heart and blood vessels in children with JAWSO depend on the timing of the disease manifestation, the activity of the systemic inflammatory process and are characterized by impaired functional activity of the heart, overexpression of vascular endothelial growth factor - VEGF, which determines the degree of risk of cardiovascular complications in this pathology.

The effect ofintra-articular steroid injection onthe cartilage andtendon thicknesses injuvenile idiopathic arthritis.

Intra-articular corticosteroid injection (IACI) is a safe first-line or adjunct therapy used in any subtype of juvenile idiopathic arthritis (JIA). Limited studies evaluated the effect of IACI on cartilage. Our study aimed to examine the femoral cartilage thickness of patients with JIA who received IACI to the knee joint using ultrasound. We randomly selected JIA patients who performed IACI in the knee joint. Baseline bilateral joint cartilage and tendon thicknesses were measured. The articular fluid was aspirated, and applied IACI at the same period. Six months after injection, the exact measurements were repeated. Distal femoral cartilage, quadriceps tendon, and distal and proximal patellar tendon thicknesses were compared at the baseline (before IACI) and 6 months after IACI. Thirty patients with JIA were included, and 23 (76.7%) were female. The median age was 11 years (interquartile range, 6 to 14), and the median disease duration was 3.3 years (interquartile range, 5 months to 5 years). The subtypes of JIA were oligoarticular in 25 (83.3%), polyarticular in 2 (6.7%), enthesitis-related arthritis in 2 (6.7%), and juvenile psoriatic arthritis in 1 (3.3%). Distal femoral cartilage thickness was 2.96 ± 0.79 mm at baseline and 2.85 ± 0.70 mm at 6 months after IACI (P = .35). The tendon thicknesses were similar at 6 months after baseline measurements. Our findings reveal that knee IACI in patients with JIA did not significantly change cartilage and tendon thicknesses. This observation could indicate that IACIs have no detrimental effects on the cartilage and the tendons.