JIA-associated Uveitis Research Articles

Corneal endothelium in paediatric patients with uveitis: a prospective longitudinal study

PurposeTo study the longitudinal effect of anterior chamber inflammation on the corneal endothelium in children.MethodsIn this prospective longitudinal observational study, children (aged <18 years) with anterior chamber inflammation and those...

S100 Proteins, Cytokines, and Chemokines as Tear Biomarkers in Children with Juvenile Idiopathic Arthritis-associated Uveitis

ABSTRACT Purpose: Biomarkers for juvenile idiopathic arthritis-associated uveitis (JIA-U) are needed. We aimed to measure inflammatory biomarkers in tears as a noninvasive method to identify biomarkers of uveitis activity. Methods: Tears were collected from children with JIA-U (n = 20) and pediatric controls (n = 20) using Schirmer strips. S100A8, A9, A12, IL-18, IL-8, IP-10, MCP-1, RANTES, and sICAM-1 were measured by ELISA and Luminex assays. Levels of biomarkers were compared between children with JIA-U and controls, and active and inactive JIA-U. Results: IL-8, sICAM-1, and S100A12 levels were similar between JIA-U and controls, but differed by activity. Active JIA-U had significantly increased S100A12 compared to inactive JIA-U (mean 27,722.5 pg/ML (SE 1.3) vs. 5,937.2 (1.3), p = .002), IL-8 (73.5 [1.2] vs. 36.2 [1.2], p = .009), and sICAM-1 (15,822.7 [1.2) vs. 8,778.0 [1.6], p = .024). Conclusion: We detected inflammatory biomarkers noninvasively in tears of children with JIA-U. IL-8, sICAM-1, and S100A12 are potential biomarkers for uveitis activity.

Juvenile idiopathic arthritis in Southeast Asia: the Singapore experience over two decades.

To examine the clinical characteristics, treatment and outcomes of juvenile idiopathic arthritis (JIA) patients evaluated in Singapore and compare those with reports elsewhere. Patients with JIA were recruited from our Singapore pediatric rheumatology registry from January 1997 to December 2015. Demographic, clinical, treatment, and outcome data were retrospectively collected. Nonparametric statistics were used to describe the data. Chi-squared, Mann-Whitney U, or Kruskal-Wallis tests were applied to compare differences between groups where appropriate. Multivariate logistic regression analyses were used to identify predictors for clinical parameters. Two hundred eighty-seven JIA patients with 60.6% males of predominantly Chinese descent were included in the study. The median onset age was 9years (IQR 5.3-12.6), and the median follow-up duration was 30.1months (IQR 9.1-61.7). Enthesitis-related arthritis (ERA, 32.8%) followed by persistent oligoarthritis (31.0%) was the most common. Elbow or ankle involvement predicted oligoarthritis extension (OR 15.8 (95% CI: 2.3-108.3, p = 0.005), 8.1 (95% CI: 1.5-45.3, p = 0.017)). JIA-associated uveitis was rare (2.8%) which paralleled the less common positive-ANA rate. Majority of our ERA patients had HLA-B27 (79.8%), together with older age predicted sacroiliitis (OR 4.7 (95% CI: 2.0-11.1, p < 0.05), OR 1.2 (95% CI: 1.1-1.3, p = 0.002)). TMJ involvement was under-reported. Methotrexate remained the most common DMARD used, but 36% of patients required biologics for which ERA and polyarthritis were the majority. Joint damage was rare. This study highlights geographical and ethnic differences in JIA epidemiology. Compared with reports elsewhere, our JIA population had many unique findings and good functional outcomes requiring regional study validation. Key points • ERA is the most prominent JIA subtype in Singapore with high prevalence of HLA-B27. • JIA-associated uveitis is rare in SEA and is not associated with ANA or JIA-subtypes. • Elbow and/or ankle involvement at presentation is associated with oligoarthritis extension in our JIA cohort.

