Juvenile Arthritis Disease Activity Score Research Articles

Markers associated with the development of osteoporosis in children with juvenile idiopathic arthritis

Background. The purpose of the study was to investigate the association between bone mineralization le­vel in children with juvenile idiopathic arthritis (JIA) and various exogenous and endogenous factors. Materials and ­me­thods. The study group consisted of 162 children diagnosed with JIA. Medical history, clinical features, and treatment of JIA were studied. Disease activity was evaluated using the Juvenile Arthritis Disease Activity Score (JADAS-27), health status — with the Childhood Health Assessment Questionnaire (CHAQ). Complete blood count with erythrocyte sedimentation rate, biochemical analysis (C-reactive protein, total and ionized calcium) were performed, and instrumental methods (ultrasound densitometry, X-ray and ultrasound examination of joints) were used. Serum levels of osteocalcin, 25-hydroxyvitamin D (25(OH)D), and parathyroid hormone were evaluated using an enzyme-linked immunosorbent assay on an ER500 analyzer (Sinnowa, China). Results. According to our study, osteoporosis was observed in 19 patients (11 girls and 8 boys) out of 162 children with JIA, accounting for 11.7 %. It was significantly more frequent in patients with polyarthritis (73.7 %) compared to those with oligoarthritis (26.3 %) (p < 0.05). Conclusions. Osteoporosis can develop in children with JIA who do not receive systemic glucocorticoid therapy. The main markers associated with osteoporosis in children with JIA include non-infectious disease onset trigger, cervical joint involvement at disease onset, involvement of more than 3 joint groups at onset, gait disturbances, high JADAS-27 and CHAQ scores, signs of systemic inflammatory process, including elevated erythrocyte sedimentation rate.

Comparative Effectiveness of a Second Tumor Necrosis Factor Inhibitor Versus a Non-Tumor Necrosis Factor Biologic in the Treatment of Patients With Polyarticular-Course Juvenile Idiopathic Arthritis.

The objective of this study was to compare the effectiveness of a second tumor necrosis factor inhibitor (TNFi) versus a non-TNFi biologic following discontinuation of a TNFi for patients with polyarticular-course juvenile idiopathic arthritis (pJIA). Using the Childhood Arthritis and Rheumatology Research Alliance Registry, patients with pJIA who started receiving a second biologic following a first TNFi were identified. Patients were required to have no active uveitis on the index date and a visit six months after the index date. Outcome measures included Clinical Juvenile Arthritis Disease Activity Score with a maximum of 10 active joints (cJADAS10), cJADAS10 inactive disease (ID; ≤2.5) and cJADAS10 minimal disease activity (MiDA; ≤5). Multiple imputation was used to account for missing data. Adjusted odds ratios (aORs) were calculated using propensity score quintiles to compare outcomes at six months following second biologic initiation. There were 216 patients included, 84% initially received etanercept, and most patients stopped receiving it because of its ineffectiveness (74%). A total of 183 (85%) started receiving a second TNFi, and 33 (15%) started receiving a non-TNFi. Adalimumab was the most common second biologic received (71% overall, 84% of second TNFi), and tocilizumab was the most common non-TNFi second biologic received (9% overall, 58% of non-TNFi). There was no difference betweenreceiving TNFi versus non-TNFi in cJADAS10 ID (29% vs 25%; aOR 1.23, 95% confidence interval [CI] 0.47-3.20) or at least MiDA (43% vs 39%; aOR 1.11, 95% CI 0.47-2.62) at six months. Most patients with pJIA started receiving TNFi rather than non-TNFi as their second biologic, and there were no differences in disease activity at six months.

Defining criteria for disease activity states in systemic juvenile idiopathic arthritis based on the systemic Juvenile Arthritis Disease Activity Score.

