Juvenile Idiopathic Arthritis Cases Research Articles

Seronegative Polyarticular Juvenile Idiopathic Arthritis: A Case Report.

Juvenile idiopathic arthritis (JIA) is a chronic rheumatologic childhood disorder of unknown etiology usually presenting with peripheral arthritis. Usually involving the small joints symmetrically, polyarticular JIA is characterized by involvement of 5 or more peripheral joints and is further categorized depending on RA Factor positivity. We aim to report a case of seronegative polyarticular JIA to increase awareness regarding this disease. A 15-year-old boy presented with 3-month history of bilateral symmetrical joint pains, swelling and morning stiffness lasting 3-4 hours. For his ankle and knee pain, he had consulted a local doctor who suspected Rheumatic Fever and started penicillin and analgesics resulting in minimal improvement. He was a student but was unable to attend school in last 2 months due to this illness and had become bed-bound for last 2 weeks. On examination, there was swelling and tenderness of ankles, knees, elbows, wrists, metacarpophalangeal and proximal interphalangeal joints bilaterally. On investigation, ESR, CRP and serum ferritin were raised with normal complete blood count, urinalysis, liver and renal function tests, ASO test, blood and urine cultures, TSH, serum CPK and serum aldolase. Echocardiography did not reveal any abnormalities. His ANA, RA factor and Anti-CCP antibodies were negative. He was diagnosed with Seronegative Polyarticular Juvenile Idiopathic Arthritis and started on oral prednisolone and methotrexate. Currently the patient is asymptomatic on methotrexate 20mg/week. Our patient with symmetrical polyarthritis had negative ANA, RA Factor and Anti-CCP antibodies leading to a diagnosis of seronegative polyarticular JIA. Early screening and prompt management of JIA is required to improve prognosis and reduce risk of disability. The differential diagnosis of childhood joint pains is wide and variable including diseases of autoimmune, cardiac and haematological systems. For diagnosis of JIA, arthritis persisting for more than 6 weeks with onset of symptoms prior to 16 years of age is required with exclusion of secondary causes of joint inflammation. Keywords: Juvenile Idiopathic Arthritis, ANA, RA Factor, Anti-CCP antibodies, Methotrexate.

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.

Revealing Novel Genomic Insights and Therapeutic Targets for Juvenile Idiopathic Arthritis through Omics.

The genetic architecture of juvenile idiopathic arthritis (JIA) remains only partially comprehended. There is a clear imperative for continued endeavors to uncover insights into the underlying causes of JIA. This study encompassed a comprehensive spectrum of endeavors, including conducting a JIA GWAS meta-analysis that incorporated data from 4,550 JIA cases and 18 446 controls. We employed in silico and genome-editing approaches to prioritize target genes. To investigate pleiotropic effects, we conducted phenome-wide association studies. Cell-type enrichment analyses were performed by integrating bulk and single-cell sequencing data. Finally, we delved into potential druggable targets for JIA. Fourteen genome-wide significant non-HLA loci were identified including four novel loci, each exhibiting pleiotropic associations with other autoimmune diseases or musculoskeletal traits. We uncovered strong genetic correlation between JIA and bone mineral density (BMD) traits at 52 genomic regions, including three GWAS loci for JIA. Candidate genes with immune functions were captured by in silico analyses at each novel locus, with additional findings identified through our experimental approach. Cell-type enrichment analysis revealed 21 specific immune cell types crucial for affected organs in JIA, indicating their potential contribution to the disease. Finally, 24 known or candidate druggable target genes were prioritized. Our identification of four novel JIA associated genes, CD247, RHOH, COLEC10 and IRF8, broadens novel potential drug repositioning opportunities. We established a new genetic link between COLEC10, TNFRSF11B and JIA/BMD. Additionally, the identification of RHOH underscores its role in positive thymocyte selection, thereby illuminating a critical facet of JIA's underlying biological mechanisms.

Serum Immunoglobulin Concentrations in Juvenile Idiopathic Arthritis Cases during Active and Inactive Disease States

Serum Immunoglobulin Concentrations in Juvenile Idiopathic Arthritis Cases during Active and Inactive Disease States

The incidence and prevalence of Juvenile Idiopathic Arthritis differs between ethnic groups in England.

