Juvenile Idiopathic Arthritis Subtypes Research Articles

Genetic Background and Molecular Mechanisms of Juvenile Idiopathic Arthritis.

Juvenile idiopathic arthritis (JIA) is the most common chronic rheumatic disease in the paediatric population. JIA comprises a heterogeneous group of disorders with different onset patterns and clinical presentations with the only element in common being chronic joint inflammation. This review sought to evaluate the most relevant and up-to-date evidence on current knowledge regarding the pathogenesis of JIA subtypes to provide a better understanding of these disorders. Despite significant improvements over the past decade, the aetiology and molecular mechanisms of JIA remain unclear. It has been suggested that the immunopathogenesis is characterised by complex interactions between genetic background and environmental factors that may differ between JIA subtypes. Human leukocyte antigen (HLA) haplotypes and non-HLA genes play a crucial role in the abnormal activation of both innate and adaptive immune cells that cooperate in causing the inflammatory process. This results in the involvement of proinflammatory cytokines, including tumour necrosis factor (TNF)α, interleukin (IL)-1, IL-6, IL-10, IL-17, IL-21, IL-23, and others. These mediators, interacting with the surrounding tissue, cause cartilage stress and bone damage, including irreversible erosions. The purpose of this review is to provide a comprehensive overview of the genetic background and molecular mechanisms of JIA.

Juvenil İdiyopatik Artiritte Karotis İntima Media Kalınlığı ve Preklinik Ateroskleroz için Risk Faktörleri: Tanımlayıcı Araştırma

Objective: Juvenile idiopathic arthritis (JIA) is the most common chronic rheumatic disease in childhood. Cardiovascular morbidity and mortality are becoming important health problems for children with inflammatory rheumatic diseases. The aim of this study was to evaluate carotid intima-media thickness (CIMT) in children with JIA and to examine its association with JIA subtype, markers of inflammation, and early atherosclerosis. Material and Methods: We included 112 (50 boys and 62 girls) patients who have been followed by a diagnosis of JIA for at least 6 months and 54 healthy control subjects (32 girls and 22 males) who were matched with the patients age and gender. All children underwent a carotid artery. Inflammation markers were evaluated in the patient group. Cumulative drug doses, total disease duration (TDD), active disease duration (ADD) were calculated. All CIMT values were compared in patients, subgroups, and control groups, and their relationship with inflammation markers was investigated. Results: CIMT values in found that children with JIA were significantly higher than in healthy children. There was no difference between the disease subgroups in terms of CIMT. No relationship was found between CIMT measurements and atherosclerosis risk factors, drugs, erythrocyte sedimentation rate, white blood cell and C-reactive protein. A negative correlation was found between mean platelet volume (MPV) and right CIMT. A statistically significant positive correlation was detected between the right CIMT and TDD (r=0.221, p=0.022), and ADD (r=0.248, p=0.010). Conclusion: The study showed that the patients with JIA had more risks than healthy controls for cardiovascular disease (CVD) regardless of subgroup. We concluded early and aggressive therapies may be protective for CVD. Negative correlation was found between MPV and CIMT that is consistent with recently published literature, but there is need for further studies with a larger patient population.

Investigating Wrist Joint Position Sense and Hand Functions in Children With Polyarticular Juvenile Idiopathic Arthritis With Wrist Involvement.

Comparing the wrist joint position sense and hand functions between children with juvenile idiopathic arthritis (JIA) and healthy controls, and determining possible relationships between these parameters in children with JIA were the aims of this study. Twenty children with polyarticular JIA with wrist involvement (JIAWrist+), 20 children with other subtypes of JIA without wrist involvement (JIAWrist-), and 20 healthy controls were included. Wrist joint position sense was evaluated by measuring joint repositioning error. Hand functions were assessed by using the Purdue Pegboard test, hand grip strength, pinch strength, and Duruoz Hand Index. Joint position sense and hand functions were diminished in the JIAWrist+ group compared with healthy control group (P < .05). Few moderate relationships were detected between hand functions and wrist joint position sense (P < .05). Improving proprioceptive acuity by appropriate training methods may have a role in enhancing hand functions.

Overview on Juvenile Psoriatic Arthritis: A Review

Psoriatic juvenile idiopathic arthritis is a subtype of juvenile idiopathic arthritis that is characterized by both arthritis and psoriasis. juvenile idiopathic arthritis (JIA) is a heterogeneous category of idiopathic inflammatory arthritis that affects children under the age of 16 and lasts six weeks or more as it represents approximately 5% of the whole Juvenile Idiopathic Arthritis (JIA) population. The most prevalent rheumatic illness in children from Western countries is JIA. Chronic arthritis in JIA has an unknown etiology and causes. It is hypothetical if abnormal immune reactions may be brought on by the interplay of environmental elements in a person with a predisposed genetic makeup. Some environmental variables, such exposure to antibiotics and C-section births, provide significant dangers. If there are no characteristic psoriatic lesions present. According to International League of Associations of Rheumatology (ILAR) classification, JPsA is defined by the association of arthritis and psoriasis or, in the absence of typical psoriatic lesions, with at least two of the following: dactylitis, nail pitting, onycholysis or family history of psoriasis in a first-degree relative. In this review we will be looking at the disease etiology, epidemiology, treatment as well as some literature review.