Similarities in clinical course and outcome between juvenile idiopathic arthritis (JIA)-associated and ANA-positive idiopathic anterior uveitis: data from a population-based nationwide study in Germany

BackgroundTo analyze whether ANA-positive idiopathic anterior uveitis differs from JIA-associated uveitis concerning clinical course, response to treatment, and disease outcome.MethodsProspective study of the National Paediatric Rheumatological Database (NPRD) including its uveitis add-on module from the years 2002 to 2016. Cross-sectional data from the years 2002 to 2016 were analyzed. Patients with JIA-associated uveitis and with ANA-positive idiopathic anterior uveitis were included and the disease manifestation investigated in terms of uveitis characteristics and disease course.ResultsOf the total cohort of 34,458 patients enrolled in the NPRD, including 3551 patients with uveitis, those with detailed uveitis documentation were taken into account: 62 ANA-positive patients with idiopathic anterior uveitis (group 1), 688 patients with initial uveitis diagnosis after JIA onset (group 2), and 61 JIA patients with initial uveitis diagnosis before arthritis onset (group 3). Anterior uveitis was documented in 100%, 94%, and 80% of patients and with insidious onset of uveitis flare in 50%, 70.9%, and 56.1% each in groups 1, 2, and 3, respectively. Use of topical or systemic corticosteroids and conventional synthetic or biological DMARDs did not significantly differ between the patient groups, either at the initial or the 2-year follow-up (2-FU) visits (mean 2 years, each p > 0.05). At 2-FU, uveitis inactivity was achieved in 64.7%, 55.8%, and 61.5% of patients in groups 1, 2, and 3 (p > 0.05). Uveitis-related complications were more frequent at the initial visit and at 2-FU in groups 1 and 3, as compared to group 2.ConclusionsANA-positive idiopathic uveitis and JIA-associated uveitis do not significantly differ concerning clinical course of uveitis, treatment, and response to corticosteroids and DMARDs.

Importance of pediatric rheumatologists and transitional care for juvenile idiopathic arthritis-associated uveitis: a retrospective series of 9 cases

BackgroundJuvenile idiopathic arthritis-associated uveitis (JIA-U) is a serious condition associated with the risk of blindness. However, pediatric rheumatologists rarely encounter cases of blindness, because most patients reach adulthood during the course of follow-up before blindness occurs. Here, we report the progress of 9 patients with JIA-U, including 2 patients who became blind after the transition period. We aimed to highlight the importance of the role of pediatric rheumatologists and transitional care in preventing blindness associated with JIA-U.Case presentationWe conducted a retrospective analysis of the case records of 9 JIA-U patients (1 male, 8 female; median age 16.8 years, range 5.5–19.8 years). All patients presented with oligo-juvenile idiopathic arthritis (oligo-JIA) (one presented with extended oligo-JIA); the median age of uveitis onset was 5.0 years (range 3.0–13.0 years), and the onset of uveitis preceded the onset of arthritis in 2 patients. The median disease duration was 12.5 years (range 3.5–24.7 years); 4 patients had anti-nuclear antibody (ANA) positivity (≧1:160) (all with a homogeneous and speckled-pattern subtype). All patients were negative for rheumatoid factor. Eight patients received methotrexate, 7 patients received one or more biologic drugs (etanercept, infliximab, adalimumab, and golimumab), and 6 patients required ophthalmic surgery at an early age (≦ 18 years).Two patients developed blindness after the transition period. Medical examination by pediatric rheumatologists and use of biologics had been delayed in both patients. One patient developed depression after transition and interrupted her own treatment.ConclusionsThe reason for blindness in the 2 patients was thought to be the delay in the commencement of treatment and failure to provide transitional care. Inflammation is difficult to control in JIA-U even with appropriate treatment. Pediatric rheumatologists must be informed about the risk of JIA-U blindness, especially after transition. To ensure a good prognosis, the specialized treatment with the involvement of pediatric rheumatologists is necessary early on, and consideration for transitional medicine is essential. Therefore, this report reaffirms the importance of planned transitional care that has been advocated for globally.

Risk of Cataract Development among Children with Juvenile Idiopathic Arthritis-Related Uveitis Treated with Topical Corticosteroids