Juvenile Idiopathic Arthritis (JIA) is a heterogenous group of rare autoimmune disorders characterised by chronic joint inflammation of unknown aetiology with onset under 16years. Accurate estimates of disease rates help understand impacts on individuals and society, and provide evidence for health service planning and delivery. This study aimed to produce the first national estimates of incidence and prevalence by ethnic group using electronic health records. Data from the Clinical Practice Research Datalink (CPRD) Aurum, a primary care electronic health record database in England, were used to estimate the incidence and prevalence of JIA by ethnic group amongst children and young people aged under 16 years between 2003 and 2018, with cases validated using Hospital Episode Statistics (HES). Chi square was used to test the difference in proportions compared to the ethnic distribution of England. A total of 424 incident cases of JIA were identified, 389 validated using HES records. Incidence of JIA was higher amongst those of White ethnic group (6.2 per 100,000 population) compared to Mixed (3.0 per 100,000), Asian (2.7 per 100,000) and Black (2.9 per 100,000) communities. The ethnic group distribution of cases differed significantly compared to the general population (p < 0.0001). Incidence and prevalence of JIA differs between ethnic groups, and is different from the population. This is likely to be due to a combination of genetic and equity factors. Further research to understand the underlying cause of these differences is important, to enable targeted interventions and appropriate service provision.

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.

Prevalence of tissue transglutaminase antibodies and IgA deficiency are not increased in juvenile idiopathic arthritis: a case-control study

BackgroundThe prevalence of Celiac Disease (CD) in Juvenile Idiopathic Arthritis (JIA) has been reported to be 0.1–7% in various small studies. As a result of the limited number of research and their inconclusive results there are no clear recommendations for routine CD screening in asymptomatic patients with JIA. Our aim is to estimate the prevalence of IgA deficiency and tissue transglutaminase (tTG) IgA in a cohort of JIA followed in two large academic medical centers.MethodsSerum was collected and stored from all subjects and analyzed in a reference laboratory for total IgA (Quantitative Nephelometry) and tTG IgA antibody levels (Semi-Quantitative Enzyme-Linked Immunosorbent Assay). Fisher’s exact tests were performed for statistical significance. Risk estimates (odds ratios) with 95% confidence intervals were calculated.Results808 JIA cases and 140 controls were analyzed. Majority were non-Hispanic whites (72% vs. 68% p = 0.309). A total of 1.2% of cases were IgA deficient compared to none of the controls (p = 0.373). After excluding IgA deficient subjects, 2% of cases had tTG IgA ≥ 4u/mL compared to 3.6% of controls (p = 0.216) (OR = 0.5; 95% C.I = 0.1–1.4); and 0.8% of cases had tTG IgA > 10u/mL compared to 1.4% of controls (p = 0.627) (OR = 0.5; 95%C.I = 0.1–2.9).ConclusionsUsing the largest JIA cohort to date to investigate prevalence of celiac antibodies, the prevalence of positive tTG IgA was 0.8% and of IgA deficiency was 1.2%. The results did not demonstrate a higher prevalence of abnormal tTG IgA in JIA. The study did not support the routine screening of asymptomatic JIA patients for CD.

Advances in the Diagnosis and Treatment of Enthesitis-Related Arthritis.

Enthesitis-related arthritis (ERA) represents 5-30% of all cases of juvenile idiopathic arthritis (JIA) and belongs to the spectrum of the disorders included in the group of juvenile spondyloarthritis. In the last decade, there have been considerable advances in the classification, diagnosis, monitoring, and treatment of ERA. New provisional criteria for ERA have been recently proposed by the Paediatric Rheumatology INternational Trials Organisation, as part of a wider revision of the International League of Associations for Rheumatology criteria for JIA. The increased use of magnetic resonance imaging has shown that a high proportion of patients with ERA present a subclinical axial disease. Diverse instruments can be used to assess the disease activity of ERA. The therapeutic recommendations for ERA are comparable to those applied to other non-systemic JIA categories, unless axial disease and/or enthesitis are present. In such cases, the early use of a TNF-alpha inhibitor is recommended. Novel treatment agents are promising, including IL-17/IL-23 or JAK/STAT pathways blockers.

