Juvenile Idiopathic Arthritis Subtypes Research Articles

Polyarticular juvenile idiopathic arthritis has a distinct co-inhibitor receptor profile.

Juvenile Idiopathic Arthritis (JIA) is the most common rheumatic disease of childhood; the pathogenesis is associated with T cell activation. T cell activation can be counter-balanced by signals generated by inhibitory receptors (IRs) such as CTLA-4, PD-1, LAG-3, and TIM-3. Here, we identify the role of IRs in the pathogenesis of different JIA subtypes. In total, we included 67 oligoarticular JIA, 12 IgM-RF negative polyarticular JIA, 17 enthesitis related arthritis, 11 systemic JIA patients and 10 healthy controls. We collected plasma (and synovial fluid) samples from the patients either at the onset or during a flare of their disease. We measured the soluble levels of co-IRs (IL-2Rα, 4-1BB, CD86, TGF-β1, CTLA-4, PD-L1, PD-1, TIM-3, LAG- 3, Galectin-9) by cytometric bead array kits and their cellular expression (PD-1, CTLA-4, TIM-3, LAG-3) by flow cytometry. We compared the plasma levels and cellular expressions of different co-IRs within different JIA subgroups. The polyarticular-JIA group was different from the three other examined JIA subgroups, having higher levels of plasma sCTLA-4(p< 0.001), sPD-1(p< 0.05), and s4-1BB(p< 0.05) when compared with the other JIA subgroups and healthy controls. We analyzed the cellular surface expression of different co-IRs on the PBMCs of different JIA subtypes. Similar to plasma levels, both the percentage(p< 0.05) and the MFI (mean fluorescence intensity) (p< 0.01) of CTLA4 expression were higher in the poly-JIA subgroup. This is the first report studying the expression profile of different co-IRs in different subtypes of JIA. Polyarticular JIA patients had a different co-IR profile, having more CTLA-4, PD-1 and 4-1BB in their plasma than the other subtypes of JIA.

Factors determining resistance to conventional disease-modifying anti-rheumatic drug treatment in oligoarticular juvenile idiopathic arthritis.

Our study was designed to investigate the reasons for starting the conventional disease-modifying anti-rheumatic drugs (DMARDs) and the variables that impact the response to DMARD treatment in oligoarticular juvenile idiopathic arthritis (JIA) patients. Oligoarticular JIA patients (n = 187) were categorized into two groups: Group A consisted of patients who achieved remission with DMARD, and Group B comprised those who did not respond to DMARD therapy. DMARDs were initiated for various reasons: 68 (36.4%) due to active disease despite nonsteroidal anti-inflammatory drugs (± intra-articular corticosteroid) treatment, 59 (31.6%) due to uveitis, 49 (26.2%) due to extended oligoarticular JIA, and 11 (5.9%) due to inflammatory bowel disease. One hundred twenty-three patients (65.8%) achieved remission with DMARDs (Group A), while 64 patients (34.2%) did not respond to DMARD therapy (Group B). In Group B, patients had higher C-reactive protein (CRP) levels as well as higher Juvenile Idiopathic Arthritis Disease Activity Scores-71 (JADAS-71) at diagnosis (both p < 0.001). Moreover, extended oligoarticular JIA subtype (p = 0.017) and involvement of small joints at diagnosis (p = 0.043) were more prevalent among these patients. Group A exhibited a higher frequency of antinuclear antibody positivity (p = 0.014). Elevated CRP levels (> 1.1mg/dL) (OR 1.308, 95% CI 1.203-3.574; p < 0.001) and high JADAS-71 at diagnosis (> 15.8) (OR 1.659, 95% CI 1.179-2.941; p < 0.001) were associated with DMARD resistance. Elevated CRP and high JADAS-71 at diagnosis were the main factors associated with DMARD resistance in oligoarticular JIA. Prospective long-term studies may help verify the role of these factors associated with DMARD resistance in oligoarticular JIA. Key Points • Conventional DMARDs were most commonly started due to active disease despite NSAID (± intra-articular corticosteroids). • Remission was achieved with DMARD in 65.8% of oligoarticular JIA patients. • Elevated CRP and high JADAS-71 at diagnosis were associated with DMARD resistance.

Caregiver burden in families of children with juvenile idiopathic arthritis in India.