Management of hereditary gingival fibromatosis in juvenile idiopathic arthritis patient - A case report

Hereditary gingival fibromatosis (HGF) is a rare condition with the prevalence of one per 175000 population and equal distribution in sexes. The onset of gingival enlargement is concurrent with the eruption of permanent teeth or in combination of other symptoms as part of a syndrome. Juvenile idiopathic arthritis (JIA) is the most common chronic rheumatic disease of unknown etiology in childhood and predominantly presents with peripheral arthritis. Enthesitis-related arthritis is a separate disease subtype of JIA, characterized by enthesitis and asymmetric lower-extremity arthritis. A case report of 22-year-old male patient with generalized gingival enlargement involving up to coronal one third of crown was observed clinically and diagnosis was made as HGF based on clinical and histopathological examination. After phase I therapy, laser gingivectomy was performed and good wound healing was observed at 3 months post operatively. Key Words: Juvenile idiopathic arthritis, Hereditary gingival fibromatosis, Enthesitis-related arthritis, laser gingivectomy.

Contribution of Ultrasound in Current Practice for Managing Juvenile Idiopathic Arthritis.

The interest and application of musculoskeletal ultrasound (MSUS) in juvenile idiopathic arthritis (JIA) are increasing. Numerous studies have shown that MSUS is more sensitive than clinical examination for detecting subclinical synovitis. MSUS is a well-accepted tool, easily accessible and non-irradiating. Therefore, it is a useful technique throughout JIA management. In the diagnostic work-up, MSUS allows for better characterizing the inflammatory involvement. It helps to define the disease extension, improving the classification of patients into JIA subtypes. Moreover, it is an essential tool for guiding intra-articular and peritendinous procedures. Finally, during the follow-up, in detecting subclinical disease activity, MSUS can be helpful in therapeutic decision-making. Because of several peculiarities related to the growing skeleton, the MSUS standards defined for adults do not apply to children. During the last decade, many teams have made large efforts to define normal and pathological US features in children in different age groups, which should be considered during the US examination. This review describes the specificities of MSUS in children, its applications in clinical practice, and its integration into the new JIA treat-to-target therapeutic approach.

Optical coherence tomography angiography (OCTA) findings in juvenile idiopathic arthritis

Aim of the workTo report optical coherence tomography angiography (OCTA) findings in juvenile idiopathic arthritis (JIA) patients and to study the relation to disease activity. Patients and methodsThe study included 20 JIA patients (38 eyes) who underwent ophthalmologic and rheumatologic examination plus OCT/OCTA. Juvenile arthritis disease activity score (JADAS27) was assessed, and patients were divided into those with no/low activity (group 1; n = 13) and moderate/severe activity (group 2; n = 7). OCTA findings were compared with 11 control (11 eyes). ResultsThe study included 20 JIA-U patients (38 eyes) with a mean age of 10.7 ± 2.6 years and disease duration of 72.5 ± 34.7 months and they were 9/20 (45 %) females. 13(65 %) patients had no/mild activity (group 1, 25 eyes) while 7(35 %) had moderate to severe activity (group 2, 13 eyes). The mean foveal superficial and deep capillary plexuses (SCP/DCP) vascular density (VD) were significantly lower in patients with moderate/severe activity (3x3 scan: p = 0.001 and p < 0.001; 6x6 scan p = 0.008 and p = 0.001 respectively). The foveal avascular zone (FAZ) 3x3 and 6x6 scans were significantly increased and the central macular thickness (CMT) decreased in patients with moderate/severe disease activity (p < 0.001, p = 0.001, and p = 0.001). Fovea SCP VD in 6x6 and 3x3 scans were significantly different between JIA subtypes (p = 0.01 and p = 0.03, respectively), with less VD in oligoarticular type. FAZ, CMT and DCP-VD significantly correlated with visual analogue scale (r = 0.58, p < 0.001; r = −0.5, p = 0.01; r = −0.58, p < 0.001, respectively). ConclusionsNon-invasive OCTA-derived vascular parameters of the macula could be potential biomarkers for evaluating the severity of the systemic disease activity in JIA patients.

A Nordic screening guideline for juvenile idiopathic arthritis-related uveitis.