Rehabilitation in Juvenile Idiopathic Arthritis with Hip Ankylosis

Introduction: Juvenile idiopathic arthritis (JIA) is the most common type of arthritis in children and teens. The inflammation of various joints causes pain. Early diagnosis and management of the patients, which include medication and rehabilitation therapies, can reduce the onset of complications. Despite the progress in managing this disease, there are cases which present severe complications. Material and Methods: A 13-year-old girl, diagnosed with JIA in a paediatric service in 2019, who is under treatment with Sulfasalzine since diagnosis, presented in the Paediatric Rehabilitation Centre of Băile 1 Mai with inflammatory pain in both knees, gait impairment with need of wheelchair, limited function in hands, elbows, shoulders, knees and hips which worsened in 2022, due to low compliance to the treatment and no check-up with a paediatric rheumatologist. She had 8 admissions in the Paediatric Rehabilitation Centre between October 2022 and July 2023. The examination reveals that there is a severe limitation of mobility in both hips and knees, the hands’ small joints, elbows and shoulders. Laboratory tests were conducted showing no inflammatory markers. The radiographic finds sustained the changes encountered at the clinical examination. During her admissions in the Paediatric Rehabilitation Centre, she underwent a complex rehabilitation program which consisted of kinetotherapy, hydrokinetotherapy, massage, occupational therapy and physical agents. The program was designed according to the needs and the disease activity. Pharmacological means were used for the management of pain. The results were limited and the ability of standing up and walking were not recovered. Conclusion: In conclusion, JIA is a complex disease, which needs an early diagnosis and an early management program, which comprises of pharmacological treatment, rehabilitation program. Teamwork is necessary for managing JIA cases. The lack of compliance, and inadequate medication can lead to disabling complications.

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)

E25 The pattern of juvenile psoriatic arthritis in a tertiary centre in Libya

Abstract Background Juvenile psoriatic arthritis (JPsA) is a relatively rare condition in childhood as it represents ∼5% of the whole Juvenile Idiopathic Arthritis (JIA). There are fewer reports describing the characteristics and outcome of patients with JPsA. Aim To describe the characteristic features and the treatment modalities of JPsA among Libyan children in pediatric rheumatology clinic at the Tripoli Children Hospital which is the only referral clinic covering the western, northern and southern part of Libya and to compare our results with other populations worldwide. Methods All the medical records of the children who were diagnosed to have JPsA according to ILAR classification criteria or Vancouver criteria (definite or probable) from January 2001 to January 2020 were included and retrospectively reviewed. Data were collected about demographic, clinical, and laboratory features and treatment Results The study included a total of 30 cases of JPsA that represents a 12% of total JIA cases over the study period, the mean age at presentation was 5.8 ± 5.3 years with male to female ratio of 1:1, with the mean duration of symptoms prior to diagnosis of JIA of 9 ± 10 months. The foremost common type of arthritis was peripheral polyarthritis (83% &amp; 58.3%), with the Small joints commonly affected, 30% of patients had hip joint involvement. 13% of the patient had psoriasis, half of them before presentation of arthritis. Nail changes secondary to psoriasis was seen in 1 of the cohort, and the percentage of patients with dactylitis among the group was 30%. Family history of psoriasis found in 83% of the cohort, 26% found to be ANA positive. Chronic uveitis occurred in 10%. Patients of the total cohort, ANA positive, Unilateral anterior uveitis being the most common presentation. The following table summarizes the Medications used during the course of the disease At the time of the first visit 100%of total patients fulfilled the Vancouver criteria for juvenile PsA, While 5 of the12 patients didn’t fulfilled the ILAR criteria for JPsA. Conclusions Juvenile psoriatic arthritis constituted 12% of our patients with juvenile idiopathic arthritis. Juvenile psoriatic arthritis constitutes 8% to20% of JIA patients worldwide with equal gender distribution and peak age at presentation of 6–13 years. More than four peripheral joints involvement were the most frequent presentation. Half of the patients had skin involvement before the development of arthritis. Prevalence of chronic anterior uveitis was higher in our cohort than worldwide (10% vs 5% respectively) as an extra articular manifestation. All patients were treated with NSAIDs at presentation and/or during follow-up. Methotrexate was the most commonly used second-line agent Ethics Our study was approved by local ethical committee of pediatric department at Tripoli children hospital.

