Psoriatic Juvenile Idiopathic Arthritis Research Articles

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.

Golimumab for Polyarticular Juvenile Idiopathic Arthritis and Psoriatic Arthritis: Pharmacologic and Clinical Considerations.

Psoriatic arthritis is a chronic debilitating autoimmune condition, and when diagnosed in patients before the age of eighteen, it is considered pediatric polyarticular juvenile idiopathic arthritis. Monoarticular or polyarticular psoriatic arthritis can be distinguished from other arthropathies by its unique cutaneous manifestations. With numerous treatments already in clinical practice, there are numerous options for treatment. The current literature indicates an elevated level of tumor necrosis factor is present in the epidermis of patients with psoriatic arthritis when compared with the general population. For this reason, anti-tumor necrosis factor therapies have become a hallmark option for psoriatic arthritis patients. Golimumab, a human monoclonal antibody tumor necrosis factor-alpha (TNF-a) receptor antagonist, was chosen as the focus therapy for this investigation. The mechanism of action behind anti-tumor necrosis factor-alpha blockers involves the binding of human TNF-a soluble and transmembrane proteins to competitively inhibit TNF-a from binding to its cellular receptors. The present investigation evaluated current treatment options available for both juvenile- and adult-onset psoriatic arthritis and compared them with the efficacy seen with golimumab use. Pediatric patients included children ages 2-17, while adult populations included adults 18-83 years old. The Food and Drug Administration has approved golimumab for the treatment of rheumatoid arthritis, psoriatic arthritis, ankylosing spondylitis, ulcerative colitis, and polyarticular juvenile idiopathic arthritis. The results of four different studies reporting on the therapeutic effects and adverse events of golimumab use in psoriatic arthritis, juvenile psoriatic arthritis, juvenile idiopathic arthritis, and juvenile polyarticular arthritis were used for comparison. The meta-analysis referenced studies including children ages 2-17 with no reference mentioning children less than age 2. Based on the results of each study, it can be concluded that golimumab, a human monoclonal antibody that prevents the activation of cellular inflammatory reactions when it binds to the TNF-a receptor, is an effective option for patients with active psoriatic arthritis and psoriatic juvenile idiopathic arthritis and for patients who are no longer responding to their current treatment with adalimumab. Each study also reported minimal adverse events associated with golimumab use, and the drug can be safely used in the pediatric population.

Patterns of clinical joint inflammation in juvenile idiopathic arthritis

ObjectivesWe studied patterns of joint inflammation in juvenile idiopathic arthritis (JIA) to assess whether joint activity recurs locally in the same joints.MethodsJoints of 91 patients of the BeSt for Kids...

Juvenile idiopathic arthritis in children and adolescents with atopic dermatitis: A Finnish nationwide registry study

Juvenile idiopathic arthritis in children and adolescents with atopic dermatitis: A Finnish nationwide registry study

Overview on Juvenile Psoriatic Arthritis: A Review

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

AB1237 COMPARISON BETWEEN ENZYME IMMUNOASSAY AND CHEMILUMINESCENCE TO DETERMINE THE CONCENTRATION OF SERUM CALPROTECTIN AND ITS ASSOCIATION WITH CLINICAL VARIABLES IN PEDIATRIC RHEUMATOLOGY.

