Uveitis In Juvenile Idiopathic Arthritis Research Articles

Prospective 5-Year Study of Chronic Uveitis in Juvenile Idiopathic Arthritis (JIA) in the Iraqi Population

Prospective 5-Year Study of Chronic Uveitis in Juvenile Idiopathic Arthritis (JIA) in the Iraqi Population

MicroRNAs as Biomarkers for Uveitis in Juvenile Idiopathic Arthritis

ABSTRACT Purpose Uveitis associated with juvenile idiopathic arthritis (JIA-U) is a clinically silent vision-impairing disease. Early detection and aggressive treatment are crucial for optimal visual outcome. Alterations in levels of microRNAs (miRNAs) are characteristic of autoimmune diseases. The present clinical study sought to explore the expression of miRNAs in JIA-U and their potential role as a predictive biomarker. Methods MiRNA expression profiling was performed on peripheral blood mononuclear cells derived from pediatric patients with JIA, JIA-U, or other types of uveitis using the high-throughput small-RNA sequencing (on Next Generation Sequencing (NGS)). Patient- and disease-related data were retrieved from the medical files. Main outcome measure was the differential expression of miRNAs among the groups. Results The cohort included 35 patients; 20 children with JIA-U (8 with active disease), 10 with JIA without ocular involvement, and 5 with other types of uveitis (4 with active disease). Mean age was 8.6 years; 83% were female. Nineteen patients (54%) received immunomodulatory treatment. The expression of miR-4485-3p was significantly increased in patients with JIA-U compared to patients with JIA alone (p < 0.05), with no difference between patients with active or inactive uveitis. The expression in patients with uveitis of other etiologies was similar to the expression in JIA-U patients. Conclusions This study demonstrates a differential expression profile of a specific miRNA in JIA patients with and without uveitis. If verified in larger studies, the findings may assist to identify JIA patients at risk to develop uveitis and to improve early detection of disease activity.

P010 To establish compliance with British Society for paediatric and adolescent rheumatology (BSPAR) and the Royal College of Ophthalmology (RCOphth) guidelines for screening for uveitis in juvenile idiopathic arthritis (JIA) at a District General Hospital: a retrospective audit

Abstract Background/Aims JIA associated Uveitis (JIA-U) is most common extra-articular manifestation of JIA. The prevalence of uveitis in JIA is approximately 8-30%, but may be as high as 45-57% in young patients with oligoarticular disease. Uveitis is predominantly asymptomatic and requires slit-lamp examination for diagnosis. Up to 67% of JIA-U cases develop ocular complications such as cataracts (20%), glaucoma (19%), band keratopathy (16%), macular oedema and irreversible permanent visual impairment. The primary objective of our audit was to establish compliance of our service with BSPAR/RCOphth 2006 uveitis screening guidelines and therefore reduce the risk of visual complications in children with JIA. Methods A retrospective review of clinical records of children with rheumatological conditions attending our clinic was performed over five-year period. A total of 42 patients with JIA were identified. Data was collated using excel sheet audit tool. Results Results were analysed in accordance with the four major guideline domains as below. 1. Initial Screening exam within 6 weeks of referral: 40 out of 42 children screened within six weeks with 95% compliance rate. 2. Symptomatic patients seen within one week of referral: 4 out of 13 children seen in time with 30% compliance. 70% non-compliance was due to non-availability of ophthalmology appointment. 3. Uveitis may flare on stopping Methotrexate/DMARD’s. Patients should get screened two monthly for six months: 9 out of 13 children were screened with 70% compliance. 15% non-compliance was due to non-availability of ophthalmology appointment. 4. Ongoing screening at two-monthly intervals from onset of arthritis for six months, then 3-4 monthly: 15 out of 20 children screened with 75% compliance. 20% non-compliance was due to non-availability of ophthalmology appointment. Conclusion Practice at our centre as judged against the BSPAR/RCOphth guidelines was suboptimal. The main cause of non-compliance was due to staffing issue, rather than lack of healthcare professional training, delay in onward referral or non-attendance of patients. Non-availability of regular ophthalmology cover with cross-site commitment stretched the uveitis screening service at our centre. We presented this audit in our trust paediatric governance meeting. As a result of this audit, the frequency of paediatric ophthalmology clinics was increased to one a week. The paediatric nurse specialist facilitated liaison with the ophthalmology department and improved compliance with attendance. Patient database on shared drive was regularly updated with separate column for ophthalmology follow-up. The ophthalmologists were granted access to this database detailing clinic attendance and diagnosis. Parent education, reinforced with appropriate literature and a child friendly clinic environment improved awareness and reduces non-attendance. Combined clinics with ophthalmology are being explored. We plan to re-audit the service in near future to assess the impact after above remedial measures. A similar multi-site nation-wide audit may help identify and address local and national concerns. Disclosure K.K. Garg: None. T. Walton: None. A. Holliday: None.