E27 Performance of systemic juvenile idiopathic arthritis classification criteria: one-centre experience—Libya

Abstract Systemic juvenile idiopathic arthritis (sJIA) constitutes a small part of juvenile idiopathic arthritis (JIA), yet has a disproportionally higher rate of mortality. In comparison to the other (JIA), systemic juvenile idiopathic arthritis has many distinct clinical features that arthritis may be a non-prominent and/or a delayed manifestation. The Pediatric Rheumatology International Trial Organization (PRINTO), currently is developing a new criterion for JIA classification which will allow for better identification of sJIA cases with delayed onset of arthritis and fail to fulfil the currently used International League Against Rheumatism (ILAR) and Yamaguchi criteria. It is useful in classification of children with systemic juvenile idiopathic arthritis (sJIA) particularly in ‘pre-arthritic’, pure systemic phase of the illness. Objectives The purpose of this study is to compare the performances of the Yamaguchi diagnostic criteria vs PRINTO criteria vs current ILAR criteria for systemic juvenile idiopathic arthritis. We compared the proportions of patients classified and how closely the 3 instruments are related. Methods The study was performed at the Department of Paediatric Rheumatology in Tripoli children hospital. It is a retrospective study, covering the date range 2006–2020. We used clinicobiologic data recorded within 7 months of diagnosis of Patients diagnosed with sJIA. We assigned a diagnosis of sJIA according to the 3 sJIA classification systems (the Yamaguchi diagnostic criteria, PRINTO criteria and according to ILAR classification). Results Three different classification criteria were analysed in 38 assigned sJIA children (13% of total JIA cases). Females were 28 (52.6%), and mean age of JIA onset was 7 ± 3.9 yr. The mean of the time delay between disease onset and diagnosis was 7 ± 9 months. Overall, the ILAR and PRINTO criteria were fulfilled in a higher number of patients in the study (n = 35) (92% of patients), As compared with the Yamaguchi criteria (n = 29).(76% of patients). Among the 7.9% patients who were unclassifiable using the ILAR criteria were diagnosed as sJIA by both the PRINTO and the Yamaguchi diagnostic criteria. 7.89% neither fulfilled the Yamaguchi or the PRINTO Criteria. The table below compares the performance of the three different criteria. Conclusions In this cohort the ILAR criteria appear to be superior in the early recognition and classification of sJIA. Yamaguchi diagnostic criteria can only be used to classify a minority of sJIA patients, leaving a large proportion of sJIA patients unclassified. Compared with the Yamaguchi criteria, ILAR JIA categories aligned better with PRINTO criteria. All the criteria should incorporate strengths of each other. Ethics Our study was approved by local ethical committee of pediatric department at Tripoli children hospital.

Normal neonatal TREC and KREC levels in early onset juvenile idiopathic arthritis

Dysregulated central tolerance predisposes to autoimmune diseases. Reduced thymic output as well as compromised central B cell tolerance checkpoints have been proposed in the pathogenesis of juvenile idiopathic arthritis (JIA). The aim of this study was to investigate neonatal levels of T-cell receptor excision circles (TRECs) and kappa-deleting element excision circles (KRECs), as markers of T- and B-cell output at birth, in patients with early onset JIA. TRECs and KRECs were quantitated by multiplex qPCR from dried blood spots (DBS), collected 2-5days after birth, in 156 children with early onset JIA and in 312 matched controls. When analysed from neonatal dried blood spots, the median TREC level was 78 (IQR 55-113) in JIA cases and 88 (IQR 57-117) copies/well in controls. The median KREC level was 51 (IQR 35-69) and 53 (IQR 35-74) copies/well, in JIA cases and controls, respectively. Stratification by sex and age at disease onset did not reveal any difference in the levels of TRECs and KRECs. T- and B-cell output at birth, as measured by TREC and KREC levels in neonatal dried blood spots, does not differ in children with early onset JIA compared to controls.