Juvenile Idiopathic Arthritis (JIA) causes caregiver burden on families with children affected with it. Our study aimed to explore this multifaceted burden in the Indian context. In this cross-sectional study, we administered the Hindi translated CAREGIVER questionnaire to adult caregivers in the families of JIA patients ≤ 18years. The responses to the 28 items were used to calculate the burden scores in various dimensions. The relationship of the global burden scores with demographic and socioeconomic factors were analysed. Non parametric tests were used. Two hundred twenty-one caregivers participated with a median age of 39years (IQR 32-45). This included 116 fathers, 50 mothers, 32 brothers, 18 uncles, three grandfathers, one sister, and one grandmother. The JIA patients had a median age of 15 (12-17) years, and the male-to-female ratio was 3.2:1. Enthesitis-related arthritis was the predominant subtype (72.4%). Most caregivers (70.6%) expressed sadness at diagnosis, and 29.9% continued to express sadness. Nearly two-thirds (65.6%) had to borrow money from others. More than half (59.3%) of the caregivers neglected their health, and 9.0% became sick. Male gender of the child, systemic JIA subtype, low socioeconomic status, high disease activity, extra-articular damage, high parent-reported disease activity and poor quality of life were associated with higher global caregiver burden. JIA has a significant emotional, social, economic, and labour impact on caregivers. Economic and psychosocial support needs to be given to family caregivers caring for children with JIA.

P088 Uveitis screening in Juvenile Idiopathic Arthritis: Are we following the guidelines?

Abstract Background/Aims Juvenile Idiopathic Arthritis (JIA) is the most common inflammatory arthritis in children. Uveitis is a common (8-30%) and potentially sight-threatening complication of JIA. Early detection and treatment of uveitis can prevent permanent visual impairment, making uveitis screening an essential part of the management of JIA patients. The purpose of this audit was to evaluate the referral and management of uveitis screening in JIA patients in our healthcare setting compared with Guidelines for Screening for Uveitis in Juvenile Idiopathic Arthritis (JIA) produced jointly by BSPAR and the RCOphth 2006. Methods The patients with a diagnosis of JIA at our District General Hospital (DGH) were reviewed retrospectively. The data was collected based on demographics, JIA diagnosis, subtypes, referral for uveitis screening, timing of uveitis screening, and screening outcome. Data was then analysed to assess if we met BSPAR and the RCOphth 2006 standards. Results A total of 42 patients were identified with a diagnosis of JIA between January 2021 and January 2023. Based on our criteria, four patients were excluded and the final analysis was done with 38 patients. 34 patients (89%) with JIA were referred for uveitis screening. 31 of those patients (91%) were referred within six weeks, while three patients (9%) were referred after six weeks of diagnosis. Only nine patients (24%) were seen for uveitis screening within the six weeks after initial referral as per BSPAR and the RCOphth 2006 standards. Uveitis was diagnosed in five patients(13%). One out of 38(2.6%) patients was diagnosed with Uveitis prior to diagnosis of JIA. Regarding the distribution of JIA subtypes, there were 15 cases of Polyarticular JIA, 14 instances of Oligoarticular JIA, six occurrences of Psoriatic JIA, two instances of Enthesitis-related JIA, and 1 case of Systemic JIA. Out of the five patients diagnosed with Uveitis, two had Polyarticular JIA, two had Oligoarticular JIA, and one presented with Psoriatic JIA. Furthermore, two of these patients tested positive for ANA, one tested negative, while the ANA status remained unknown for the remaining two individuals. Conclusion The results showed the need for improvement to be fully compliant with the guidelines for screening uveitis in JIA patients. We aim to ensure that all patients are referred and reviewed within 6 weeks for uveitis screening, as early detection and treatment of uveitis can prevent permanent visual impairment. We recommend collaborating with the ophthalmology unit to streamline the referral pathway and explore the idea of doing MDT clinics. Education should be arranged to raise awareness among all relevant healthcare staff. Disclosure J. Ahmad: None. A. Gupta: None. A. Bharadwaj: None. D. Olusanya: None.

Assessment of Bone Mineral Density in a cohort of Juvenile Idiopathic Arthritis patients