To develop and validate cutoff values in the systemic Juvenile Arthritis Disease Activity Score 10 (sJADAS10) that distinguish the states of inactive disease (ID), minimal disease activity (MiDA), moderate disease activity (MoDA), and high disease activity (HDA) in children with systemic juvenile idiopathic arthritis (sJIA), based on subjective disease state assessment by the treating pediatric rheumatologist. The cutoffs definition cohort was composed of 400 patients enrolled at 30 pediatric rheumatology centers in 11 countries. Using the subjective physician rating as an external criterion, 6 methods were applied to identify the cutoffs: mapping, calculation of percentiles of cumulative score distribution, Youden index, 90% specificity, maximum agreement, and ROC curve analysis. Sixty percent of the patients were assigned to the definition cohort and 40% to the validation cohort. Cutoff validation was conducted by assessing discriminative ability. The sJADAS10 cutoffs that separated ID from MiDA, MiDA from MoDA, and MoDA from HDA were ≤ 2.9, ≤ 10, and > 20.6. The cutoffs discriminated strongly among different levels of pain, between patients with or without morning stiffness, and between patients whose parents judged their disease status as remission or persistent activity/flare or were satisfied or not satisfied with current illness outcome. The sJADAS cutoffs revealed good metrologic properties in both definition and validation cohorts, and are therefore suitable for use in clinical trials and routine practice.

Blood brain barrier permeability and astrocyte-derived extracellular vesicles in children with juvenile idiopathic arthritis: a cross-sectional study

BackgroundJuvenile idiopathic arthritis (JIA) is the most prevalent rheumatic disease in children, and the inflammatory process is widely studied, primarily characterized by its impact on joint health. Emerging evidence suggests that JIA may also affect the central nervous system (CNS). This study investigates the potential CNS involvement in JIA by analyzing the presence of astrocyte-derived extracellular vesicles (EVs) and the S100B protein in plasma, both of which are indicative of astrocyte activity and blood-brain barrier (BBB) integrity.MethodsEDTA plasma from 90 children diagnosed with JIA and 10 healthy controls, matched by age and gender, was analyzed for extracellular vesicles by flow cytometric measurement. Astrocyte-derived EVs were identified using flow cytometry with markers for aquaporin 4 (AQP-4) and glial fibrillary acidic protein (GFAP). Levels of the S100B protein were measured using a commercial ELISA. Disease activity was assessed using the Juvenile Arthritis Disease Activity Score (JADAS27, 0–57), and pain levels were measured using a visual analogue scale (VAS, 0–10 cm).ResultsOur analyses revealed a significantly higher concentration of astrocyte-derived EVs in the plasma of children with JIA compared with healthy controls. Furthermore, children with JADAS27 scores of 1 or higher exhibited notably higher levels of these EVs. The S100B protein was detectable exclusively in the JIA group.ConclusionThe elevated levels of astrocyte-derived EVs and the presence of S100B in children with JIA provide evidence of BBB disruption and CNS involvement, particularly in those with higher disease activity. These findings underscore the importance of considering CNS health in the comprehensive management of JIA. Further research is required to elucidate the mechanisms behind CNS engagement in JIA and to develop treatments that address both joint and CNS manifestations of the disease.

Efficacy and safety of canakinumab in systemic juvenile idiopathic arthritis, the first Chinese experience

BackgroundSystemic juvenile idiopathic arthritis (sJIA) is a severe form of juvenile arthritis that is characterized by chronic joint inflammation and systemic symptoms such as fever, rash, and organ involvement. Anti-IL-6 receptor monoclonal antibody tocilizumab is an effective treatment. However, some patients still experience persisting or recurrent symptoms and the real-world effectiveness of canakinumab in Chinese patients with sJIA has never been reported. Therefore, this study aimed to assess the efficacy and safety of canakinumab in Chinese patients with sJIA using real-world data.MethodsWe conducted a retrospective study on children with active sJIA. Clinical features, laboratory data, corticosteroid dosage, and adverse events (AEs) were collected at baseline and at 4, 8, 12, and 24 weeks after initiating canakinumab treatment.ResultsSeven female and four male patients were included in the study. All patients had previously been treated with tocilizumab and were administered canakinumab for 12.4 ± 3.4 months. Notably, significant improvements were observed in both clinical signs and symptoms as well as laboratory indicators. Four children under corticosteroid treatment were able to successfully discontinue their corticosteroid therapy: one at week 4, two at week 12, and one at week 24. Notably, there was a significant reduction in the number of tender and swollen joints (P = 0.0059) as well as the systemic juvenile arthritis disease activity score (P < 0.0001). The most common AE was infection, but no patients experienced serious AEs. No cases of macrophage activation syndrome or death were reported during the follow-up period.ConclusionsCanakinumab was found to be potentially efficacious and safe in Chinese patients with sJIA. No new AEs were observed with canakinumab treatment.