We sought to investigate the risk of cataract development among patients with juvenile idiopathic arthritis (JIA)-associated uveitis treated with topical corticosteroids. Retrospective cohort study. We included 75 patients with JIA-associated uveitis observed from July 1984 through August 2005 at a single academic center. Clinical data on these patients were collected by chart review and were analyzed. Incidence of new-onset cataract. Risk factors for cataract development were assessed with attention paid to the use of topical corticosteroids. Over a median follow-up of 4 years, the incidence of new-onset cataract was 0.04/eye-year (EY; 95% confidence interval [CI], 0.02-0.09). Of the 60 eyes in 40 patients who received topical corticosteroid therapy, there was a dose-dependent increase in the rate of cataract development among eyes receiving topical corticosteroids. The incidence of cataract was 0.01/EY for eyes treated with <3 drops daily and 0.16/EY (P = 0.0006 for log-rank test) for eyes treated with >3 drops daily. Among eyes receiving <2 drops daily, the incidence of cataract was 0/EY (95% CI [1 sided], 0.03/EY). Presence of posterior synechiae, active uveitis, and use of topical corticosteroids at presentation were significantly associated with cataract development after controlling for confounding variables. Use of topical corticosteroids was associated with cataract formation independent of uveitis activity. Using longitudinal data analysis and controlling for duration of uveitis, presence and degree of active uveitis, and concomitant use of other forms of corticosteroids in a time-updated fashion, treatment with <3 drops daily of topical corticosteroid was associated with an 87% lower risk of cataract formation compared with eyes treated with >3 drops daily (relative risk, 0.13; 95% CI, 0.02-0.69; P= 0.02). In our cohort, topical corticosteroid use was associated with an increased risk of cataract formation independent of active uveitis or presence of posterior synechiae. However, chronic use of topical corticosteroids dosed at <3 drops daily seemed to be associated with a lower risk of cataract development relative to eyes receiving higher doses over follow-up in the setting of suppressed uveitis. Proprietary or commercial disclosure may be found after the references.

Phenotype of Innate Immune Cells in Uveitis Associated with Axial Spondyloarthritis- and Juvenile Idiopathic Arthritis-associated Uveitis

ABSTRACT Purpose: To analyze circulating immune cells in patients with anterior uveitis (AU) associated to axial spondyloarthritis (SpA), or juvenile idiopathic arthritis (JIA). Methods: Venous blood samples were collected from healthy controls (n = 16), and either SpA (n = 19) or JIA (n = 23) patients with associated anterior uveitis (AU) during active flare, or after ≥3 months of inactivity. Frequencies of CD56+, MHC-I+, and S100A9+ monocytes, CCR7+ dendritic cells, CD56+dim natural killer (NK) cells and CD3+CD56bright T-cells were analyzed via flow cytometry. Serum S100A8/A9 levels were determined via ELISA. Results: SpA patients showed a reduced frequency of CD56+dim NK cells during uveitis activity, a constitutively activated monocyte phenotype, and elevated S100A8/A9 serum levels. In contrast, JIAU patients showed elevated frequencies of CD56+ monocytes and CCR7+ DC. Conclusion: Phenotype of peripheral immune cells differ between patients, probably contributing to different courses of acute onset AU in SpA and insidious onset AU in JIAU patients. Abbreviations: AU: anterior uveitis, AR: arthritis, JIA: juvenile idiopathic arthritis, SpA: axial spondyloarthritis

Quality of life in children suffering from juvenile idiopathic arthritis-associated uveitis.

Uveitis (JIA-U), the most common extra-articular manifestation in juvenile idiopathic arthritis (JIA), may cause severe impairment of vision in children and affect their quality of life (QoL). Considering the lack of uveitis-related QoL assessment questionnaire, and multidimensional nature of the Juvenile Arthritis Multidimensional Assessment Report (JAMAR), commonly used for monitoring and assessing the health status of children with JIA, we performed a cross-sectional study to investigate the potential of the JAMAR in estimation of QoL in children suffering from JIA-U. The study included 42 children with JIA, 21 of whom had JIA-U. Both children and their parents completed the JAMAR. We compared two groups of children (JIA-U and JIA without uveitis) and their parents against five extracted questionnaires items (QoL, functional ability, pain level, disease activity estimation, and current emotional state of the child) using the independent-samples t test to verify the differences and the Pearson correlation coefficient to measure the strength of a linear association between variables. No significant statistical difference in any of the examined variables was found between the two groups of children. In the groups of parents, current emotional state of children with JIA-U was assessed to be significantly worse (t = 2.05, p < 0.05) and the overall level of functioning significantly lower (t = 2.03, p < 0.05) than children without uveitis. Our results suggest the need for adding the uveitis-specific questionnaires items to JAMAR to improve its sensitivity and specificity in the assessment of QoL in children suffering from JIA-U, as well as designing a second assessment tool such as uveitis-specific questionnaires.