Introduction: Low bone mass is encountered in patients with Juvenile idiopathic arthritis (JIA) as a serious long-term complication of this pediatric condition and may be associated with fractures and its complications.&#x0D; The present study aimed to evaluate the frequency of low bone mineral density (BMD) and fragility fractures in patients with JIA in its polyarticular or oligo articular forms. Secondary, our aim was to identify factors associated with low bone mass.&#x0D; Patients and methods: This is a retrospective study conducted on 26 patients with JIA meeting the ILAR criteria for poly or oligoarticular form. Patients were divided into two groups according to their age : group1 including patients aged over 16 years-old and group 2 including patients aged under 16 years-old. Sociodemographic, clinical, biological and functional data were collected by consulting patient’s medical records. Bone mineral density was measured at three different sites: whole body, lumbar spine and hip using the dual-energy X-ray absorptiometry (DXA) equipment. Data were entered and analyzed using SPSS version 11.5 software.&#x0D; Results: Twenty-six JIA patients including 20 females (74.1%) and six males (22.2%) were enrolled with a mean disease duration of 20.23 years [2-54 years]. The mean age was 28.2 years [7-58 years]. The distribution of JIA subtypes among patients was as follows: 6 with oligo articular JIA and 20 with polyarticular JIA. Sixteen patients (59%) practiced regular physical activity. In our series, the frequency of low bone mineral density was 41% in children with JIA and 68 % in adults with JIA. Only two patients presented a fragility fracture in adulthood represented by a hip fracture following low-energy trauma and treated surgically in both cases. No patient received an anti-osteoporotic treatment. In-group 1, there was a significant correlation between low bone mass and weight (p=0.020), BMI (p=0.028), disease duration (p=0.045), positivity of AAN (p=0.050), disease activity (p=0.019), oral corticosteroid (p=0.034) and biologic treatment (p=0.043). In-group 2, a statistically significant correlation was found between low bone mass and age at the time of JIA diagnosis (p=0.041), weight (p=0.03), physical activity (p=0.05), disease duration (p=0.021), functional impact of the disease assessed by the HAQ score (p=0.018), oral corticosteroid therapy (p=0.014) and biological therapy (p=0.012).&#x0D; Conclusion: our study showed that a combination of factors contributed to compromise bone health in patients with JIA. Fragility fractures were rare and occurring mainly in adulthood. An adequate control of disease activity, proper management of treatment, adequate calcium and vitamin D intake, as well as regular physical activity, may contribute to preserve bone health in those patients.

Clinical, Immunological and Inflammatory Characteristics among Mexican Children with Different Subtypes of Juvenile Idiopathic Arthritis: Exploring the Correlation between Anti-Cyclic Citrullinated Peptide (anti-CCP) and Rheumatoid Factor (RF).

Juvenile idiopathic arthritis (JIA) is the most common chronic rheumatic disease in childhood, affecting one to four of every 1000 children worldwide. It is characterized by joint inflammation lasting more than six weeks in children under 16 years. The aim of this study was to estimate the frequency of JIA subtypes in the Mexican patient population; compare clinical, immunological and inflammation markers by JIA subtype; and examine the correlation between these variables. We conducted a cross-sectional study of 50 patients with JIA (2-15 years). We estimated the frequency of each JIA subtype, assessed and compared the immunological characteristics (RF, ANA and anti-CCP) by JIA subtype at the time of diagnosis using Kruskal-Wallis or chi-square tests, and calculated Spearman correlation coefficients between the assessments. Our analysis included 50 patients, 29 (58%) girls and 21 (42%) boys, aged at the time of diagnosis 10.56 ± 3.99 years. The frequencies of JIA subtypes were RF-seropositive polyarthritis (34%), RF-seronegative polyarthritis (28%), systemic arthritis (16%), oligoarthritis (14%) and arthritis-related enthesitis (8%). We found a significant association between sex and JIA subtype (p = 0.014). There was a significant difference in anti-CCP levels by JIA subtype (p < 0.001). We also detected positive correlations between RF and anti-CCP (r = 0.63, p < 0.001) and between age and anti-CCP (r = 0.29, p = 0.041). Our study suggests that the frequency of the polyarticular subtypes of JIA is higher in Mexican children compared to other populations. Our findings highlight the importance of considering the presence of anti-CCP and RF as important criteria when deciding on treatment for JIA patients as elevated levels of these antibodies may indicate early forms of adult rheumatoid arthritis.

Evaluating the Diagnostic Performance of Systemic Immune-Inflammation Index in Childhood Inflammatory Arthritis: A Focus on Differentiating Juvenile Idiopathic Arthritis from Reactive Arthritis.

In pediatric care, the range of potential diagnoses for arthritis can be relatively extensive, primarily involving infectious and inflammatory causes and, to a lesser extent, oncological conditions. Specifically, when addressing inflammatory causes, differentiating between Juvenile Idiopathic Arthritis (JIA) and Reactive Arthritis (ReA) can prove to be challenging during the first weeks, owing to the lack of specific antibodies in several JIA subtypes. This single-center retrospective study of 108 children with arthritis aimed to evaluate in greater detail the complete blood count (CBC) profiles of children with JIA and ReA in greater detail. The most significant differences were noted in terms of the Systemic Immune-Inflammation Index (SII), with higher values in the JIA group. Moreover, within the JIA group, SII displayed a significant positive correlation with conventional inflammatory biomarkers, specifically C-reactive protein (ρ = 0.579) and Erythrocyte Sedimentation Rate (ρ = 0.430). It was the only independent factor associated with the presence of JIA after adjusting for age (p = 0.030). Also, even with the moderate diagnostic value, the discriminating capacity of SII was superior to those of each of its component CBC parameters according to receiver operating characteristic (ROC) analysis. In summary, this study identified elevated SII values in the JIA group compared to the ReA group, indicating the potential utility of SII as an adjuvant discriminatory marker between these two arthritis forms.