The purpose of this perspective was to shed light on screening of uveitis among Nordic children with juvenile idiopathic arthritis (JIA). A literature search was conducted to review predictors of JIA-uveitis and previous JIA-uveitis screening recommendations. Predictors of uveitis in JIA are younger age and positive antinuclear antibody titre at onset of JIA, specific subtypes of JIA (extended and persistent oligoarthritis, rheumatoid factor negative polyarthritis and psoriatic arthritis) and short duration of JIA. Methotrexate and monoclonal tumour necrosis factor (TNF) inhibitor treatment reduce the risk JIA-uveitis. Children with all of the above risk factors should be screened frequently but if they receive TNF inhibitor or methotrexate therapy, they may be screened less frequently. Children with none of the risk factors do not benefit from long-term screening for uveitis. A guideline for intervals and overall length of screening was prepared considering currently known risk factors for JIA-uveitis, the Nordic population and previous guidelines.

Емоційна складова якості життя пацієнтів з ювенільним ідіопатичним артритом

For children with juvenile idiopathic arthritis (JIA), the quality of life (QoL) emotional component is influenced by numerous factors: the arthritis subtype, disease duration, and activity, patient’s treatment, patient’s physical condition, and pain level, also his age, and sex. However, it’s important to determine the emotional component in the structure of QoL in children with different JIA subtypes (polyarthritis, oligoarthritis, and uveitis-associated arthritis) and features of psychological adaptation in modern conditions. Purpose - to determine both the emotional component of QoL in patients with JIA and their emotional condition considering the disease subtype, its duration, and the therapy complex. Materials and methods. The study was carried out at the SI «Institute for Children and Adolescents Health Care of the NAMS of Ukraine», Kharkiv, from November 2020 to November 2021. The assessment was provided by JADAS27, functional state by CHAQ, QoL according to PedsQLTM 4.0 Generic Core Scales, and emotional state according to the Montgomery-Asberg scale (MADRS) for depression assessment and clinical indicators. Statistical processing of the material was carried out using parametric and non-parametric methods, the critical level of significance for statistical testing hypotheses comparing groups was set at 0.05. Results. The 118 patients with JIA were examined, of them 47 patients with polyarticular, 43 patients with oligoarticular, and 28 patients with JIA-u subtypes. The study involved 77 girls and 41 boys aged 2 to 18 years (133.3±4.6 months). The majority (112 patients) received methotrexate therapy, 30 of them - methotrexate in combination with immunobiological therapy, 6 - sulfasalazine. The overall QoL in the group of children with JIA was at the level of 70.9±1.4 points for the last month and 72.6±1.4 points for the last week without significant difference, and the emotional component in the group as a whole was significantly higher for the last month - 73.1±1.6 points, while during the hospital stay it decreased to 66.3±1.7 points (p≤0.05). According to the results of the assessment of emotional state by the MADRS questionnaire, children aged 8 years and older did not have a depressive episode - 6.02±0.72 points. No significant differences were observed for HRQoL and emotional component in groups with different variants of arthritis and depending on age. However, certain results with a decrease in QoL and emotional state were observed in children with polyarthritis and uveitis-associated variant of JIA. The duration of the disease and treatment affected the QoL and emotional state, especially in the first year (QoL = 62.5±3.8 points for the last month). Correlations between MADRS and emotional state according to PedsQL were indirect and strong both for the last week and for the last month (r=-0.821; r=-0.784; p≤0.05). MADRS and total PedsQL scores also had indirect, medium strength correlations for the study periods (r=-0.558; r=-0.528; p≤0.05). Conclusions. At the beginning of the disease children with JIA have the reduction of QoL indicators with the emotional component. Such results depend on the course of JIA, which predicts the formation of emotional depressive disorders in some of them. The first year of the disease is the most vulnerable for children with polyarthritis, and for children with JIA-u increased disease duration enhances symptoms of emotional disturbances. The research was carried out in accordance with the principles of the Helsinki Declaration. The study protocol was approved by the Local Ethics Committee of the participating institution. The informed consent of the patient was obtained for conducting the studies. No conflict of interests was declared by the authors.

The Role of Interleukin-17 in Juvenile Idiopathic Arthritis: From Pathogenesis to Treatment.