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.

Analysis of research hotspots and trends in pediatric ophthalmopathy based on 10 years of WoSCC literature.

Ophthalmopathy occurring in childhood can easily lead to irreversible visual impairment, and therefore a great deal of clinical and fundamental researches have been conducted in pediatric ophthalmopathy. However, a few studies have been performed to analyze such large amounts of research using bibliometric methods. This study intended to apply bibliometric methods to analyze the research hotspots and trends in pediatric ophthalmopathy, providing a basis for clinical practice and scientific research to improve children's eye health. Publications related to pediatric ophthalmopathy were searched and identified in the Web of Science Core Collection (WoSCC) database. Bibliometric and visualized analysis was performed using the WoSCC analysis system and CiteSpace.6.2.6 software, and high-impact publications were analyzed. This study included a total of 7,177 publications from 162 countries and regions. Of these, 2,269 from the United States and 1,298 from China. The centrality and H-index were highest in the United States at 0.27 and 66, respectively. The University of London and Harvard University had the highest H-index at 37. Freedman,Sharon F published 55 publications, with the highest H-index at 19. The emerging burst keyword in 2020-2023 was "eye tracking," and the burst keywords in 2021-2023 were "choroidal thickness," "pediatric ophthalmology," "impact" and "childhood glaucoma." Retinopathy of prematurity, myopia, retinoblastoma and uveitis in juvenile idiopathic arthritis were the main topics in the high-impact publications, with clinical studies in the majority, especially in retinopathy of prematurity. Eye health in children is a research hotspot, with the United States publishing the largest number of papers and having the greatest influence in research on pediatric ophthalmopathy, and China coming in second. The University of London and Stanford University had the greatest influence. Freedman, Sharon F was the most influential author. Furthermore, "choroidal thickness," "pediatric ophthalmology," "impact," "childhood glaucoma" and "eye tracking"are the latest hotspots in the field of pediatric ophthalmopathy. These hotspots represent hot diseases, hot technologies and holistic concepts, which are exactly the research trends in the field of pediatric ophthalmopathy, providing guidance and grounds for clinical practice and scientific research on children's eye health.

E10 Temporo-mandibular joint involvement risk factors in juvenile idiopathic arthritis

Abstract Introduction Recent recommendations suppose temporomandibular joint (TMJ) involvement in juvenile idiopathic arthritis (JIA) is a risk factor of poor outcomes and severe disease course. Objectives To compare the clinical characteristics of patients with juvenile idiopathic arthritis with and without TMJ involvement. Methods We analyzed the data of 753 patients with juvenile idiopathic arthritis aged 2–17 years, depending on TMJ arthritis (n = 43; 5.7%) or no TMJ arthritis (n = 710; 94.3%). Clinical, laboratory characteristics and treatment regimens were compared. Odds ratio (OR) analysis of sensitivity (Se) and specificity (Sp) was performed to obtained the predictors of TMJ involvement. Results Despite the similar age of onset, TMJ arthritis was associated with longer course of the disease, polyarticular JIA category, use of systemic corticosteroids and longer achievement of remission with similar rate of biologic administration. TMJ arthritis was associated with involvement of cervical spine, hip and shoulder arthritis. The main risk factors, associated with TMJ involvement in JIA were active joints &amp;gt; 8 (OR = 14.9 (5.8; 38.3), P = 0.0000001), delayed remission &amp;gt; 7 years (OR = 3.1 (1.6; 5.8), P = 0.0004), delayed hip involvement (OR = 4.6 (0.9; 22.6), P = 0.041), hip osteoarthritis (OR = 4.0 (1.2; 13.0), P = 0.014), and avascular necrosis of the hip. Cervical spine arthritis (OR = 10.3 (5.4; 19.8), P = 0.000001), and corticosteroid treatment (OR = 2.3 (1.2; 4.4), P = 0.0007) were associated with TMJ arthritis. Patients with TMJ arthritis required more biologics (OR-3.2 (1.6; 6.2), P = 0.0006). Oligoarticular JIA category and uveitis were protective against TMJ involvement. Data are in the table. Conclusion TMJ involvement is marker of poor disease prognosis. Early initiation of biologics and avoidance of corticosteroids might improve the risk of TMJ involvement. Ethics Written consent was obtained according to the declaration of Helsinki. This retrospective study's protocol was approved by the local Ethical Committee of Saint Petersburg State Pediatric Medical University (protocol number 11/10 from 23.11.2020).