Assessment of quality of life and physical activity in patients with oligoarticular juvenile idiopathic arthritis in remission.

The purpose of this study was to evaluate physical activity (PA) and health-related quality of life (HRQOL) in children with oligoarticular juvenile idiopathic arthritis (JIA) in remission in comparison with healthy peers and to determine the disease-related factors affecting PA levels. This study was conducted with 50 oligoarticular JIA patients in remission and 50 healthy peers between 9 and 14years. Demographic and clinical characteristics, laboratory parameters, and treatments were noted from electronic medical records. HRQOL was assessed with the Pediatric Quality of Life Inventory (PedsQL). PA was evaluated with the Physical Activity Questionnaire for Children (PAQ-C). Oligoarticular JIA patients had significantly lower self-reported median PedsQL scores in the domains of school functioning and social functioning compared to the control group (67.5 (10) vs. 75 (25), p = 0.001 and 70 (15) vs. 85 (26.3), p < 0.001, respectively). The median PAQ-C score was 2.6 (1.1) in patients with JIA and 3 (0.9) in their healthy peers (p = 0.02). The PAQ-C score was 2.8 (1.2) in patients < 8years at the disease onset and 2.3 (1) in those aged ≥ 8years (p = 0.022). There was no significant difference in the number of affected joints, type of affected joint, MTX and biologic agent treatment, and remission with or without drugs with the total score of the PedsQL and PAQ-C. All PedsQL domains were positively correlated with the PAQ-C. Conclusion: Oligoarticular JIA patients demonstrated lower PA and HRQOL scores compared to healthy controls despite favorable disease control. What is Known: • Oligoarticular JIA has fewer functional limitations and disabilities compared to other JIA subtypes. • As JIA can affect all aspects of a child's life, there is a need to improve the quality of life related to the disease. What is New: • It should be considered that patients with oligoarticular JIA may show lower PA and HRQOL scores compared to healthy controls despite favorable disease control. • Since there may be a relationship between PA and HRQOL, factors that may affect PA should be investigated to provide a holistic approach to JIA treatment.

Clinical and Demographic Profile of Patients with Juvenile Idiopathic Arthritis in a Tertiary Care Center in Mumbai, Western India

Abstract Background: Juvenile Idiopathic Arthritis (JIA) is the most common cause of chronic rheumatic disease affecting children younger than 16 years of age and lasting six weeks or longer. It causes both short-term and long-term disability. There are very few epidemiological studies from western India on JIA. The objectives of this study were to identify the clinical and demographic profile of JIA patients and compare to existing epidemiological data. Material and Methods: It was a retrospective observational study carried out at BJ Wadia Hospital for children in Mumbai, over a period of 30 months. The analysis of their clinical, demographic and treatment profile was done. Results: We had a total of 61 cases, 31 were male and 30 female. Maximum cases were of SoJIA and ERA (n=18 in each, 29.5%), followed by polyarticular JIA (n=16, 26.2%) and oligoarticular JIA (n=8, 13.1%). Knee (75.4%) was the commonest joint involved. Two patients had uveitis (one with chronic and other with acute anterior uveitis). The mean ESR was 72mm and CRP 45.87mg/dL. ANA was positive in 7 patients, whereas RF in 3 patients. There was a mean 6 months delay in their diagnosis, maximum being in polyarticular JIA with 11 months dealy. All patient were treated with NSAIDs (naproxen or indomathacin). cDmards were given for treatment - either methotrexate or sulfasalzine. Twelve were given biologicals DMARDs. Conclusion: SoJIA and ERA followed by polyarticular JIA were most common subtypes of JIA in our study. Uveitis and ANA positivity were rare findings in our subset of children.

Etiological Theories of Juvenile Idiopathic Arthritis (JIA): A Narrative Review

Juvenile idiopathic arthritis (JIA) is the most common pediatric rheumatic disease. It can be classified by diverse clinical presentations across subtypes classified by inflammation, swelling, and pain. This review examines current perspectives on factors impacting JIA etiology and future areas of development. Genetic and environmental factors, including early-life nutrition (i.e. breastmilk), mode of infant delivery, antibiotic exposure, and infections have been proposed as etiological candidates in the literature. Various human leukocyte antigen (HLA) genes and non-HLA genes related to immune signaling are risk factors for specific JIA subtypes. The impact of infection on JIA development has also been examined; it remains unclear whether infection plays a protective or harmful role. Moreover, shorter durations of breastfeeding during childhood may be associated with an increased risk of JIA development, given that breast milk consumption favorably increases bifidobacterium, changes the composition of the microbiome, and increases microbial diversity. Additionally, mode of delivery can play a role in the onset of JIA as birth delivery through Cesarean section puts children at a higher risk of JIA compared to infants born vaginally. Lastly, antibiotic exposure and its dose- and timedependent relationship also play a role in the microbiome and JIA pathogenesis. This research is significant, as an increased understanding of the etiology of clinically heterogeneous JIA subtypes may improve future treatments. Current limitations, research gaps, and future directions in the field are also discussed.