Background and Objectives: Interleukin-17 (IL-17) is a cytokine family consisting of six members and five specific receptors. IL-17A was the first member to be identified in 1993. Since then, several studies have elucidated that IL-17 has predominantly pro-inflammatory activity and that its production is involved in both the defense against pathogens and the genesis of autoimmune processes. Materials and Methods: In this review, we provide an overview of the role of interleukin-17 in the pathogenesis of juvenile idiopathic arthritis (JIA) and its relationship with IL-23, the so-called IL-23-IL-17 axis, by reporting updated findings from the scientific literature. Results: Strong evidence supports the role of interleukin-17A in the pathogenesis of JIA after the deregulated production of this interleukin by both T helper 17 (Th17) cells and cells of innate immunity. The blocking of IL-17A was found to improve the course of JIA, leading to the approval of the use of the human anti-IL17A monoclonal antibody secukinumab in the treatment of the JIA subtypes juvenile psoriatic arthritis (JPsA) and enthesitis-related arthritis (ERA). Conclusions: IL-17A plays a central role in the pathogenesis of JIA. Blocking its production with specific biologic drugs enables the effective treatment of this disabling childhood rheumatic disease.

24 Scoliosis in children with juvenile idiopathic arthritis: any link?

BackgroundJuvenile idiopathic arthritis (JIA) is an unusual aetiology of scoliosis in childhood. With few data in literature, the associated factors of scoliosis in JIA are still unknown.ObjectivesTo describe the incidence and associated factors of scoliosis in children with JIA.MethodsA cross-sectional study including children with JIA according to the International League of Associations for Rheumatology (ILAR). The recorded data included anthropometric measurements (weight, size, body mass index (BMI), and leg length), disease characteristics (JIA subtypes and duration), and static foot posture disorders. Regarding scoliosis, we collected clinical and radiographic findings.ResultsThirty-five patients, forty-three percent of the patients were boys (n = 15). The mean age was 12.2 ± 3.61 years. The mean disease duration was 4.1 ± 3.29 years. The mean patient global assessment and the mean visual analogic scale were 3.4 ± 3.02 and 3.37 ± 2.92, respectively. The patient's distribution of JIA subtypes was oligoarticular (n = 13), enthesitis-related arthritis (n = 9), polyarticular (n = 4), undifferentiated (n = 4), psoriasis-related JIA (n = 4) and systemic-onset (n = 1). The mean CRP and ESR were 7.51 ± 11.85 mg/l and 18.88 ± 15.53 mm, respectively. The mean JADAS was 7.58 ± 6.3. Seventeen percent of the patients had an inactive disease (n = 6). Seven patients (20%) (6 females) had developed scoliosis during their disease, the spine regions of scoliosis were thoracolumbar scoliosis (n = 6) and thoracic scoliosis (n = 1). There was no specific distribution of scoliosis among JIA subtypes (p = 0.464). Most patients with scoliosis had associated leg length discrepancy (71.43% vs 28.57%, p = 0.025). Static foot posture disorders were present in most patients with scoliosis (85.71% vs 14.29%) but this difference was not significant (p = 0.673) compared with patients with no scoliosis. Weight, size and BMI were not associated with scoliosis (p = 0.955, p = 0.922, p = 0.303 respectively).ConclusionOur study shows that scoliosis is associated with leg length discrepancy. Indeed, the joint inflammation of the lower limb may explain the postural syndrome in children with JIA, with a prompt impact on the axial skeleton.

07 Predictor factors of hip involvement in juvenile idiopathic arthritis

BackgroundJuvenile idiopathic arthritis (JIA) is one of the commonest rheumatic diseases in children. Hip involvement is a common problem in JIA patients and is associated with functional disability and poor outcomes. Intensive therapy is required to avoid joint replacement surgery. Little studied in the literature, the predictors of hip involvement are still unknown.ObjectivesOur study aims to identify the clinical, biological characteristic of patients with hip involvement and determine the associated risk factors.MethodsA cross-sectional study including children with JIA according to the International League of Associations for Rheumatology (ILAR). The recorded data included sociodemographic features, disease characteristics (subtype disease, duration, and juvenile arthritis disease activity score (JADAS)) as well as treatment modalities. Regarding coxitis, we collected radiographs, ultrasound (US), and magnetic resonance imaging (MRI) of the hip when performed. Coxitis was defined by clinical (limited range of motion) and/or radiographic findings (destruction, synovitis, bone marrow oedema).ResultsThirty-five patients (20 females) with a median age of 12 years (5–18) and disease duration of 3 years (0.25–15) were recruited. The patient's distribution of JIA subtypes were oligoarticular (n = 13), enthesitis-related arthritis (n = 9), polyarticular (n = 4) (negative rheumatoid factor in 3 patients), undifferentiated (n = 4), psoriatic-arthritis (n = 4) and systemic-onset (n = 1). ESR and CRP median values were 15 mm/h (0–63) and 2 mg/l(0–47) respectively. Sixteen patients were under DMARDs (Methotrexate (n = 10), biological agent (n = 3), biological agent and methotrexate (n = 2), salazopyrine (n = 1)).Sixteen patients (45.71%) developed coxitis (radiographic (n = 8), MRI (n = 5) and US findings (n = 3)) with eight (50%) presenting limited range of motion and 10 (62.5%) developing radiological evidence of hip damage. Hip involvement was associated with a longer disease duration (p = 0.051). JADAS score value of patients with coxitis was higher (Mean 8.35 vs 7.05) but not significantly (p = 0.565). Higher CRP and ESR values were found in patients with coxitis (mean 8.39 mg/l vs 6.83 mg/l, 22.29 mm/h vs 16.37 mm/h respectively) but not significantly (p = 0.718, p = 0.287 respectively). No associations were found between hip involvement and BMI (p = 0.233), age-onset (p = 0.496), JIA subtype (p = 0.509), nor sex (p = 0.767).ConclusionOur study shows that long disease duration exposes to a higher risk of hip involvement in children with JIA. Active disease and biological inflammatory syndrome could be associated risk factors. Studies with larger sample sizes are needed to draw definite causal associations.