Tocilizumab in patients with anti-TNF refractory juvenile idiopathic arthritis-associated uveitis (APTITUDE): a multicentre, single-arm, phase 2 trial.

SummaryBackgroundUveitis associated with juvenile idiopathic arthritis is a cause of major ocular morbidity. A substantial proportion of children are refractory to systemic methotrexate and TNF inhibitors. Our aim was to study the safety and efficacy of tocilizumab in children with juvenile idiopathic arthritis-associated uveitis refractory to both methotrexate and TNF inhibitors.MethodsThis multicentre, single-arm, phase 2 trial was done following a Simon's two-stage design at seven tertiary hospital sites in the UK. Patients aged 2–18 years with active juvenile idiopathic arthritis-associated uveitis were eligible. All patients had been on a stable dose of methotrexate for at least 12 weeks and had not responded to treatment with a TNF inhibitor. Patients weighing 30 kg or more were treated with 162 mg subcutaneous tocilizumab every 2 weeks for 24 weeks, and participants weighing less than 30 kg were treated with 162 mg every 3 weeks for 24 weeks. The primary outcome was treatment response defined as a two-step decrease, or decrease to zero, from baseline in the level of inflammation (anterior chamber cells) at week 12, per the standardisation of uveitis nomenclature criteria. A phase 3 trial would be justified if more than seven patients responded to treatment. An interim analysis was planned to assess whether the trial would be stopped for futility, with futility defined as two or fewer treatment responses among ten participants. Adverse events were collected up to 30 calendar days after treatment cessation. The primary analysis was done in the intention-to-treat population and the safety analysis was done in all patients who started the treatment. This trial is registered with the International Standard Randomised Controlled Trial Number registry (ISRCTN95363507) and EU Clinical Trials Register (EudraCT 2015-001323-23).Findings22 participants were enrolled to the trial between Dec 3, 2015, and March 9, 2018, and 21 participants received treatment. One participant was found to be ineligible immediately after enrolment and was therefore withdrawn. Seven of 21 (median unbiased estimate of proportion 34% [95% CI 25–57]) responded to treatment (p=0·11). Safety results were consistent with the known safety profile of tocilizumab.InterpretationThe primary endpoint was not met, and thus the results do not support a phase 3 trial of tocilizumab in patients with juvenile idiopathic arthritis-associated uveitis. Importantly, data on the use of tocilizumab in clinical practice is now captured in national registries. Despite this trial not meeting the threshold required to justify a larger phase 3 trial, several patients responded to treatment; as such, tocilzumab might still be a therapeutic option in some children with uveitis refractory to anti-TNF drugs, given the absence of other treatment options.FundingVersus Arthritis and the National Institute for Health Research Clinical Research Network: Children.

Tocilizumab in patients with juvenile idiopathic arthritis-associated uveitis.

Tocilizumab in patients with juvenile idiopathic arthritis-associated uveitis.

Анализ увеита, ассоциированного с ювенильным идиопатическим артритом, по данным московского городского регистра детей с ревматическими заболеваниями

Objective. To evaluate the frequency, nature, and structure of therapy for uveitis associated with juvenile idiopathic arthritis (JIA) using the data from the Moscow city register of children with rheumatic diseases. Patients and methods. We analyzed the data of 117 patients with JIA and uveitis aged between 3 and 17 years residing in Moscow. Results. JIA-associated uveitis was diagnosed in 11% of children with JIA. It was more common in females than in males (13.1% vs 7.3% respectively). More than half of patients with uveitis (53.8%) had oligoarticular JIA, whereas 34.2% of patients had polyarticular RF-seronegative JIA. The majority of children (85.5%) received basic therapy, primarily with methotrexate (97% of them). Biologicals were administered to 64.1% of patients; of them, 68% received adalimumab. Sixty-one percent of children had bilateral eye lesions. In 41.5% of patients, the disease started with joint syndrome, while 46.3% of patients had both joint syndrome and eye lesions at onset and 12.2% of children initially had eye lesions. Remission of uveitis was registered in 75.6% of patients. Conclusion. Our results are consistent with both Russian and foreign data. Early diagnosis of uveitis and its timely treatment will decrease the incidence of complications in patients with JIA (including complete vision loss) and reduce the burden of disability for patients, their families, and the state. Key words: biological therapy, rheumatoid uveitis, rheumatic diseases in children, juvenile arthritis

Golimumab in Juvenile Idiopathic Arthritis-associated Uveitis Failing Adalimumab

Golimumab in Juvenile Idiopathic Arthritis-associated Uveitis Failing Adalimumab

Oral phospholipidic curcumin in juvenile idiopathic arthritis-associated uveitis.