Canakinumab in the treatment of systemic juvenile idiopathic arthritis: a retrospective single center study in China.

Systemic juvenile idiopathic arthritis (sJIA) is characterized by excessive production of proinflammatory cytokines. As an anti-IL-1 agent, canakinumab has been approved in the USA and Europe for the treatment of sJIA patients aged ≥2 years. However, the use of canakinumab has never been reported in China. In this study, we aimed to assess the efficacy and safety of canakinumab in Chinese patients with sJIA. A total of 11 patients with sJIA who were treated with canakinumab were included in this study. Clinical data were collected retrospectively from medical records. Efficacy was evaluated by the systemic juvenile arthritis disease activity score (sJADAS). The follow-up was performed at canakinumab initiation, at months 1, 3, 6, 9 and 12, or at the last follow-up. Of the 11 patients enrolled, 91.0% (10/11) had previously received treatment with tocilizumab. The mean duration of canakinumab was 9 (3-18) months. 45.5% (5/11) of patients showed complete response, 45.5% (5/11) showed partial response, and 9.0% (1/11) showed no response. 18.2% (2/11) experienced disease flare during the treatment with canakinumab. 81.8% (9/11) of patients successfully reduced the dose of corticosteroids, with six discontinuing corticosteroids. 45.6% (5/11) of patients experienced infection. No serious adverse events occurred during the treatment with canakinumab. Canakinumab may be effective and tolerable for Chinese sJIA patients, helping to reduce the dosage of corticosteroids. However, additional researches on large samples are required to evaluate its efficacy and safety.

Validity of an ultrasonographic joint-specific scoring system in juvenile idiopathic arthritis: a cross-sectional study comparing ultrasound findings of synovitis with whole-body magnetic resonance imaging and clinical assessment

ObjectiveTo assess the validity of an ultrasonographic scoring system in juvenile idiopathic arthritis (JIA) by comparing ultrasound detected synovitis with whole-body MRI and clinical assessment of disease activity.MethodsIn a cross-sectional...

Disease activity trajectories from childhood to adulthood in the population-based Nordic juvenile idiopathic arthritis cohort

ObjectivesTo identify long-term disease activity trajectories from childhood to adulthood by using the clinical Juvenile Arthritis Disease Activity Score (cJADAS10) in juvenile idiopathic arthritis (JIA). Second, to evaluate the contribution...

Safety and effectiveness of abatacept in juvenile idiopathic arthritis: results from the PRINTO/PRCSG registry.