Uveitis In Children

Uveitis in children is rarer than in adults, has variable clinical aspects, depending on etiopathogenesis, many forms are asymptomatic, and there can be difficulties in diagnosis and treatment. Uveitis in children can cause significant eye morbidity, with vision loss in 25/30% of cases, through complications, such as: cataract, glaucoma, band keratopathy. Uveitis in children is more frequently bilateral, with persistent, recurrent chronic evolution, resistant to conventional treatment and can be anterior, intermediate, posterior, panuveitis, acute or chronic. Juvenile idiopathic arthritis (JIA) is the most common cause of anterior uveitis in children 60% (girls, under 6 years old), in which uveitis is unilateral, non-granulomatous, with chronic evolution, associated with positive ANA. Uveitis in JIA has multiple complications and sequelae, with decreased vision. The prognosis of the disease is dependent on the establishment of an adequate early treatment in which the first line of therapy is topical, periocular, systemic, intravitreal corticotherapy, with possible side effects of steroid medication. Immunosuppressive treatment – the second line of therapy, is instituted if children do not respond to steroids, are cortico-dependent or have ocular and/or systemic complications – Methotrexate, Azathioprine, Cyclophosphamide, Cyclosporine, Chlorambucil, antiTNF alfa which are administered to patients at high risk of decreased vision – Infliximab, Adalimumab. The objective remains the treatment of complications: phacoemulsification in cataract with/without IOL (per primam, per secundam), medical and surgical treatment of glaucoma. Intermediate uveitis in children (10-25%) is manifested by myodesopsia, with a white eye, the most common etiology being idiopathic intermediate uveitis. Posterior uveitis represents almost 30% of cases, with multiple etiology, infectious inflammatory diseases (bacterial, viral, parasitic) non-infectious diseases (sarcoidosis, Behcet, VKH). Panuveitis in children may be present in acute tubulointerstitial nephritis in adolescents. Pseudouveitis is uveitis in retinoblastoma, leukemia. Uveitis in children is a serious disease, in which the diagnosis must be established quickly, with an appropriate (even aggressive) treatment, because the complications and sequelae present in the prolonged evolution of uveitis are accompanied by decrease/loss of vision. Ocular and systemic monitoring and screening of the disease are necessary for a favourable prognosis of the disease.

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.

Identification of novel autoantigens as potential biomarkers in juvenile idiopathic arthritis associated uveitis.