To evaluate the efficacy and the safety of curcumin-phosphatidylcholine complex in children affected by juvenile idiopathic arthritis-associated uveitis as an adjunctive treatment to chronic systemic immunosuppressive therapy. In this retrospective, longitudinal study, we treated patients affected by juvenile idiopathic arthritis-associated uveitis with residual low-grade inflammatory activity in the anterior chamber with one tablet of curcumin-phosphatidylcholine complex per day, over a year. Low-grade inflammatory activity was characterized by flare 1+ at slit-lamp examination and 10-50 photon counts per ms) at the FC500 laser flare meter. Inactivity of uveitis was defined as complete disappearance of flare at the slit-lamp examination and values <10 ph/ms at laser flare meter. Conversely, recurrence of the uveitis was defined as a one-step increase from baseline in anterior chamber cells levels or laser flare meter measurements >50 ph/ms. A total of 22 out of 27 patients (81%) achieved inactivity at the end of the study. Five patients (19%) did not show a significant reduction in anterior chamber flare, remaining stable throughout the follow-up. Only three episodes of flare-ups in three different patients were recorded. Overall, the treatment was well tolerated by all patients and no ocular discomfort, ocular side effects, or allergic reactions were registered. Adjunctive therapy with curcumin in patients affected by juvenile idiopathic arthritis-associated uveitis improves mild chronic anterior chamber flare and presents a good safety profile. Despite being mild, anterior chamber inflammation should be minimized to avoid the development of sight-threatening complications in these patients.

Treatment Withdrawal Following Remission in Juvenile Idiopathic Arthritis: A Systematic Review of the Literature.

Early diagnosis and treatment of juvenile idiopathic arthritis (JIA) with conventional and biologic disease-modifying anti-rheumatic drugs have vastly improved outcomes for children with these diseases. Currently, a large proportion of children with JIA are able to achieve clinical inactive disease and remission. With this success, important questions have arisen about when medications can be stopped and how to balance the risks and benefits of continuing medications versus the potential for flare after stopping. The aim was to conduct a systematic review of the available literature to summarize current evidence about medication withdrawal for JIA in remission. We conducted a systematic literature search in PubMed and Embase from 1990 to 2019. References were first screened by title and then independently screened by title and abstract by two authors. A total of 77 original papers were selected for full-text review. Data were extracted from 30 papers on JIA and JIA-associated uveitis, and the quality of the evidence was evaluated using National Institutes of Health (NIH)/National Heart, Lung, and Blood Institute (NHLBI) tools. Studies on biochemical and radiologic biomarkers were also reviewed and summarized. Most studies investigating treatment withdrawal in JIA have been observational and of poor or fair quality; interpretations of these studies have been limited by differences in study populations, disease and remission durations, the medications withdrawn, approaches to withdrawal, and definitions of disease outcomes. Overall the data suggest that flares are common after stopping JIA medications, particularly biologic medications. Clinical characteristics associated with increased risks of flare have not been consistently identified. Biochemical biomarkers and ultrasound findings have been shown to predict outcomes after stopping medications, but to date, no such predictor has been consistently validated across JIA populations. Studies have also not identified optimal strategies for withdrawing medication for well-controlled JIA. Promising withdrawal strategies include discontinuing methotrexate before biologic medications in children receiving combination therapy, dose reduction for children on biologics, and treat-to-target approaches to withdrawal. These and other strategies require further investigation in larger, high-quality studies. The published literature on treatment withdrawal in JIA has varied in design and quality, yielding little conclusive evidence thus far on the management of JIA in remission. Given the importance of this question, international collaborative efforts are underway to study clinical and biologic predictors of successful medication withdrawal in JIA. These efforts may ultimately support the development of personalized approaches to withdrawing medication in children with JIA in remission.