37 Subtype frequencies and demographic characteristics of juvenile idiopathic arthritis in Batna, Algeria

BackgroundJuvenile idiopathic arthritis (JIA) is the most common chronic rheumatic disease in childhood. There is a disparity in the prevalence of Juvenile idiopathic arthritis (JIA) subsets between different geographical areas or ethnic groups. In Arabic and African populations, data describing JIA are scarce. However, the epidemiological studies remain the best tool to understand the disease and to improve its management.ObjectivesTo determine subtype, frequency, demographic and clinical features of JIA in Batna -Algeria- and to compare the findings with other JIA populations worldwide.MethodsA multicentre retrospective descriptive study was conducted in Batna health centers (public and private sectors), over a seven-year period from January 2013 to December 2019, based on data collected on JIA patients. As public sector source, we referred to the department of pediatrics of the university hospital center (CHU Benflis Touhami Batna), and as private sector source, we referred to private adult rheumatologists based in Batna. The studied variables were: gender, age at the initial symptoms, age at diagnosis, JIA subtype based on International League of Associations for Rheumatology (ILAR) criteria, symptoms at onset, disease duration at the latest follow up, presence of uveitis, auto antibodies (antinuclear antibodies, Rheumatoid Factor and anti-CCP) pattern, joint imaging results, JIA status at the time of enrolment and the latest follow-up.ResultsThe study included a total of 69 cases of JIA that were being followed in Batna health centers over the study period. The female to male ratio was 1.83. The median age at diagnosis was 9 years (range 1–16). Forty-six patients (72%) were diagnosed within the first year after disease onset. At the latest follow-up, the median disease duration onset was 1 year (range 1–8 years). There were 34 oligoarthritis (49.3%), 9(13%) rheumatoid factor (RF) negative polyarticular JIA, 8(11.6%) RF positive polyarticular JIA, 6(8.7%), systemic arthritis, 6(8.7%) enthesitis-related arthritis, 3(4.3%) psoriatic arthritis and 3(4.3%) undifferentiated arthritis. Nine patients (18.7%) were anti-nuclear antibody (ANA) positive, and 21 patients (30.4%) had indeterminate ANA status.ConclusionOligoarthritis was the most common JIA subtype in our study. The RF positive polyarthritis frequency was higher than in literature. Prospective multicentre studies are necessary to better identify the JIA peculiarities in our country.Disclosure of InterestNone declared

25 Impact of early feeding on the occurrence of juvenile idiopathic arthritis

BackgroundJuvenile idiopathic arthritis (JIA) is considered to be an autoimmune disease, which is a result of an immune reaction caused or triggered by environmental factors in a genetically susceptible individual. However, interactions between early feeding and risk Juvenile idiopathic arthritis (JIA) remains largely unexplored.ObjectivesTo determine the impact of early alimentation on the occurrence of JIA in Tunisia.MethodsWe conducted a case-control study including 26 children with JIA fulfilling the international league against rheumatism (ILAR) 2010 criteria compared with a homogeneous control group of 21 children hospitalized in a paediatric orthopedic unit for traumatic reasons and healthy for any chronic inflammatory rheumatism. Parents of patients were asked after having their consent to participate. We collected the following data: Type of JIA, juvenile disease activity score (JADAS), type of feeding, total duration of breastfeeding, duration of exclusive breastfeeding and age at introduction of gluten. Patients were divided into two groups: group 1 (G1): JIA patients, group 2 (G2): control group. Data were analyzed using the SPSS statistical package. A p-value < 0.05 was considered significant.ResultsForty-seven children (26 diagnosed with JIA and 21 controls) with a mean age of 10.07 years ± 4.96 were enrolled. JIA subtypes were in decreasing order of frequency: oligoarticular (45.8%), enthesitis-related arthritis (20.8%), undifferentiated JIA (12.5%), seronegative polyarticular JIA (8.3%), juvenile psoriatic arthritis (8.3%), and seropositive polyarticular JIA (4.2%). The mean JADAS was 6.966 ± 5.38. There was no significant difference between the G1 and G2 with regard to the duration of breastfeeding (12.23 ± 3.8 months vs 8.14 ± 4.1 months, p = 0.09), exclusive breastfeeding (3.8 ± 3.6 vs 4.1 ± 2.62, p = 0.40) and age at introduction of gluten (G1: 8.48 ± 5.30 months vs G2: 7.48 ± 1,2 months, p = 0.35)ConclusionOur study didn’t show any impact of early alimentation on the occurrence of JIA in Tunisia. However, more studies are necessary to conclude these issues.