Juvenile idiopathic arthritis-associated uveitis: the experience in Libya

Background: Juvenile idiopathic arthritis (JIA) is the most common childhood rheumatic disease. The development of associated uveitis represents a significant risk for serious complications, including permanent loss of vision. Initiation of early treatment is important for controlling JIA-uveitis, but the disease can appear asymptomatically, making frequent screening procedures necessary for patients at risk. As our understanding of pathogenic drivers is currently incomplete, it is difficult to assess which JIA patients are at risk of developing uveitis. In the present study, we described the prevalence and clinical profile of JIA-associated uveitis and out come among children in the Libyan clinical settings. Methods: A total of 90 JIA patients who fulfilled International League of Associations for Rheumatology (ILAR) diagnostic criteria were included in this retrospective study. The data collected were age, gender, age at disease onset and at diagnosis, and follow-up duration. Duration from JIA diagnosis to uveitis diagnosis. Antinuclear antibody (ANA), rheumatoid factor (RF), and human leukocyte antigen B-27 were evaluated for each patient. Results: A total of eight uveitis cases were identified among the 90 JIA cases, which gives a prevalence of 8.9%. All cases were females (100.0%), the majority were Libyans (87.5%), and their mean age was 12.3 (SD=4.3) years old. The mean age at JIA onset for this group was 5.3(SD=2.3) years old and that for JIA diagnosis 6.2 (SD=2.6) years old, with an average duration of JIA of 6.8 (SD=3.7) years. The most common JIA subtype in this group was oligoarthritis (50%), followed by poly arthritis (37.5%). The majority of the cases had a negative rheumatoid factor test (75%), and ANA was positive only in 1 of 7 valid cases (14.3%). Conclusion: Better recognition of uveitis in JIA is required to improve its outcome and avoid serious complications.

Developing electronic health record algorithms that accurately identify patients with juvenile idiopathic arthritis

BackgroundThe objective of this study was to develop an algorithm that accurately identifies juvenile idiopathic arthritis (JIA) patients in the electronic health record (EHR). MethodsAlgorithms were developed in a de-identified EHR by searching for a priori JIA ICD-9 (International Classification of Diseases, Ninth Revision) and ICD-10-CM (International Classification of Diseases, Tenth Revision, Clinical Modification) codes and JIA-related keywords. Exclusion criteria were selected to remove other autoimmune diseases. A training set of 200 patients was randomly selected from patients containing ≥1 occurrence of a JIA ICD-9 or ICD-10-CM code. Case status was determined by a rheumatology clinic note documenting a JIA diagnosis before age 20. For each algorithm, positive predictive value (PPV), sensitivity, and F-measure were determined using the training set. ResultsWe developed 103 algorithms using combinations of ICD codes, keywords, and exclusion criteria. The algorithm requiring 4 or more counts of JIA ICD-9 or ICD-10-CM codes, keywords “enthesitis” and “uveitis”, and exclusion of ICD-9 or ICD-10-CM codes for systemic lupus erythematosus, dermatomyositis, polymyositis, and dermatopolymyositis had the highest PPV of 97% in the training set with an F-measure of 87%. There were 1,131 JIA cases returned by this algorithm. We validated the highest performing algorithm in a separate cohort from the training set with a PPV of 92% and an F-measure of 75%. ConclusionWe developed and validated JIA EHR algorithms with ICD-9 and ICD-10-CM codes to accurately identify a JIA cohort. Three algorithms achieved PPVs of 97%, each with different algorithm criteria, allowing for users to select an algorithm to best fit their research needs.

Juvenile Psoriatic Arthritis: Myth or Reality? An Unending Debate

Juvenile psoriatic arthritis (JPsA) accounts for 1-7% of all cases of juvenile idiopathic arthritis (JIA) and its definition has been a matter of controversy among pediatric rheumatologists for many years. The traditional attribution of JPsA to the spondyloarthropathy group was challenged in the early 1990s, whereas the recent demonstrations of its heterogenous nature have led to questions about its identification as a distinct category in JIA classification. It has been shown that children with the phenotype of JPsA can be divided in two subgroups, one presenting with the features of early-onset ANA-positive JIA, and another that belongs to the spectrum of spondyloarthropathies. The few studies that have compared the clinical characteristics and genetic determinants of JPsA with those of the other JIA categories have obtained contrasting findings. The debate on the categorization of JPsA as a distinct entity within JIA classification is still ongoing and has prompted the revision of its current classification.