To report the interim 5-year safety and effectiveness of abatacept in patients with juvenile idiopathic arthritis (JIA) in the PRINTO/PRCSG registry. The Abatacept JIA Registry (NCT01357668) is an ongoing observational study of children with JIA receiving abatacept; enrolment started in January 2013. Clinical sites enrolled patients with JIA starting or currently receiving abatacept. Eligible patients were assessed for safety (primary end point) and effectiveness over 10 years. Effectiveness was measured by clinical 10-joint Juvenile Arthritis Disease Activity Score (cJADAS10) in patients with JIA over 5 years. As-observed analysis is presented according to the Strengthening the Reporting of Observational Studies in Epidemiology (STROBE) guidelines. As of 31 March 2020, 587 patients were enrolled; 569 are included in this analysis (including 134 new users) with 1214.6 patient-years of safety data available. Over 5 years, the incidence rate (IR) per 100 patient-years of follow-up of serious adverse events was 5.52 (95% confidence interval [CI]: 4.27, 7.01) and of events of special interest was 3.62 (95% CI: 2.63, 4.86), with 18 serious infections (IR 1.48 [95% CI: 0.88, 2.34]). As early as month 3, 55.9% of patients achieved cJADAS10 low disease activity and inactive disease (20.3%, 72/354 and 35.6%, 126/354, respectively), sustained over 5 years. Disease activity measures improved over 5 years across JIA categories. Abatacept was well tolerated in patients with JIA, with no new safety signals identified and with well-controlled disease activity, including some patients achieving inactive disease or remission. Clinicaltrials.gov, NCT01357668.

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.

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.

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.

A decade of progress in juvenile idiopathic athritis treatments and outcomes in Canada: results from ReACCh-Out and the CAPRI registry.

To assess changes in juvenile idiopathic arthritis (JIA) treatments and outcomes in Canada, comparing a 2005-2010 and a 2017-2021 inception cohorts. Patients enrolled within three months of diagnosis in the Research in Arthritis in Canadian Children Emphasizing Outcomes (ReACCh-Out) and the Canadian Alliance of Pediatric Rheumatology Investigators Registry (CAPRI) cohorts were included. Cumulative incidences of drug starts and outcome attainment within 70 weeks of diagnosis were compared with Kaplan Meier survival analysis and multivariable Cox regression. The 2005-2010 and 2017-2021 cohorts included 1128 and 721 patients, respectively. JIA category distribution and baseline clinical juvenile idiopathic arthritis disease activity (cJADAS10) scores at enrolment were comparable. By 70 weeks, 6% of patients (95% CI 5, 7) in the 2005-2010 and 26% (23, 30) in the 2017-2021 cohort had started a biologic DMARD (bDMARD), and 43% (40, 47) and 60% (56, 64) had started a conventional DMARD (cDMARD), respectively. Outcome attainment was 64% (61, 67) and 83% (80, 86) for Inactive disease (Wallace criteria), 69% (66, 72) and 84% (81, 87) for minimally active disease (cJADAS10 criteria), 57% (54, 61) and 63% (59, 68) for pain control (<1/10), and 52% (47, 56) and 54% (48, 60) for a good health-related quality of life. Although baseline disease characteristics were comparable in the 2005-2010 and 2017-2021 cohorts, cDMARD and bDMARD use increased with a concurrent increase in minimally active and inactive disease. Improvements in parent and patient reported outcomes were smaller than improvements in disease activity.

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.

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).

The validity and reliability of the Juvenile Arthritis Functional Assessment Report (JAFAR) in children/adolescents with Juvenile Idiopathic Arthritis: The Turkish version study

The aim of the study was to describe the validity and reliability of the Turkish version of Juvenile Arthritis Functional Assessment Report (JAFAR) in children/adolescents with Juvenile Idiopathic Arthritis (JIA). Sixty-nine children/adolescents with JIA were included in the study. JAFAR(TR)-Child and Parent forms were applied to the patients with JIA and to their parents for test retest at one-week intervals, the patients did not receive additional treatment and his/her pharmacological treatment did not change for that week. Test-retest reliability was evaluated by intraclass correlation coefficient (ICC), and internal consistency reliability of multi-item subscales was evaluated by calculating Cronbach's alpha coefficient. Correlations between JAFAR(TR)-Child and Parent with the Pediatric Quality of Life Inventory 3.0. Module Arthritis (PedsQL), the Childhood Health Assessment Questionnaire (CHAQ), and the Juvenile Arthritis Disease Activity Score (JADAS) were evaluated to determine construct validity. The ICC value for the test/retest reliability of JAFAR(TR)-Child was 0.963 and of JAFAR(TR)-Parent was 0.576. JAFAR(TR)-Child total score had low to moderate correlations with PedsQL Child (r=-0.34; p=0.004), CHAQ (r=0.40; p=0.001), and JADAS total score (r=0.42; p=0.000). JAFAR(TR)-Parent total score had moderate to high correlations with PedsQL Parent (r=-0.55; p=0.000), CHAQ (r=0.72; p=0.000) and JADAS total score (r=0.53; p= 0.000). The Turkish version of JAFAR was found to be clinically valid and reliable in JIA.