34 Subtype frequencies and demographic characteristics of juvenile idiopathic arthritis in Batna, Algeria

BackgroundJuvenile idiopathic arthritis (JIA) is the most common chronic rheumatic disease in childhood. There is a disparity in the prevalence of Juvenile idiopathic arthritis (JIA) subsets between different geographical areas or ethnic groups. In Arabic and African populations, data describing JIA are scarce. However, the epidemiological studies remain the best tool to understand the disease and to improve its management.ObjectivesTo determine subtype, frequency, demographic and clinical features of JIA in Batna -Algeria- and to compare the findings with other JIA populations worldwide.MethodsA multicentre retrospective descriptive study was conducted in Batna health centers (public and private sectors), over a seven-year period from January 2013 to December 2019, based on data collected on JIA patients. As public sector source, we referred to the department of pediatrics of the university hospital center (CHU Benflis Touhami Batna), and as private sector source, we referred to private adult rheumatologists based in Batna. The studied variables were: gender, age at the initial symptoms, age at diagnosis, JIA subtype based on International League of Associations for Rheumatology (ILAR) criteria, symptoms at onset, disease duration at the latest follow up, presence of uveitis, auto antibodies (antinuclear antibodies, Rheumatoid Factor and anti-CCP) pattern, joint imaging results, JIA status at the time of enrolment and the latest follow-up.ResultsThe study included a total of 69 cases of JIA that were being followed in Batna health centers over the study period. The female to male ratio was 1.83. The median age at diagnosis was 9 years (range 1–16). Forty-six patients (72%) were diagnosed within the first year after disease onset. At the latest follow-up, the median disease duration onset was 1 year (range 1–8 years). There were 34 oligoarthritis (49.3%), 9(13%) rheumatoid factor (RF) negative polyarticular JIA, 8(11.6%) RF positive polyarticular JIA, 6(8.7%), systemic arthritis, 6(8.7%) enthesitis-related arthritis, 3(4.3%) psoriatic arthritis and 3(4.3%) undifferentiated arthritis. Nine patients (18.7%) were anti-nuclear antibody (ANA) positive, and 21 patients (30.4%) had indeterminate ANA status.ConclusionOligoarthritis was the most common JIA subtype in our study. The RF positive polyarthritis frequency was higher than in literature. Prospective multicentre studies are necessary to better identify the JIA peculiarities in our country.Disclosure of InterestNone declared

36 Juvenile idiopathic arthritis-associated uveitis: a study of 69 cases

BackgroundJuvenile Idiopathic Arthritis (JIA) is the most common rheumatic disease association with uveitis in children. Uveitis is a significant cause of visual morbidity in children with JIA. Geographical variations in the incidence of uveitis in JIA have been reported around the world.ObjectivesThe aim of this study was to determine the prevalence of JIA associated uveitis and its features in Batna -Algeria- and to compare the findings with other JIA populations worldwide.MethodsA multicentre retrospective descriptive study was conducted in Batna health centers (public and private sectors), over a seven-year period from January 2013 to December 2019, based on data collected on JIA patients. As public sector source, we referred to the department of pediatrics of the university hospital center (CHU Benflis Touhami Batna), and as private sector source, we referred to private adult rheumatologists based in Batna. The studied variables were: gender, age at the initial symptoms, age at diagnosis, JIA subtype based on International League of Associations for Rheumatology (ILAR) criteria, symptoms at onset, disease duration at the latest follow up, presence of uveitis, auto antibodies (antinuclear antibodies, Rheumatoid Factor and anti-CCP) pattern, joint imaging results, JIA medications, JIA status at the time of enrolment and the latest follow-up.ResultsSixty-nine cases of JIA were included. The female to male ratio was 1.8 :1. The median age at diagnosis was 9 years (range 1–16). At the latest follow-up, the median disease duration onset was 1 year (range 1–8 years). There were 34(49.3%) oligoarthritis, 9(13%) rheumatoid factor (RF) negative polyarticular arthritis, 8(11.6%) rheumatoid factor (RF) positive polyarticular arthritis, 6(8.7%) systemic onset JIA, 6(8.7%) enthesitis-related arthritis, 3 (4.3%) psoriatic arthritis, and 3(4.3%) undifferentiated arthritis. Nine patients (18.7%) were anti-nuclear antibody (ANA) positive, and 21 patients (30.4%) had indeterminate ANA status. Sixty-three patients (91.3%) have benefited from the slit lamp examination. Uveitis was found in 5 patients (7.2%). three of these patients presented with oligoarticular JIA and two presented Polyarticular JIA. There was male predominance (4 boys and 1 girl). All these patients were ANA negative.ConclusionThe observed phenotypic variabilities in JIA associated uveitis underscore the existence of true diversities in disease characteristics across geographical areas. Prospective multicentre studies are necessary to better identify the JIA associated uveitis peculiarities in our country.Disclosure of InterestNone declared