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 effect of intra-articular steroid injection on the cartilage and tendon thicknesses in juvenile idiopathic arthritis.

Intra-articular corticosteroid injection (IACI) is a safe first-line or adjunct therapy that can be used in any subtype of juvenile idiopathic arthritis (JIA). However, limited studies evaluated the effect of IACI on cartilage. As a result, our study aimed to examine the distal femoral cartilage thickness of patients with JIA who received IACI to the knee joint using ultrasound imaging. We randomly selected JIA patients who performed IACI in the knee joint. Baseline bilateral joint cartilage and tendons thickness were measured. Then, the articulary fluid was aspirated, and intra articulary steroid was injected during the same period. Six months after injection, the exact measurements were repeated. Exclusion criterias were that patients had IACI past six months of the baseline measurement and more than one IACI during the study period.. Distal femoral cartilage thickness, quadriceps tendon thickness, and distal and proximal patellar tendon thicknesses were compared at baseline (before IACI) and six months after IACI. Thirty patients with JIA were included in the study, and 23 (76.7%) were female. The median age was 11 years (interquartile range (IQR), 6 to 14), and the median disease duration was 3.3 years (IQR, 5 months to 5 years). 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 six months after IACI (p=0.35). Also, the tendon thicknesses were the similar at six months after baseline measurements. Our findings reveal that knee IACI in patients with JIA did not significantly change cartilage and tendons thicknesses. This observation could indicate that IACIs have no detrimental effects on the cartilage and the tendons.

Comparison of biologic monotherapy versus biologic and disease-modifying anti-rheumatic drug combination in the treatment of non-systemic juvenile idiopathic arthritis

Purpose To explore the efficacy of biologics as mono- or combination therapy with conventional disease modifying anti-rheumatic drugs (cDMARDs) in the treatment of juvenile idiopathic arthritis (JIA). Material and Methods Medical records of patients with JIA followed-up from January 2020 to 2023 who were treated either with biologic drugs as monotherapy or with combination of cDMARD were reviewed retrospectively. Data of demographic features, clinical scores and treatments were assessed. Results Two hundred five cases received etanercept, adalimumab, or tocilizumab alone or in combination with a cDMARD for JIA were included. The male to female ratio of the cohort was almost equal. Oligoarticular was the most common subtype of JIA. Majority (n=128, 62.4%) of the group received biologic drugs as monotherapy, while the remaining third (n=77, 37.6%) used a combination of biologic and a cDMARD. Nearly half of the group (57.1%) were treated with etanercept and etanercept monotherapy was the most commonly used one among all JIA subtypes except juvenile psoriatic arthritis. Adalimumab combination therapy was prescribed in most of the children with juvenile psoriatic arthritis. Adalimumab, alone or in combination with methotrexate, was preferred for all 8 patients with uveitis at the onset of the disease. Adalimumab combined (n=9) and tocilizumab monotherapy (n=4) were the most common biologics in those who developed uveitis during follow-up. Conclusion Etanercept, adalimumab, or tocilizumab are effective and safe biologics in treatment of JIA. Considering their cost-effective properties, choosing biologic drugs timely as combined or monotherapy is effective in preventing early and late sequelae of JIA.

The childhood arthritis radiographic score of the hip: the proposal cut-off value using cluster analysis.