E21 Pain catastrophism in Juvenile Idiopathic Arthritis

Abstract Introduction Catastrophism, or dramatization, is a cognitive and affective response that is based on an inadequate perception of pain. It is characterized by a negative mindset and magnification of pain through the interaction of physiological and psychological processes. Thanks to the development of a pediatric version of the Pain Catastrophizing Scale (PCS), the interest in pain catastrophizing in the pediatric area is progressively increasing. Persistent pain is the most common and bothersome symptom of Juvenile Idiopathic Arthritis (JIA). Our goal was to evaluate pain catastrophism in JIA patients as well as the factors that are associated. Patients and Methods We conducted a cross-sectional monocentric study on 12–18-year-old patients with JIA. Only those who agreed to participate were included. We collected socio-demographic and clinical data including Juvenile Arthritis Disease Activity Score (JADAS) and Patient Global Assessment (PGA). Catastrophism was evaluated by the French version of the Pain Catastrophizing Scale (PCS) adapted for adolescents (PCS-Ado). A PCS score of 30 or more represents a high level of catastrophism. Regarding quantitative variables, the normality of the variables was assessed by the Kolmogorov-Smirnov test. We calculated the median and variance of data with no Gaussian distribution and the mean with Standard deviation in quantitative variables with the normal curve. For the comparison of 2 independent series, we used the Mann–Whitney test for independent series. The Spearman test was used to calculate the correlation coefficient (r) between two quantitative variables. The significance level in all statistical tests was set at 0.05. Results Our study included a total of 20 JIA patients with a male predominance of 65%. The mean age was 14.30 ± 1.87 years and the mean disease duration was 3.39 ± 2.49 years. The Enthesitis Related Arthritis (ERA) form was noted in 50% of JIA patients. The average PGA was 3.86 ± 2.88. Mean JADAS was 6.45 ± 6.05. The PCS-Ado score was 32/52 on average. In 60% of cases, there was a high level of catastrophism (PCS-Ado ≥30). No statistically significant differences were seen in PCS-Ado based on gender, age, disease duration, or JADAS. However, statistical analysis showed that the ERA form had significantly higher PCS-Ado than the non-ERA form (mean PCS-Ado: 36.87 vs 26.75; P = 0.026). We also found a positive correlation between PCS-Ado and PGA (P = 0.026; r = 0.591). Conclusion In our study, more than half of patients with JIA had high PCS scores with a significant relationship to ERA form and PGA. This leads us to systematically detect catastrophist thinking in this population to avoid amplifying pain and installing emotional distress and pain-related disability.

E19 Gamma-glutamyl transpeptidase level is correlated with disease activity in oligoarticular and polyarticular Juvenile idiopathic arthritis