Real-World Outcomes of Biologic Therapy for Juvenile Idiopathic Arthritis Associated Uveitis

Purpose: We aim to report the outcomes and complications of patients with uveitis from Juvenile Idiopathic Arthritis (JIA) treated with biologic therapy. Materials and Methods: All patients who underwent treatment with biologic therapy for uveitis in the context of JIA between 2016 and 2017, followed in the Uveitis Department in Hospital de Santa Maria, were retrospectively included in the study. Patient medical records were analyzed retrospectively and clinical outcome and complications were registered. Results: Nine patients (7 females, 2 males, 18 eyes) were included in the study. The mean age of JIA diagnosis was 6.6 years (±4,5) and the mean age of uveitis diagnosis was 7.9 years (±3,0). Six patients were being treated with adalimumab (ADA), 2 with Infliximab (IFX) and 1 with Tocilizumab (TCZ), and of them 8 (88,9%) were treated successfully for uveitis. The resolution of anterior chamber inflammation was obtained with a mean interval of 2 months for ADA and 3 months for IFX. Before starting biologic therapy 33,3% (n=3) patients were on oral steroids, 55,6% (n=5) were doing methotrexate and 11,1% (n=1) was doing cyclosporine. After biological treatment, no patient was on oral steroids. Regarding adverse effects, there are records of one reaction to infusion during the treatment with IFX and liver enzymes derangement with ADA, that resulted in the switch of therapy respectively to ADA and IFX. Conclusion: In this small cohort, ADA, IFX and TCZ showed to be effective and relatively safe for treatment of JIA-associated uveitis.

Association of apoptosis genes in PDCD1 but not PDCD1LG2, FAS, and FASLG with pediatric idiopathic uveitis in Han Chinese.

Previous studies have shown that aberrant T lymphocyte apoptosis is involved in the pathogenesis of uveitis. Genetic variants of apoptotic pathway-related factors (including PDCD1, PDCD1LG2, FAS, and FASLG) may affect apoptosis and in turn predict susceptibility to autoimmune disease. This has not yet been studied in pediatric idiopathic uveitis (PIU) and juvenile idiopathic arthritis (JIA)-associated uveitis and was therefore the subject of the study presented here. Fourteen single-nucleotide polymorphisms (SNPs) of several apoptosis-related pathway genes were analyzed in 1238 PIU patients, 128 JIA-associated uveitis patients and 1114 healthy controls using the iPLEX Gold Assay and MassARRAY platform. A lower frequency of the PDCD1/rs6710479 CC genotype in PIU patients was found when compared to controls (Pc = 3.42 × 10-3). A higher frequency of the PDCD1/rs7421861 A allele (Pc = 4.85 × 10-3) was observed in PIU patients as compared with controls. Stratification analysis showed a positive association of band keratopathy with the PDCD1/rs7565639 CT genotype (Pc = 1.05 × 10-2) and a negative association of this parameter with the PDCD1/rs7565639 C allele (Pc = 3.76 × 10-2). This study revealed that rs6710479 and rs7421861 in the PDCD1 gene confer susceptibility to PIU in Han Chinese. A stratified analysis showed that PDCD1/rs7565639 is associated with band keratopathy in PIU patients.

Frequency and Identification of Risk Factors of Uveitis in Juvenile Idiopathic Arthritis: A Long-term Follow-up Study in a Cohort of Italian Children.

Juvenile idiopathic arthritis (JIA) is the most common rheumatic disease of childhood. The JIA-associated uveitis represents the most common extra-articular manifestation. The main aim of this study was to evaluate frequency and risk factors of uveitis in a pediatric population affected by JIA. One hundred eight Italian children with JIA were followed during a follow-up period of 13 years. Association between uveitis, antinuclear antibodies (ANAs), and subtype of arthritis has been estimated, and Kaplan-Meier curves were generated to assess the probability of ocular complications during the follow-up period. Twenty-one patients developed uveitis, after 96.5 ± 50.4 months from the enrollment. According to JIA subtypes, the oligoarthritis subtype was characterized by the highest prevalence (39%) of uveitis. The greatest risk of uveitis has been detected in oligoarthritis patients associated to ANA positivity (risk ratio, 8.6; 95% confidence interval, 2.27-32.9; χ = 20.4), whereas the worst evolution was revealed in patients with oligoarthritis and high levels of ANAs, with a progression time of 36 months (log-rank χ = 16.39; p < 0.0001; risk ratio, 18; 95% confidence interval, 7.3-44.2). Patients with early-onset ANA-positive oligoarticular JIA have the highest risk of developing uveitis. A routine ophthalmological follow-up is required at regular intervals, even though the joint disease is clinically quiescent.