Juvenile idiopathic arthritis (JIA) is a chronic rheumatic disease that affects children. It is crucial to detect and treat hip involvement in JIA early to prevent functional impairment and reduced quality of life. The Childhood Arthritis Radiographic Score of the Hip (CARSH) is a validated radiographic scoring system used to assess hip involvement in JIA. In this study, we aimed to determine cut-off values for CARSH scores using cluster analysis. The study was conducted as a cross-sectional analysis and included JIA patients with hip involvement who underwent a pelvic radiograph. The same pelvic radiograph was interpreted by two experienced pediatric rheumatologists at baseline and after 3weeks by both readers for reliability. The CARSH scores were calculated for each hip four times (twice by each reader). For the 50 hips, a total of 200 interpretations of the CARSH score were obtained. Model-based clustering was employed to identify distinct groups of CARSH score interpretations and characterize the phenotype of each cluster. Twenty-five children with hip involvement were included. The mean age was 13.9 ± 4.6years. JIA subtypes were as follows: ERA in 64%, oligoarthritis in 16%, psoriatic arthritis in 12%, polyarthritis RF + in 4%, and RF - in 4% of patients. For the 200 hip interpretations, three clusters based on the level of the CARSH were identified by model-based clustering. Cluster 1 consisted of 17 CARSH score interpretations with a median score of 7 ± 3 (ranging from 1 to 15). This group primarily comprised patients with enthesitis-related arthritis (ERA) and psoriatic arthritis. Patients in cluster 1 were generally older, experienced longer diagnostic delays, and had a longer disease duration compared to the other clusters. Cluster 2 exhibited a moderate CARSH score, with an average score of 4 ± 3 (1 to 15). Patients in this group had significantly higher body weight compared to the other clusters. Cluster 3 represented the group with the least severe hip involvement, characterized by CARSH scores of 2 ± 1 (ranging from 0 to 9). This cluster had a higher proportion of male patients and higher C-reactive protein (CRP) levels than the other clusters. Regarding the individual items of the CARSH score, cluster 1 showed higher percentages of hip radiograph abnormalities such as joint space narrowing, erosions, growth abnormalities, and subchondral cysts. Cluster 2 was characterized by a high rate of acetabular sclerosis, with little to no abnormalities in other CARSH score items. Cluster 3 was the only group that exhibited hip subluxation, with minimal abnormalities in the other score items. In conclusion, this study identified three distinct groups of CARSH scores, representing varying levels of severity in hip involvement in JIA. These findings provide valuable insights for clinicians in assessing and managing JIA patients with hip involvement, enabling tailored treatment strategies based on the severity of the condition. Key Points • While a Childhood Arthritis Radiographic Score of the Hip (CARSH) is a valid and reliable tool in hip-related juvenile idiopathic arthritis, its use is limited in daily practice due to the lack of available cut-off values. • The cluster analysis defined three clusters based on the CARSH levels. • Cluster 1 exhibited the highest score with more damage and disability. Cluster 2 involved a moderate score and more overweight patients. Cluster 3 included the least level of the score but with an active disease parameter.

E65 Assessment of Tunisian rheumatologists’ common practices towards children with rheumatic diseases

Abstract Background In the past few years, many advances have been made in the subspecialty of pediatric rheumatology in emerging countries. However, some gaps were identified requiring an update of rheumatologists' knowledge in this field [1]. Our study aimed to assess rheumatologists’ knowledge and common practices when managing children with rheumatic diseases. Methods We conducted an anonymous cross-sectional study including Tunisian rheumatologists. They were invited to answer an online questionnaire. The survey included demographic data, as well as outcomes of interest: rheumatologists’ knowledge and attitudes towards the management of patients with rheumatic diseases. We also inquired about the scores used to evaluate children with juvenile idiopathic arthritis (JIA). The level of confidence in the management of the pediatric population was assessed by the Likert scale. Results At the end of the survey, 51 responses were collected. The participants were rheumatology specialists (63%), university hospital assistants (21%), and residents (16%). Sixty-two percent of responders worked in the hospital. The mean number of years of practice (including years of residency) was 16.3 ± 5.9 [2–36]. Two rheumatologists reported that they managed over 10 children per month for rheumatic disorders. Four rheumatologists reported that they managed from 6 to 9 children per month for rheumatic disorders. Forty rheumatologists reported that they managed from 1 to 5 children per month for rheumatic disorders. And 5 rheumatologists reported that they do not manage any children with rheumatic disorders. Only 10 (20%) doctors received training in pediatric rheumatology during their curriculum: online training (6), national training program (2) and international training program (2). Regarding the scores used to assess the different subtypes of JIA, 23 (45%) rheumatologists were not aware of the JADAS 10 score and 39 (76%) did not know the JSPADAS score. When inquiring about the level of confidence in the management of the pediatric population the average level of confidence was 2.49 ± 1.82 [0–5]. Only 2% were not confident at all. A pediatric specialist was requested for a second opinion upon diagnosis in 51% of difficult cases and in 12% of less challenging cases. Only 19 respondents managed the patients by themselves without referring them to other specialists. The rheumatologists' apprehension regarding the management of the pediatric population was attributed to the lack of training in pediatric rheumatology (94%), the absence of pediatric internship during the specialization curriculum (43%) and the lack of mastery of children’s examination (12%). Conclusion Our study highlighted that only a few Tunisian rheumatologists considered themselves confident in the management of children with rheumatic disorders. The establishment of training programs in pediatric rheumatology could enhance rheumatologists' knowledge in this field and give them more confidence in the management of this population. [1] Zisman D, Samad A, Ardoin SP, et al. US Adult Rheumatologists' Perspectives on the Transition Process for Young Adults with Rheumatic Conditions. Arthritis Care Res (Hoboken). 2020; 72(3):432–40. This was an anonymous cross-sectional study. All subjects participated voluntary.