Neutrophil Count, Platelet Indices, CRP and Their Association with Disease Activity of Juvenile Idiopathic Arthritis (JIA): A Study from Bangladesh

Background: Chronic inflammation of the joints in JIA patients is associated with raised levels of serum inflammatory biomarkers, which vary according to disease activity. Neutrophil count, platelet count, mean platelet volume (MPV), platelet distribution width (PDW), ESR and CRP are the essential markers to evaluate the disease activity status of JIA patients. Objectives: To assess neutrophil count, platelet count, MPV, PDW, CRP and Juvenile Arthritis Disease Activity Score (JADAS) in JIA patients and determine their association at the initial visit and six months after treatment. Methods: This prospective observational study was conducted from March 2019 to December 2020 in the department of paediatrics, Bangabandhu Sheikh Mujib Medical University, Dhaka, Bangladesh. Fifty newly diagnosed JIA cases fulfilling the ILAR classification criteria were included in the study. Disease activity was assessed by JADAS 27. JADAS 27 and inflammatory biomarkers were measured at the initial visit and six months of treatment and these were compared. Results: Mean neutrophil count, platelet count, and CRP significantly decreased at follow-up after six months of treatment. MPV and PDW were increased after six months compared to the initial visit, and the change was significant. Mean JADAS 27 also decreased significantly at follow-up. Neutrophil count, Platelet indices and CRP had a significant association with JADAS 27. Conclusion: In this study, neutrophil count, platelet indices, and CRP after six months of treatment were improved. A significant association of JADAS 27 was found with platelet indices, neutrophil count and CRP.

Frequency of rheumatic diseases in patients with familial Mediterranean fever

Purpose: Mutations in the Mediterranean FeVer (MEFV) gene, which causes familial Mediterranean fever (FMF), may also cause the emergence of other specific rheumatic diseases. This study aims to determine the frequency of other rheumatologic diseases in paediatric FMF patients, evaluate whether there are clinical and genetic differences between those with and without concomitant rheumatologic diseases, and compare the data with previous studies.&#x0D; Materials and methods: The files of FMF patients who were followed up at the paediatric rheumatology department were reviewed retrospectively. Demographic data, MEFV mutations, treatment, disease severity scores, and concomitant rheumatic diseases were recorded from the files.&#x0D; Results: There were 303 FMF patients (154 female/149 male). The mean age at diagnosis was 7.04±3.9 years. The mean disease duration was 5.33±3.13 years. In the cohort, 41 FMF patients (13.5%) were diagnosed with another rheumatic disease. There were 22 cases of juvenile idiopathic arthritis (53.6%), seven cases of vasculitis (17%), six cases of periodic fever aphthous stomatitis and adenitis syndrome (14.6%), three cases of Behçet disease (7.3%), two cases of acute rheumatic fever (4.8%), and one case of systemic lupus erythematosus (2.4%). 32 of these 41 FMF patients (78%) had the M694V mutation (homozygous in 11, heterozygous in 21). Disease activity scores PRAS and ISSF scores were higher in FMF with rheumatic diseases (p=0.002 and p