Many children with juvenile idiopathic arthritis (JIA) have autoantibodies, targeting nuclear components (anti-nuclear antibodies, ANA). ANA in JIA is associated with uveitis, an eye inflammation which may cause permanent vision impairment if not detected and treated. However, ANA-testing is neither specific nor sensitive enough to be a clinically reliable predictor of uveitis risk, and the precise autoantigens targeted by ANA in JIA are largely unknown. If identified, specific autoantibodies highly associated with uveitis could be used as biomarkers to facilitate identification of JIA patients at risk. Antibodies from six ANA-positive, oligoarticular JIA patients, with and without uveitis, were explored by two large-scale methods: (1) screening against 42,100 peptides on an autoimmunity profiling planar array, and (2) immunoprecipitations from cell lysates with antigen identification by mass spectrometry. Three hundred thirty-five peptide antigens, selected from proteins identified in the large-scale methods and the scientific literature were investigated using a bead-based array in a cohort of 56 patients with oligoarticular- or RF-negative polyarticular JIA, eight of which were having current or previous uveitis. In the planar array, reactivity was detected against 332 peptide antigens. The immunoprecipitations identified reactivity towards 131 proteins. Only two proteins were identified by both methods. In the bead-based array of selected peptide antigens, patients with uveitis had a generally higher autoreactivity, seen as higher median fluorescence intensity (MFI) across all antigens, compared to patients without uveitis. Reactivity towards 17 specific antigens was significantly higher in patients with uveitis compared to patients without uveitis. Hierarchical clustering revealed that patients with uveitis clustered together. This study investigated autoantigens in JIA and uveitis, by combining two exploratory methods and confirmation in a targeted array. JIA patients with current or a history of uveitis had significantly higher reactivity towards 17 autoantigens and a generally higher autoreactivity compared to JIA patients without uveitis. Hierarchical clustering suggests that a combination of certain autoantibodies, rather than reactivity towards one specific antigen, is associated with uveitis. Our analysis of autoantibodies associated with uveitis in JIA could be a starting point for identification of prognostic biomarkers useful in JIA clinical care.

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.

Comparison of pediatric patients with idiopathic uveitis, and uveitis due to juvenile idiopathic arthritis and Behçet’s disease

ABSTRACT Objectives The aim of this study is to compare the demographic, clinical features, treatment results and outcomes in pediatric patients with idiopathic uveitis and uveitis due to juvenile idiopathic arthritis (JIA) and Behçet’s disease (BD). Methods 97 pediatric uveitis patients were divided into three groups according to the etiology of uveitis: Group 1 comprised idiopathic uveitis patients, Group 2 uveitis patients who had JIA, and Group 3 uveitis patients with BD. Results Symptomatic presentation and intermediate uveitis were more common in Group 1 (p < 0.005). Asymptomatic presentation and anterior uveitis in Group 2 (p < 0.005), whereas symptomatic presentation and posterior uveitis in Group 3 (p < 0.005). Erythrocyte sedimentation rate (ESR) was higher in patients with BD or JIA uveitis than those with idiopathic uveitis (p < 0.005). Biologic therapy was more commonly used in JIA group compared to other groups (p < 0.005). Patients who had a complication related with uveitis were more common in females, asymptomatic disease course, and needed use of biologic treatment than in those without any complication (p < 0.005). Conclusion Uveitis accompanying rheumatologic diseases may have asymptomatic and insidious course but have higher ESR as an important notice.

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

30 Oligoarticular juvenile idiopathic arthritis-associated uveitis

BackgroundOligoarticular juvenile idiopathic arthritis (JIA) is a rare inflammatory disease that occur in children under the age of 16. JIA associated uveitis is the most frequent extra-articular manifestation. The uveitis can be sight-threatening and may be burdened with disabling morbidity. The uveitis seen in JIA is chronic anterior uveitis which is always asymptomatic in the initial stages.AimThe purpose of our survey is to report the incidence of uveitis in oligoarticular JIA and to determine the potential risk factors for the occurrence of uveitis in children with oligoarticular JIA.ResultsWe retrospectively evaluated 70 consecutive pediatric patients with oligoarticular JIA in the emergency and outpatient paediatric departement at the children's hospital in Tunisia between January 2005 and December 2021. A total of 12 cases (17.1%) with oligoarticular JIA associated uveitis where identified. The incidence of uveitis in these children was 0.7 cases per year.The uveitis had occurred before the joint manifestations in only one patient. The average time between the onset of symptoms and the occurrence of uveitis was 1.7 years with a maximum time interval of 5 years. Management of JIA-associated uveitis involved use of topical agents in half of our patients and systemic agents in the other half.JIA-associated uveitis has led to ocular complications such as cataracts (n = 5), glaucoma (n = 6), anterior/posterior synechiae (n = 8), and ultimately a visual impairment and blindness (n = 1). Eleven of the twelve patients with uveitis retained acceptable visual acuity.The presence of antinuclear antibody (ANA) has been identified as a risk factor for the occurrence of uveitis (p = 0.03).ConclusionCollateral damage of oligoarticular JIA include growth failure, muscle atrophy and intraocular damage. The main challenge during the management of oligoarticular JIA is the early detection of uveitis. Close cooperation between the rheumatologist and the ophthalmologist is essential to optimize outcome.