Abstract Introduction Gamma-glutamyl transpeptidase (GGT) level was found to be correlated with disease activity indexes in rheumatoid arthritis [1]. This association was not yet investigated in children with oligoarticular and polyarticular Juvenile idiopathic arthritis (JIA). Objectives The present study aims to investigate the correlation between GGT level and disease-related parameters in oligoarticular and polyarticular JIA. Methods We performed a retrospective study including patients with oligoarticular and polyarticular JIA responding to the 2001 International League of Associations for Rheumatology (ILAR) classification. Sociodemographic, disease-related parameters (age of onset, disease duration, patient global assessment (PGA), Pain Visual Analogue Scale (VAS), C-Reactive protein (CRP) level, Erythrocyte Sedimentation Rate (ESR), disease activity assessed using the Juvenile Arthritis Disease Activity Score (JADAS) based on ESR), and GGT level were collected. Continuous variables were expressed with median and Interquartile range (IQR). Spearman correlation was used to assess the association between GGT levels and disease-related data. Results Eleven patients were included with a median age of 12 (IQR: 10–15) years and 8 females. Median disease duration was 4 (IQR: 4–7) years and median age of onset was 8 (IQR: 5–10) years. Five patients had oligoarticular JIA, 3 had a polyarticular with positive Rheumatoid Factor (RF) form, 2 had a polyarticular with negative RF form and one had an undifferentiated JIA. Median JADAS was 3.6 (IQR: 0–9). Median ESR, CRP, and GGT levels were 14 (10–19) mm/H, 1.8 (2–6.4) mg/l, and 14 (12–16) IU/l, respectively. A strong positive correlation was found between GGT level and JADAS (r = 0.749, P = 0.008). Level of CRP and ESR were not correlated with GGT level. Besides, patient global assessment and pain VAS were positively correlated with GGT level (r = 0.670, P = 0.049; r = 0.814, P = 0.014, respectively). No correlation was found between GGT level and disease duration and number of tender or swollen joints. Conclusion The GGT level was significantly higher in patients with more active disease, higher pain, and global assessment. This suggests that GGT level can be used as a marker of disease activity as in JIA. References 1 Vergneault H, Vandebeuque E, Codullo V, Allanore Y, Avouac J. Disease Activity Score-28 using γGT permits a dual evaluation of joint activity and cardiovascular risk. J Rheumatol 2020.

E29 Juvenile Idiopathic Arthritis (JIA) in Algeria, from diagnosis to management

Abstract Introduction Juvenile idiopathic arthritis (JIA) is the most common chronic arthritis in children in the world. Objectives to describe a series of 120 children with juvenile idiopathic arthritis (JIA), diagnosed and recruited in the pediatric departments of 5 cities; Sétif and the cities surrounding: Bordj BouArreridj, M'sila, Béjaïa, Batna, between January 1st 2012 to December 31st, 2018 (seven years). The aim is to study the epidemiological, clinical, therapeutic, evolutionary, and the quality of life at presentation, 3rd, 6th and 12th month of follow-up of patients with JIA with evaluation of the incidence and prevalence rate. Materials and methods Our longitudinal, multicentre, and ambispective study (retrospective between 2012–2015, prospective between 2016–2018), allowed us to collect 120 newly diagnosed cases of JIA who fulfilled International League of Association for Rheumatology (ILAR) criteria. Patients were followed up for a duration of 12 months. Results 120 children were studied; females were predominant (54,2%). The average age at diagnosis was 7,46 ± 3,77 years with age at disease onset of ∼6,69 ± 3,65 years and a relatively diagnostic delay of 9,34 ± 13,05 months. Oligoarticular JIA was the most frequent (35,8%), persistent oligoarticular (32,5%) and extensive oligoarticular (3,3%).This is followed by polyarticular JIA (30,8%), Rheumatoid factor (RF) (25,8%) and with RF (5%), and systemic JIA (21,7%), 4.2% psoriatic arthritis (JPsA) and 2.5% enthesitis related arthritis (ERA) with 5% of uncategorized forms. Uveitis was present in 5.8% of cases. The initial inflammatory balance is disturbed with a erythrocyte sedimentation rate (ESR) of 56,32 ± 35,54 mm at the first h, and an average C-reactive protein (CRP) of 42,84 ± 53,76 mg/l, anaemia was seen in more than two thirds of the children (67,5%), thrombocytosis 35,8% and hyperleukocytosis in almost a quarter of children (22,5%). Antinuclear Antibodies (ANA) were present in a quarter of cases (25,8%), RF in 5%, and anti-cyclic citrullinated peptides (anti-CCP) in 3,3% of cases and Human Leucocyte Antigen B27 (HLA B27) was positive in 4.2% of children. An initial activity in non-systemic forms according to the Juvenile Arthritis Disease Activity Score (JADAS10) was moderate in more than a quarter of children, and a strong initial activity according to the American College of Rheumatology (ACR) in more than half of the systemic forms. A moderate initial average functional incapacity assessed by the Childhood Health Assessment Questionnaire (CHAQ) at 1,73 ± 0,52, a moderately altered initial average quality of life Juvenile Arthritis Quality of Life Questionnaire (JAQQ) at 4,07 ± 1,12 and a pain-physician Visual Analogue Scale (VAS) initial mean at 4,98 ± 1,74. The mean annual incidence rate in Sétif during the prospective study was 3,88/100 000 children under 16 and those of the Eastern region including Sétif and the surrounding wilayas (BBA, M'sila, Béjaïa, and Batna) is 2 per 100 000 children under 16. The rate of prevalence of JIA in Sétif is 11,25/100 000 children under 16, and in the East region of the study was 5,80/100 000 children under the age of 16. Medications used were non-steroidal anti-inflammatory drugs (NSAIDs) in (83.3%) followed by disease-modifying antirheumatic drugs (DMARDs) in 65%. Methotrexate in (63,3%), glucocorticoids in (49,2%), and aspirin used in 16,7%, intra-articular corticosteroid injections (IACs) in (15.8%), biotherapy in (19,1%). 27,5% had functional rehabilitation. Evolution at one year is marked by 44.5% disease inactivity with treatment and 31,1% remission. Conclusion JIA is the most common chronic rheumatic disease in children. Nevertheless, it is considered a rare but not exceptional pathology. It is one of the main causes of short-, medium- and long-term disability. Ethics Associate Professor of pediatrics, department of medicine University Setif-Algeria (pediatric center –Setif-Algeria)