Increased Relapse Rate During COVID-19 Lockdown in an Italian Cohort of Children With Juvenile Idiopathic Arthritis.

ObjectiveChanges of routine disease management associated with COVID‐19 lockdown might have potentially affected the clinical course of juvenile idiopathic arthritis (JIA). The aim of our study was to assess the rate of disease flare before and during COVID‐19 lockdown to investigate its impact on disease course in children with JIA.MethodsA single‐center retrospective study was conducted, including patients presenting with inactive JIA between September 1, 2018 and March 9, 2019 (group A) and between September 1, 2019 and March 9, 2020 (group B). For each patient, demographic and clinical data were collected. The rate of JIA flare from March 10, 2019 to June 30, 2019 for group A and from March 10, 2020 to June 30, 2020 for group B was compared.ResultsGroup A included 126 patients, and group B 124 patients. Statistical analysis did not show significant differences among the 2 cohorts with respect to age, sex, age at JIA onset, JIA subtype, co‐occurrence of uveitis, antinuclear antibody positivity, and past or ongoing medications. The rate of disease flare during lockdown at the time of the first COVID‐19 pandemic wave was significantly higher in comparison to the previous year (16.9% versus 6.3%; P = 0.009).ConclusionOur study showed that COVID‐19 lockdown was associated with a higher rate of joint inflammation in children with JIA. This finding has a considerable clinical implication, as restrictive measures may be necessary in order to contain pandemics. Our data highlight the need for rearrangement in the home and health care management of children with JIA during lockdowns.

23 Predictive factors of hip involvement in juvenile idiopathic arthritis

Abstract Background Juvenile idiopathic arthritis (JIA) is the most common rheumatic disease in children. Hip involvement is a common problem in JIA patients and is associated with functional disability and poor outcomes. Intensive therapy is required to avoid replacement surgery. With few studies in the literature, the predictive factors of hip involvement are still unknown. Objectives To identify the clinical, biological characteristic of patients with hip involvement and determine the risk factors associated. Methods A cross-sectional study including children with JIA according to the International League of Associations for Rheumatology (ILAR). The recorded data included sociodemographic features, disease characteristics (subtype disease, duration, and juvenile arthritis disease activity score (JADAS) as well as treatment modalities. Regarding coxitis, we collected radiographs, ultrasound (US), and magnetic resonance imaging (MRI) of the hip when performed. Coxitis was defined by clinical (limited range of motion) and/or radiographic findings (destruction, synovitis, bone marrow oedema). Results Thirty-five patients (20 females) with a median age of 12 years (5–18) and disease duration 3 years (0.25–15) were recruited. The patient's distribution of JIA subtypes were oligoarticular (n = 13), enthesitis-related arthritis (n = 9), polyarticular (n = 4) (negative rheumatoid factor in 3 patients), undifferentiated (n = 4), psoriasis-arthritis (n = 4) and systemic-onset (n = 1). ESR and CRP median values were 15 mm/h (0–63) and 2 mg/l (0–47); respectively. DMARDS was admitted to 16 patients Sixteen patients [Methotrexate (n = 10), biological agent (n = 3), biological agent and methotrexate (n = 2), salazopyrine (n = 1)]. Sixteen patients (45.71%) developed coxitis (radiographic (n = 8), MRI (n = 5) and US findings (n = 3)) with limited range of movement in eight (50%) and radiological evidence of hip damage in 10 (62.5%). Hip involvement was associated with a longer disease duration (p = 0.051). JADAS of patients with coxitis was higher (Mean 8.35 vs 7.05) but not significant (p = 0.565). Higher CRP and ESR values were found in patients with coxitis (mean 8.39 mg/l vs 6.83 mg/l, 22.29 mm/h vs 16.37 mm/h respectively) but not significant (p = 0.718, p = 0.287 respectively). No associations were found between hip involvement and BMI (p = 0.233), age-onset (p = 0.496), JIA subtype (p = 0.509) and sex (p = 0.767). Conclusion Our study shows that long disease duration exposes to a higher risk of hip involvement in children with JIA. Active disease and biological inflammatory syndrome could be associated risk factors. Studies with larger sample size are needed to draw definite causal associations.