33 Oligoarticular juvenile idiopathic arthritis-associated uveitis

BackgroundOligoarticular juvenile idiopathic arthritis (JIA) is a rare inflammatory disease that occur in children under the age of 16. JIA associated uveitis is the most frequent extra-articular manifestation. The uveitis can be sight-threatening and may be associated with disabling morbidity. The uveitis seen in JIA is chronic anterior uveitis which is always asymptomatic in the initial stage.ObjectiveTo report the incidence of uveitis in oligoarticular JIA and to determine the potential risk factors for the occurrence of uveitis in children with oligoarticular JIA.ResultsWe retrospectively evaluated 95 consecutive pediatric patients with oligoarticular JIA in the paediatric emergency and outpatient department of the children's hospital in Tunisia between January 2005 and December 2021. 70 patients were included in this study. A total of 12 cases (17.1%) with oligoarticular JIA associated uveitis where identified. The incidence of uveitis in these children was 0.7 cases per year.The uveitis had occurred before the joint manifestations in only one patient. The average time between the onset of symptoms and the occurrence of uveitis was 1.7 years with a maximum time interval of 5 years. Management of JIA-associated uveitis involved use of topical agents in half of our patients and systemic agents in the other half.JIA-associated uveitis has led to ocular complications such as cataracts (n = 5), glaucoma (n = 6), anterior/posterior synechiae (n = 8), and ultimately a visual impairment and blindness (n = 1). Eleven of the twelve patients with uveitis had acceptable visual acuity. The presence of antinuclear antibody (ANA) was identified as a risk factor for the occurrence of uveitis (p = 0.03).ConclusionCollateral damage of oligoarticular JIA include growth failure, muscle atrophy and intraocular damage. The main challenge during the management of oligoarticular JIA is the early detection of uveitis. Multidisciplinary management by the rheumatologist and the ophthalmologist is essential to optimize outcome.

Comprehensive Assessment of Quality of Life, Functioning, and Mental Health in Children With Juvenile Idiopathic Arthritis and Noninfectious Uveitis.

Pediatric uveitis can lead to sight-threatening complications and can impact quality of life (QoL) and functioning. We aimed to examine health-related QoL, mental health, physical disability, vision-related functioning (VRF), and vision-related QoL in children with juvenile idiopathic arthritis (JIA), JIA-associated uveitis (JIA-U), and other noninfectious uveitis. We hypothesized that there will be differences based on the presence of eye disease. A multicenter cross-sectional study was conducted at four sites. Patients with JIA, JIA-U, or noninfectious uveitis were enrolled. Patients and parents completed the Pediatric Quality of Life Inventory (PedsQL; health-related QoL), the Revised Childhood Anxiety and Depression Scale (RCADS; anxiety/depression), the Childhood Health Assessment Questionnaire (C-HAQ; physical disability), and the Effects of Youngsters' Eyesight on Quality of Life (EYE-Q) (VRF/vision-related QoL). Clinical characteristics and patient-reported outcome measures were compared by diagnosis. Of 549 patients, 332 had JIA, 124 had JIA-U, and 93 had other uveitis diagnoses. Children with JIA-U had worse EYE-Q scores compared to those with JIA only. In children with uveitis, those with anterior uveitis (JIA-U and uveitis only) had less ocular complications, better EYE-Q scores, and worse C-HAQ and PedsQL physical summary scores compared to those with nonanterior disease. In children with anterior uveitis, those with JIA-U had worse PedsQL physical summary and C-HAQ scores than anterior uveitis only. Further, EYE-Q scores were worse in children with bilateral uveitis and more visual impairment. There were no differences in RCADS scores among groups. We provide a comprehensive outcome assessment of children with JIA, JIA-U, and other uveitis diagnoses. Differences in QoL and function were noted based on underlying disease. Our results support the addition of a vision-specific measure to better understand the impact of uveitis.