Predictors of relapse in patients with oligoarticular juvenile idiopathic arthritis in remission off medication.

The aim of this study was to evaluate the predictors of relapse in patients with oligoarticular juvenile idiopathic arthritis (oJIA) who achieved clinical remission off medication. This retrospective observational study was conducted between June 2009 and July 2022 in 126 patients with oJIA who achieved remission off medication. The relationships between relapse status and demographic, clinical and laboratory findings, and treatment details were evaluated using electronic medical records. Of the 126 oJIA patients who achieved remission off medication, 85 (67.5%) were female. Relapse occurred in 31 patients (24.6%) with remission off medication after a median of 18months (IQR 7-26). No statistically significant relationship was found between gender, age at diagnosis, oJIA subtype, number of joints, ANA, ESR, CRP level, initial Juvenile Arthritis Disease Activity Score and relapse in oJIA patients who achieved remission off medication (p = 0.66, p = 0.25, p = 1, p = 0.54, p = 0.29, p = 0.59, p = 0.95 and p = 0.52, respectively). There was a statistically significant relationship between the number of intraarticular corticosteroid injections (IACIs) and relapse (p = 0.01). Patients who underwent IACI 2-3 times had more relapses than those who never underwent IACI and those who underwent IACI only once (p = 0.01, p = 0.02, respectively). A relationship was found between the length of follow-up and relapse in patients with oJIA who achieved remission off medication (p = 0.035). Conclusion: In oJIA patients who achieve remission off medication, the probability of relapse increases in patients who need ≥ 2 IACI during the period until remission. The length of follow-up period is associated with the probability of relapse. What is Known: • Approximately one-fourth of oJIA patients who are in remission off medication have relapse. • There is a need for markers that can predict the risk of relapse in oJIA patients who achieve remission on or off medication. What is New: • The possibility of relapse should be considered in patients with oJIA who need ≥ 2 IACIs until achieving remission off medication. • The relapse rate may increase as the follow-up period prolongs in patients who achieve remission off medication.