BackgroundSerum calprotectin (SC) is an emerging biomarker in the measurement of inflammation. It can be determined by different techniques, such as enzyme immunoassay (EIA) or chemiluminescence (CLIA). However, there are no studies comparing whether there is a correlation between the two diagnostic methods in paediatric rheumatologic diseases.Objectives(i) To assess whether there are differences between serum calprotectin (SC) levels determined by EIA (Bühlmann) method and CLIA (QUANTA Flash) in pediatric age patients with systemic autoimmune rheumatic disease (SARD). (ii) To evaluate which clinical and analytical variables are associated with an increase of SC in each method.MethodsAnalytical cross-sectional study that included patients from a pediatric rheumatology specialized unit between 02/2017 and 05/2021. We included 41 patients with SARD who had at least one SC analysis determined by EIA in their routine controls (144 serum) and afterwards had SC determined again, this time using the CLIA method.The collected variables were sex, age, remission according to clinical judgment, swollen joint count according to physical examination (PE Count) and ultrasound (US Count), Juvenile Arthritis Disease Activity Score according to physical examination (PE JADAS-27) and ultrasound (US JADAS-27), C-reactive protein (CRP) and erythrocyte sedimentation rate (ESR).As for the statistical analysis, intraclass correlation (ICC) and paired samples t-test were performed to compare the two methods. Univariate linear regression was performed to study the association between EIA, CLIA and both clinical and analytical variables.ResultsWe included 41 patients, 50.1% were women with a mean age (± SD) of 13.1 (± 3.8) years. The details of their descriptive characteristics were: mean SC (EIA) of 3.1 (±1.8) µg/ml, mean SC (CLIA) of 2.5 (±1.6) µg/ml, mean CRP of 2.6 (± 6.5) mg/l, mean ESR of 10.1 (± 11) mm/h, and mean PE JADAS-27 of 2.8 (± 8). Most frequent diagnosis was oligoarticular juvenile idiopathic arthritis (JIA) (24.4%), followed by enthesitis-related (ERA) JIA (12.2%) and polyarticular JIA (12.2%), familial Mediterranean fever (FMF) (9.8%), psoriatic JIA (4.9%), systemic JIA (4.9%) and syndrome of periodic fever, aphtous stomatitis, pharyngitis, and cervical adenitis (PFAPA) (4.9%), vasculitis (4.8%), and undifferentiated JIA (2.4%). Clinical diagnosis was unspecific in 9.8% of the patients. In our sample, 66.7% were in clinical remission at the discretion of the specialist.A statistically significant Pearson’s CCI of 0.77 (95%CI 0.70-0.83; p=0.00) was observed as a single measure between EIA and CLIA and with an average of 0.87 (95%CI=0.82-0.91; p=0.000). Figure 1 shows the dispersion of this correlation.On the other hand, we observed a statistically significant difference in the mean between both methods of 0.58 (95%CI=0.40-0.77; p=0.000), observing a greater difference in SC (EIA) > 4 µg/ml.A significant association was observed between EIA and clinical remission, joint count, JADAS and CRP; and also between CLIA and clinical remission, JADAS and CRP. The analysis performed is shown in Table 1.Table 1.Association between EIA and CLIA with clinical and analytical variables.RemissionPE CountUS CountPE JADASUS JADASbpbpbpbpbpSC(EIA)0.430.0110.140.0270.080.0240.090.0000.060.000SC(CLIA)0.530.000-0.000.9450.310.3200.050.0210.040.015SexGenderESRCRPbpbpbpbpSC(EIA)0.000.9970.050.2070.000.4870.050.027SC(CLIA)-0.240.3620.010.7950.020.1050.080.000b, regression coefficient; p, statistical significance.ConclusionThere is a good correlation between EIA and CLIA methods to determine SC in pediatric patients with SARD. Significant differences were observed between both methods above the value of 4 µg/ml. This fact could be explained by methodological differences, since CLIA discriminates better at higher values than EIA.An association was observed between both methods and variables of remission or disease activity.Disclosure of InterestsNone declared

P16 Using virtual reality to help manage needle phobia in juvenile idiopathic arthritis