E05 Is subchondral bone marrow edema predictive of bone mineral density loss in juvenile idiopathic arthritis?

Abstract Introduction Inflammation plays a crucial role in impaired bone metabolism [1]. Subchondral bone marrow oedema (BME) in Magnetic Resonance Imaging (MRI) was associated to bone mineral density (BMD) loss in adult spondyloarthritis [1]. BME was less studied in juvenile idiopathic arthritis (JIA). Objectives We aimed to study the link between MRI BME and BMD in JIA patients. Methods We conducted a retrospective study of 44 patients with JIA who fulfilled the International League of Associations for Rheumatology (ILAR) 2001 criteria. For each patient we collected the following data: age, age at the onset of JIA, JIA subtype, disease duration, C-reactive protein (CRP) and Erythrocyte sedimentation rate (ERS) levels, BMD assessed using bone densitometry and MRI. Disease activity was assessed using the JADAS score. Statistical analysis was performed using SPSS software. Results We included 28 boys and 16 girls. The mean age was 13.65 ± 4.62 years. The mean age at the onset of the disease 9.57 ± 3.97 years. The mean disease duration was 4.34 ± 3.09 years. There was enthesitis-related arthritis in 61% of the cases (n = 27), oligoarticular JIA in 14% of the cases (n = 6), polyarticular JIA in 11% of the cases (n = 5), and psoriatic arthritis in 7% of the cases (n = 3). JIA was undifferentiated in 7% of the cases (n = 3). The mean CRP and ESR were 14.42 ± 19.67 mg/l and 26.56 ± 20.87 mm, respectively. The mean JADAS was 6.6 ± 4.7. Bone densitometry was performed in 17 patients. The mean BMD and the mean Z-score were 0.961 ± 0.128 g/cm2 and -0.4 ± 1.16 standard deviation, respectively. Magnetic resonance imaging (MRI) showed BME in 52% of the patients (n = 23). Bone marrow oedema was present in the sacroiliac joints in 43% of the cases (n = 19), in the coxofemoral joints in 27% of the cases (n = 12), and in the spine in 9% of the cases (n = 4). The presence of BME was associated with a higher ESR (32.71 ± 25.71 vs 18.94 ± 10.72 mm, P = 0.032), higher JADAS (8.01 ± 5.36 vs 4.75 ± 3.29, P = 0.023), and a lower Z-score (-1.3 ± 0.77 vs 0.4 ± 0.8 standard deviation, P = 0.001). Conclusion Our study showed that MRI BME was associated with high disease activity and BMD loss in JIA patients. This result is consistent with those of Akgöl et al. [2]. Bone marrow oedema seems to predict BMD loss in JIA and should lead to optimizing the treatment.

The plasma levels of Dickkopf-1 elevated in patients with Juvenile Idiopathic Arthritis.

To explore the role of two major inhibitors of Wnt signal pathway, Dickkopf-1(DKK-1) and Sclerostin (SOST), in the pathogenesis of juvenile idiopathic arthritis (JIA). 88 patients with JIA, which including 49 patients with enthesitis-related arthritis (ERA), 21 oligoarthritis (oJIA) and 18 polyarthritis (pJIA), and 36 age-and sex-matched children as healthy controls (HC) were enrolled in this study. The plasma levels of DKK-1 and SOST, measured using commercially available ELISA kits, were analyzed the correlation between the levels of DKK-1/SOST and JIA, and were analyzed in 14 patients with JIA during before and after treatment. Plasma levels of DKK-1 were significantly higher in the patients with JIA than that in HC, the elevation of DKK-1 level was positively correlated with HLA-B27 positive JIA. DKK-1 levels dropped significantly in patient with JIA after treatment (P < 0.05). There was no significant change in SOST levels among different subtypes of JIA, patients with JIA during before and after treatment, and HC. It was suggested that the DKK-1 may have a certain correlation with the pathogenesis of JIA, and DKK-1 levels are more closely related to the HLA-B27 positive-ERA. The abnormally elevated levels of Dickkopf-1 (DKK-1) may be involved in the pathogenesis of juvenile idiopathic arthritis (JIA). DKK-1 levels were more closely related to the HLA-B27 positive-enthesitis-related arthritis (ERA). DKK-1 is an inhibitor of Wnt signaling pathway that promotes osteoblastic new bone formation; it is very rare for pediatric patients with HLA-B27 positive-ERA to manifest typical spondylitis, while sacroiliac arthritis is relatively common, which may be related to the high levels of DKK-1, which is consistent with the early stage of ankylosing spondylitis (AS).