Incidence and prevalence of paediatric noninfectious uveitis in South Korea

AbstractPurpose: To investigate the nationwide incidence and prevalence of paediatric uveitis in South Korea according to period, age, anatomic type, and systemic associations.Methods: This nationwide population‐based cohort study used data from the Korean National Health Insurance Service from 2002 to 2018. This study included patients with noninfectious uveitis with at least three claims of diagnostic codes of uveitis on separate days with at least once claim of prescription codes of steroid and immunosuppressive agents. All the cases were classified as anterior or non‐anterior uveitis, and the incidence and prevalence were estimated by age, sex, period, and systemic associations.Results: A total of 10 862 616 patients over 128 688 078 person‐years were evaluated from 2005 to 2016. Overall, 5368 cases of anterior uveitis and 604 cases of non‐anterior uveitis were identified. The incidence and prevalence of paediatric noninfectious uveitis were 4.64 per 100 000 person‐years and 8.25 per 100 000 persons. Both the incidence and prevalence increased with age. Anterior uveitis accounted for 84.7% of prevalent cases (6.99 per 100 000 persons). Cases of juvenile idiopathic arthritis (JIA)‐associated uveitis accounted for 8.7% of paediatric noninfectious uveitis cases with a prevalence of 0.72 per 100 000. The proportion of systemic associations was higher and JIA‐related uveitis accounted for 11.2% of recurrent or chronic cases with a prevalence of 0.62 per 100 000.Conclusions: This is the first population‐based study investigating the largest population of paediatric patients with uveitis in Korea. The nationwide incidence and prevalence in 2005–2016 were 4.64 per 100 000 person‐years and 8.25 per 100 000, respectively. The proportion of JIA in paediatric noninfectious uveitis was 8.7%. These population‐based study findings provide a better understanding of the public health burden and aid in the planning of health‐care strategies for paediatric patients with uveitis.

13 Juvenile idiopathic arthritis and cardiovascular risk factors

BackgroundJuvenile idiopathic arthritis (JIA) is the most common pediatric rheumatic disease. Although, some patients achieve remission, some cases of JIA may persist into adulthood. Patients with JIA and other inflammatory joint diseases have increased cardiovascular disease risk compared with the general population.ObjectivesTo study the cardiovascular risk factors in JIA and their association with disease parameters.MethodsThis was a retrospective study including adults with long-standing JIA according to the International League of Associations for Rheumatology (ILAR) criteria over a period of 28 years (1994–2022). We collected sociodemographic and anthropometric parameters, clinical data, results of biological assessments, and data on prescribed therapies.We studied the following cardiovascular risk factors: family history of cardiovascular event, physical inactivity, smoking, arterial hypertension, diabetes, dyslipidaemia and obesity.ResultsWe included 29 patients. The M/F sex ratio was 0.71, the mean age was 35.69 ± 11.72 [18–61] years. The mean age of disease onset was 11.10 ± 4.25 [2–16] years. The average diagnostic delay was 52.96 ± 95.97 [0–336] months. The average disease duration was 24.48 ± 12.76 [1–47] years. The mean BMI was 21.20 ± 4.88 kg/m2 [14.17–27.55].The polyarticular form was the most frequent, noted in 55.2% of cases (n = 16). Extra-articular manifestations were observed in 55.2% of cases. Mean CRP was 42.74 ± 63.37 [2–218] mg/l and biological inflammatory syndrome was present in 19 cases. Rheumatoid factor, ACPA and anti-nuclear antibodies were observed in 12, 7 and 5 cases respectively.Corticosteroid therapy and NSAIDs were prescribed to 18 of the subjects.Cardiovascular risk factors were present in 41.4% (n = 12) of cases: family history of cardiovascular event (n = 2 cases), physical inactivity (n = 5 cases), smoking (n = 3 cases), arterial hypertension (n = 4 cases), diabetes (n = 4 cases), dyslipidaemia (n = 4), and BMI ≥ 25 kg/m² (n = 4).Following parameters were significantly higher in patients with cardiovascular risk factors: the presence of a biological inflammatory syndrome (81.8% vs 35.3%; p = 0.016), the frequency of prescription of corticosteroids (91.7% vs 52.9%; p = 0.026) and NSAIDs (83.3% vs 47.1%; p = 0.047).However, no significant difference was noted when comparing these parameters: gender, age, age of disease onset, disease duration and presence of extra-articular manifestations. Moreover, cardiovascular risk factors were not associated with the presence of rheumatoid factor, ACPA, and antinuclear antibodies.ConclusionInflammation, corticosteroid therapy and NSAIDs are associated with the presence of cardiovascular risk factors in JIA. The evaluation and control of this risk must be regular during patient follow-up. Control of inflammation and rationalization of treatment are necessary.