Juvenile idiopathic arthritis-associated uveitis: a retrospective analysis from a centre of South Italy

PurposeTo investigate the clinical and laboratory characteristics of the children affected by juvenile idiopathic arthritis (JIA) who developed uveitis.MethodsIn this retrospective study, we have examined data of 109 patients aged from 3 to 16 years, affected by JIA and followed at Paediatrics Rheumatology Clinic and Ophthalmology Clinic of University Hospital of Messina in the period from 2007 to 2017. The main outcome measures were clinical and laboratory findings related to JIA and ocular involvement. The prevalence of ocular signs and symptoms was determined and correlated with age.ResultsTwenty-one (19.3%) subjects developed uveitis. Two different peaks of age with ocular involvement were registered. The first occurred between 4 and 6 years and the second between 10 and 12 years. All subjects in the first group resulted to be female, presented oligoarticular arthritis and chronic anterior uveitis. In the second group, the 84% of patients were male with different types of JIA and acute anterior uveitis. The prevalence of ocular complications was higher in the first group.ConclusionsTwo peaks of age emerged and were characterized by different clinical outcomes of arthritis and ocular involvement. The first occurred between 4 and 6 years and interested females affected by oligoarticular JIA who develop chronic anterior uveitis. The second appeared at 10–12 years and interested older males affected by different types of JIA with acute anterior uveitis. Early diagnosis and cooperation between paediatric rheumatologist and ophthalmologist are of great importance in the proper management of JIA children with uveitis.

Visual Function And Quality Of Life In A Cohort Of Swedish Children With Juvenile Idiopathic Arthritis.

PurposeTo evaluate quality of life (QoL) in children with juvenile idiopathic arthritis (JIA).MethodsForty children with a mean age of 7.9 years were included. The children underwent an ophthalmological examination and completed questionnaires on physical function (CHAQ) and vision-related (VR) QoL (EYE-Q).ResultsNo differences regarding visual acuity (VA), refraction, intraocular pressure or physical or VRQoL were found between those with JIA without (n=33) and those with JIA-associated uveitis (n=7). When comparing physical function measured by CHAQ disability index and JIA subtype, a difference was found; children with polyarthritis scored the worst (p=0.0098). Children with subnormal VA scored worse on EYE-Q compared with those with normal VA (p=0.013). We found correlations between duration of JIA and CHAQ disability index (r=−0.42, p=0.0007) and CHAQ well-being (r=−0.34, p=0.022).ConclusionThis study indicates the importance of measuring not only physical function but also VRQoL in children with JIA and JIA-associated uveitis.

Identification of Ocular Autoantigens Associated With Juvenile Idiopathic Arthritis-Associated Uveitis.

The purpose of the current study was to analyze the binding patterns of serum autoantibodies from juvenile idiopathic arthritis (JIA) and JIA-associated uveitis (JIAU) patients to proteomes from different ocular tissues and to identify potential ocular autoantigens in JIAU. Proteomes from porcine iris, ciliary body, or retina tissue were isolated, separated using 2D-gel electrophoresis, and transferred to a blotting membrane. The binding pattern of serum antibodies from JIA or JIAU patients or healthy controls to ocular proteins was visualized by using anti-human IgG secondary antibodies and chemiluminescence reaction. Selected protein spots were excised from silver-stained 2D gels and subjected to mass spectrometry. Serum antibodies binding to ocular proteins were detected in all patient groups and healthy controls. Irrespective of the patient groups, serum antibodies bound to 49 different protein spots of the retina proteome, to 53 of the ciliary body proteome, and to 44 of the iris proteome. The relative binding frequency of sera to these iris protein spots was significantly higher in JIAU than in JIA patients or healthy controls. Particularly in JIAU patients, cluster analyses indicated a broad range of serum antibodies directed against ocular antigens, mostly in the iris proteome. Iris proteins frequently bound by serum antibodies in all groups were identified as tubulin beta chain, vimentin, ATP synthase subunit beta, actin, and L-lactate dehydrogenase B chain. Iris proteins exclusively bound by JIAU serum antibodies were heat shock cognate 71 kDa protein and keratin. Although serum autoantibody binding to ocular antigens was not disease-specific, a significant diversity of autoantibodies against a broad range of antigens, particularly from the iris tissue, was detected in JIAU patients. As the iris is a major site of inflammation in JIAU, the present data give further evidence that autoantibodies may be involved in JIAU immunopathology.