Clinical Features and Risk Factors of Uveitis in Korean Children with Juvenile Idiopathic Arthritis: A Retrospective Cohort Study.

This study aimed to investigate the clinical features and risk factors of uveitis in Korean children with juvenile idiopathic arthritis (JIA). The medical records of JIA patients diagnosed between 2006 and 2019 and followed up for ≥1 year were retrospectively reviewed, and various factors including laboratory findings were analyzed for the risk of developing uveitis. JIA-associated uveitis (JIA-U) developed in 30 (9.8%) of 306 JIA patients. The mean age at the first uveitis development was 12.4 ± 5.7 years, which was 5.6 ± 3.7 years after the JIA diagnosis. The common JIA subtypes in the uveitis group were oligoarthritis-persistent (33.3%) and enthesitis-related arthritis (30.0%). The uveitis group had more baseline knee joint involvement (76.7% vs. 51.4%), which increased the risk of JIA-U during follow-up (p = 0.008). Patients with the oligoarthritis-persistent subtype developed JIA-U more frequently than those without it (20.0% vs. 7.8%; p = 0.016). The final visual acuity of JIA-U was tolerable (0.041 ± 0.103 logMAR). In Korean children with JIA, JIA-U may be associated with the oligoarthritis-persistent subtype and knee joint involvement.

Biological Agent Switching in Patients With Juvenile Idiopathic Arthritis: A Tertiary Center Experience.

The purpose of this study is to investigate the causes and outcomes of switching biological agents in juvenile idiopathic arthritis (JIA) patients using biological agents and compare the characteristics of patients whose biological agents are switched and those whose are not. This medical records review study was conducted with 128 patients who were diagnosed with JIA at our clinic between January 2009 and January 2022 and were receiving biologic agents. Factors affecting the biologic agent switching were investigated. The JIA subtype with the most frequent switching in biological agents was systemic JIA (n = 13, 40.6%). Systemic JIA was followed by rheumatoid factor-negative polyarticular JIA and persistent oligoarticular JIA with 5 patients (15.6%), extended oligoarticular JIA and enthesitis-related JIA with 3 patients (9.3%), rheumatoid factor-positive polyarticular JIA with 2 patients (6.2%), and undifferentiated JIA with 1 patient (3.1%). Among the patients, 32 (25%) patients had their biological agent switched once, and 5 (3.9%) had theirs switched twice. The most frequently used biological agent was etanercept (n = 76, 59.3%), whereas the most frequently observed cases of biological agent switching were from an anti-TNF agent to another anti-TNF agent (40.6%). The reason for switching was unresponsiveness to the agent in 22 patients (68.8%), adverse effects in 6 patients (18.7%), drug intolerance in 1 patient (3.1%), and other reasons in 3 patients (9.3%). The most frequently used biological agent was etanercept; the most frequent cases of biological agents switching were from an anti-TNF agent to another anti-TNF agent.

Enthesitis-related arthritis in children: A literature review of the clinical features and differential diagnosis

BACKGROUND: Enthesitis-related arthritis is one of the subtypes of juvenile idiopathic arthritis and is characterized by the involvement of the joints, enthesitis, and axial skeleton (sacroiliitis and spondylitis). The clinical variability of enthesitis-related arthritis and similar manifestations with orthopedic diseases present difficulties in diagnosis.&#x0D; AIM: To present the clinical features of enthesitis-related arthritis and issues of differential diagnosis based on literature analysis.&#x0D; MATERIALS AND METHODS: A literature search was conducted in the open electronic databases of eLibrary, PubMed, and Cochrane Library. In total, 46 foreign and 4 Russian publications were analyzed, which were limited to 19812021. The keywords used in the literature search were as follows: enthesitis, enthesitis-related arthritis, juvenile spondyloarthritis, and SAPHO syndrome. Own archive data for instrumental investigations were used in the article.&#x0D; RESULTS: The clinical manifestations can be variable, and laboratory tests do not always allow us to prove the inflammatory nature of the pain syndrome. The most priority diagnostic tests were imaging methods, namely, magnetic resonance imaging and ultrasonography. The greatest diagnostic difficulty was found in patients in whom enthesitis prevailed over arthritis, and in some cases, it was the only disease manifestation. The classification criteria used for the diagnosis of EAA were considered. The differential diagnosis of enthesitis included various orthopedic diseases. Ultrasound diagnostics of joints and enthesis should be performed in every patient with local pain musculoskeletal symptoms, which allows patients to be correctly routed.&#x0D; CONCLUSIONS: The alertness of both orthopedists in relation to enthesitis-related arthritis and the awareness of rheumatologists of the most common orthopedic diseases that affect the entheses are necessary.