Клинический случай пациентки с увеитом, ассоциированным с ювенильным идиопатическим артритом

The prevalence of juvenile idiopathic arthritis is 0.05–0.6% in different countries, and in the territory of the Russian Federation it reaches 62.3 100 thousand children. The incidence of uveitis associated with juvenile idiopathic arthritis is 10–30%. Uveitis in juvenile idiopathic arthritis occurs in 80–93% of the type of chronic iridocyclitis. In 3–19.6% of cases, recurrent macular edema develops. In order to treat uveitis associated with juvenile idiopathic arthritis, systemic immunosuppressive therapy, local and systemic administration of antiinflammatory drugs are used. Uveitis in juvenile idiopathic arthritis is asymptomatic in the early stages of the disease, so screening ophthalmological examination of children 2–4 times a year is recommended. Purpose. Description of a clinical case of a patient with uveitis associated with juvenile idiopathic arthritis. Material and methods. A clinical observation of a patient with uveitis associated with juvenile idiopathic arthritis was described. Patient Z., 10 years old, underwent a standard ophthalmological examination, ultrasound examination, optical coherence tomography. Results. The patient had a low-symptomatic course of chronic persistent anterior uveitis, the development of cystic macular edema of the left eye. Anti-inflammatory treatment was carried out in the conditions of the academician S.N. Fyodorov Federal State Institution National Medical Research Center “Intersectoral Research and Technology Complex “Eye microsurgery” Ministry of Health of the Russian Federation”, Novosibirsk Branch. The rheumatologist recommended intensification of systemic immunosuppressive therapy. The prescribed treatment made it possible to preserve visual functions, reduce the frequency and severity of the development of relapses of the disease. Conclusions.Uveitis associated with juvenile idiopathic arthritis is a serious pathology, often accompanied by the development of complications, requires a multidisciplinary approach, regular monitoring and timely adequate treatment. Key words: uveitis, macular edema, juvenile idiopathic arthritis.

Epidemiology of Childhood Uveitis in a Tertiary Care Center: A 20-Year Study.

To investigate demographics and causes of pediatric uveitis in a Swiss tertiary reference center over a 20-year period. Retrospective cohort study on patients with uveitis aged less than 16 years seen at Jules-Gonin Eye Hospital between 1 January 2000 and 31 December 2019. Out of 2846 patients with uveitis seen in the Jules-Gonin Eye Hospital Ocular Immune-Infectiology Department, 317 (11.1%) were under 16 years of age and were included in this study. Median age at onset of the uveitis was 8.9 years (range 0 - 16). Anterior uveitis was the most frequent presentation (45.1%) followed by posterior uveitis in 26.2%, intermediate uveitis in 23.3%, and panuveitis in 5.4%. The inflammation was most frequently bilateral and non-granulomatous. A systemic inflammatory disease was found in 34% of the cases and an infectious cause in 24%. The repartition of the location of the uveitis was similar to previous reports from Western countries. Uveitis in juvenile idiopathic arthritis is the most frequent etiology related to a systemic disease in children. An infectious cause was found in 24% of our patients, which is a greater proportion than in adult cohorts.

Imaging-Based Uveitis Surveillance in Juvenile Idiopathic Arthritis: Feasibility, Acceptability, and Diagnostic Performance.