P48 Service evaluation of disease burden of, and damage caused by, wrist arthritis in a single centre paediatric cohort of JIA

Introduction/BackgroundWrist synovitis is associated with more severe disease and less drug-free remission periods, therefore, the objective of this study was to investigate the demographic, disease course, and complexity of wrist disease in children being treated for Juvenile Idiopathic Arthritis (JIA).Description/MethodA single-centre, retrospective case note review of all children and adolescents diagnosed with JIA, who received steroid joint injections of their wrists from 2014 to 2019. All patients received standard therapy and underwent regular follow-up at Birmingham Children’s Hospital. Inclusion criteria were those with an established diagnosis of any JIA-subtype according to the International League of Associations for Rheumatology (ILAR) classification. Patients were excluded if (i) the data were incomplete, (ii) they were over the age of 16y at diagnosis, or (iii) the eventual diagnosis was not JIA. Clinical information was gathered from case notes, departmental databases and hospital clinical systems on to a standardised proforma. Information collected were demographics, JIA subtype, immunology, age at diagnosis, disease duration, number of wrist injections and outcome of every wrist MRI performed. All treatments including intraarticular injections were administered by a paediatric Rheumatologist while MRIs were reviewed by an experienced senior Radiologist.Discussion/ResultsOf all 94 eligible patients, 88 (94%) were included in this study; 71 girls, with a mean age at diagnosis of 6 years (0-15 years). Wrist arthritis was more common in seronegative polyarthritis (40%) and extended oligoarthritis (23%) but least pronounced in Enthesitis-related arthritis (2%). 85 (95.6%) received Methotrexate therapy with a mean of 10 months from time of diagnosis. 77 patients (88%) required a step-up from MTX, with Etanercept (88%) being the most popular option as a first-line biologics, followed by Adalimumab (11%). All patients who had Adalimumab as a first biologics had evidence of uveitis.Wrist disease was present at diagnosis in 60/88 (68%) of our cohort. 66 patients (75%) had bilateral wrist disease. Over one-quarter of patients who developed wrist arthritis within the first year of diagnosis were young children (0-2 years). A second predominate group was adolescent (10-15 years) accounting for 33% of our cohort. The mean (IQR) duration between diagnosis and first clinical evidence of wrist synovitis was 15 months (1–17). Patients had a median of 3 years from diagnosis until development of first radiological evidence of erosive changes.12 children (14%) from this cohort underwent a laparoscopic synovectomy for their wrist damage between 2017 and 2021, out of which 9 had polyarticular involvement while two had extended and one had persistent oligoarticular arthritis. Half of the patients reported improvement in pain and Health Assessment Questionnaire (HAQ) scores after synovectomy, while three remained stable at 0 score, and 3 had insufficient data.Key learning points/ConclusionTreatment of wrist arthritis can be challenging to both professionals and health care systems, emphasizing the need for clear steps to improve early recognition and treatment. Wrist disease at presentation was relatively common in our cohort and damage once occurred was irreversible with patients continuing to have ongoing disease activity despite optimization of their treatment. Furthermore, MRI as shown in our analysis, can accurately detect early erosive changes and inform management decision.

Association of HLA-B27 antigen with clinical and laboratory parameters in patients with juvenile idiopathic arthritis.

Aim To analyse the association of human leukocyte antigen B27 with clinical and laboratory parameters in patients with juvenile idiopathic arthritis (JIA) at the disease onset. Methods A retrospective review of medical records of 25 HLAB27 positive and 25 HLA-B27 negative JIA patients was performed. The diagnosis of JIA was based on the 1997-2001 International League Against Rheumatism (ILAR) criteria. Collected data: age, sex, HLA- B27 antigen presence, C-reactive protein (CRP), erythrocyte sedimentation rate (ESR), rheumatoid-factor (RF), antinuclear antibody (ANA), fever, rash, uveitis, enthesitis, inflamed joints and subtype of JIA. Results HLA- B27 positive study group had more boys (p=0.01), higher erythrocyte sedimentation rate (p=0.038), higher presence of fever (p= 0.025) and enthesitis (p=0.024). Any significant difference in age of the disease onset, CRP, ANA, RF, rash, uveitis, inflamed joint and dactylitis was not noticed. The most common subtype of JIA in the HLA-B27 positive patients was ERA (60%). Conclusion This study showed that the presence of HLA- B27 antigen plays a significant role in determining the presenting clinical and laboratory characteristics in JIA patients.