Children with juvenile idiopathic arthritis (JIA) need regular examinations for uveitis to avoid visual morbidity from the most common extraarticular manifestation of disease. This study was undertaken to investigate the feasibility, acceptability, and performance of optical coherence tomography (OCT) imaging-based diagnosis of uveitis. This observational cross-sectional study included children with and those without uveitis. The children underwent routine clinical examinations and anterior segment OCT scanning of intraocular inflammatory cells. Acceptability of image acquisition was assessed using a visual analog scale and length of time needed to acquire images. Interobserver and intraobserver variability of manual counting of acquired images (Bland-Altman limits of agreement), correlation between imaging and routine assessment, and sensitivity and specificity of anterior segment OCT detection of active inflammation were assessed. Of the 26 children ages 3-15 years (median age 8 years) who underwent imaging, 12 had active inflammation. All patients rated the acceptability of image acquisition as at least 8.5 on a scale of 0-10. Time taken to acquire images ranged from 1.5 minutes to 22 minutes (median time 8 minutes). There was good positive correlation between clinical assessment and image-based cell quantification (R2 = 0.63, P = 0.002). Sensitivity of anterior segment OCT manual image cell count for diagnosis of active inflammation was 92% (95% confidence interval [95% CI] 62-99%), specificity was 86% (95% CI 58-98%), and negative predictive value (ruling out uveitis) was 92% (95% CI 65-99%). Non-contact, high-resolution imaging for JIA uveitis surveillance is feasible, acceptable to patients, and holds the promise of transforming pediatric practice. Further work is needed to determine the analytic and clinical validity of anterior segment OCT quantification of active inflammation, and the clinical utility and cost-effectiveness of imaging-based disease monitoring.

Uveitis in Juvenile Idiopathic Arthritis: 18-Year Outcome in the Population-based Nordic Cohort Study

To assess the long-term outcome of uveitis in juvenile idiopathic arthritis (JIA). Population-based, multicenter, prospective JIA cohort, with a cross-sectional assessment of JIA-associated uveitis (JIA-U) 18 years after the onset of JIA. A total of 434 patients with JIA, of whom 96 had uveitis, from defined geographic areas of Denmark, Finland, Norway, and Sweden. Patients with onset of JIA between January 1997 and June 2000 were prospectively followed for 18 years. Pediatric rheumatologists and ophthalmologists collected clinical and laboratory data. Cumulative incidence of uveitis and clinical characteristics, JIA and uveitis disease activity, ocular complications, visual outcome, and risk factors associated with the development of uveitis-related complications. Uveitis developed in 96 (22.1%) of 434 patients with JIA. In 12 patients (2.8%), uveitis was diagnosed between 8 and 18 years of follow-up. Systemic immunosuppressive medication was more common among patients with uveitis (47/96 [49.0%]) compared with patients without uveitis (78/338 [23.1%]). Active uveitis was present in 19 of 78 patients (24.4%) at the 18-year visit. Ocular complications occurred in 31 of 80 patients (38.8%). Short duration between the onset of JIA and the diagnosis of uveitis was a risk factor for developing ocular complications (odds ratio [OR], 1.4; 95% confidence interval [CI], 1.1-1.8). Patients with a diagnosis of uveitis before the onset of JIA all developed cataract and had an OR for development of glaucoma of 31.5 (95% CI, 3.6-274). Presence of antinuclear antibodies (ANAs) was also a risk factor for developing 1 or more ocular complications (OR, 3.0; 95% CI, 1.2-7.7). Decreased visual acuity (VA) <6/12 was found in 12 of 135 eyes (8.9%) with uveitis, and 4 of 80 patients (5.0%) with JIA-U had binocular decreased VA <6/12. Our results suggest that uveitis screening should start immediately when the diagnosis of JIA is suspected or confirmed and be continued for more than 8 years after the diagnosis of JIA. Timely systemic immunosuppressive treatment in patients with a high risk of developing ocular complications must be considered early in the disease course to gain rapid control of ocular inflammation.

Success of Weekly Adalimumab in Refractory Ocular Inflammatory Disease

Abstract Purpose Evaluate the efficacy of weekly adalimumab (ADA) in patients with non-infectious ocular inflammation who failed standard every other week (EOW) dosing. Design Single-center, retrospective, study. Subjects Patients with uncontrolled inflammation on ADA EOW who were escalated to weekly dosing. Methods Chart review conducted at Northwestern University, January 2012 to April 2019. Outcome: Number of treatment successes on weekly ADA at 6 and 12 months. Results Fourteen of 25 patients (56%) achieved disease control at 6 months; no additional failures occurred at 12 months. Conclusion Weekly ADA is a reasonable treatment option in patients with recalcitrant inflammation on standard dosing.