Case report - IntroductionNeedle phobia is a common issue amongst children, particularly with long-term illnesses requiring repeated interventions. Psychological interventions such as coping strategies and distraction techniques can be effective in many of these children, but is not universally successful. Recent research has reportedthat the use of virtual reality (VR) headsets as a distraction technique can be effective at alleviating both pain and fear in children receiving intravenous (IV) injections, cannulation and venepuncture. Although use of VR distraction is becoming more widespread in some disciplines (such as paediatric oncology/A&E), it is yet to be widely adopted in paediatric rheumatology.Case report - Case descriptionWe report the case of a 9-year-old boy (ME) who in early 2019 developed psoriatic juvenile idiopathic arthritis (JIA) and an associated severe, widespread plaque psoriasis causing him substantial discomfort and distress. He has been treated with subcutaneous adalimumab biosimilar 40 mg fortnightly and oral methotrexate 20 mg weekly, alongside Keppra 1000 mg twice daily for epilepsy. Although he is just tolerating the subcutaneous adalimumab, he is requiring intermittent steroid joint injections under general anaesthetic for recurrent flares of his polyarticular arthritis and regular monitoring phlebotomy. He has been developing progressive needle phobia over the past 8 months, with superimposed anxiety about general anaesthetics with particular fear of the anaesthetic provoking an epileptic seizure.Despite psychological intervention to teach coping strategies and standard play therapy distraction techniques, both phlebotomy and cannulation for general anaesthetics have become more distressing and challenging for him. During two recent day case admissions for joint injections, despite premedication with sedatives, distraction techniques and psychology interventions, he became so distressed that attempts to anaesthetise him had to be abandoned. This has resulted in prolonged periods of continuing joint inflammation and pain. Discussion with local paediatric oncology colleagues resulted in a suggestion of using recently acquired VR headsets as an effective distraction technique. He has had a previous positive experience of using VR headsets in a play environment. He is receiving VR training intervention from the oncology team and is now fully engaged with the idea of using VR to distract him from negatively focussing on his next planned general anaesthetic intervention.Case report - DiscussionUnmanaged needle-related procedural pain in children is associated with increasing pain and stress in subsequent procedures and fear/avoidance of medical care which can affect compliance in chronic disease management. The psychological distress for children with needle phobia can add to existing psychological impact from JIA and increase family stress of caring for an ill child. The cost of poor management of needle-phobic children can be considerable, with cancelled or increased time for procedural interventions and restricted therapeutic interventions. Needle phobias in childhood may persist into adulthood.Employing distraction techniques when children undergo medical procedures can help them reduce anxiety. VR is a method of captivating an individual’s interest in a virtual environment and can help divert attention from potentially distressing situations in the real world. Recent studies have reported VR as an effective distraction technique in children. Chan (2019) reports that VR significantly decreased the pain and post procedural anxiety experienced by children aged 4—11 years old during venepuncture/IV cannulation compared to controls. The majority of children in the VR group expressed interest in utilising VR again in the future, to improve their experience of IV injections. Chen (2020) subsequently reported a randomised controlled trial which significantly reduced pain and fear experienced from IV injections by the children in the VR group.There are cost implications to both buying VR headsets/software and training play specialists/specialist nurses to use the equipment effectively. Furthermore, the acceptability of using VR with regards to the child’s age, engagement with age-appropriate software packages and whether a passive VR or a more interactive ‘gaming’ experience is preferred should be considered. The use of VR is not a panacea, but is potentially effective as an additional new tool in clinicians’ efforts to reduce the impact of needle phobia.Case report - Key learning pointsAddressing needle phobia in children can require novel strategies.VR has shown success as a distraction technique to alleviate pain and fear in children undergoing needle related procedures.The implementation of VR in paediatric rheumatology has cost and staff training implications.The use of age-appropriate software and an individual child’s preference for passive/active interaction with VR should be considered.

Patterns of etanercept use in juvenile idiopathic arthritis in the Childhood Arthritis and Rheumatology Research Alliance Registry

BackgroundWe aimed to characterize etanercept (ETN) use in juvenile idiopathic arthritis (JIA) patients enrolled in the Childhood Arthritis and Rheumatology Research Alliance (CARRA) Registry.MethodsThe CARRA Registry is a convenience cohort of patients with paediatric onset rheumatic diseases, including JIA. JIA patients treated with ETN for whom the month and year of ETN initiation were available were included. Patterns of ETN and methotrexate (MTX) use were categorized as follows: combination therapy (ETN and MTX started concurrently), step-up therapy (MTX started first and ETN added later), switchers (MTX started and then stopped when or before ETN started), MTX add-on (ETN started first and MTX added later), and ETN only (no MTX use). Data were described using parametric and non-parametric statistics as appropriate.ResultsTwo thousand thirty-two of the five thousand six hundred forty-one patients with JIA met inclusion criteria (74% female, median age at diagnosis 6.0 years [interquartile range 2.0, 11.0]. Most patients (66.9%) were treated with a non-biologic disease modifying anti-rheumatic drug (DMARD), primarily MTX, prior to ETN. There was significant variability in patterns of MTX use prior to starting ETN. Step-up therapy was the most common approach. Only 34.0% of persistent oligoarticular JIA patients continued treatment with a non-biologic DMARD 3 months or more after ETN initiation. ETN persistence overall was 66.3, 49.4, and 37.3% at 24, 36 and 48 months respectively. ETN persistence among spondyloarthritis patients (enthesitis related arthritis and psoriatic JIA) varied by MTX initiation pattern, with higher ETN persistence rates in those who initiated combination therapy (68.9%) and switchers/ETN only (73.3%) patients compared to step-up (65.4%) and MTX add-on (51.1%) therapy.ConclusionThis study characterizes contemporary patterns of ETN use in the CARRA Registry. Treatment was largely in keeping with American College of Rheumatology guidelines.

Comparison of paediatric and adult classification criteria in juvenile idiopathic arthritis during the transition from paediatric to adult care.

To determine the characteristics of juvenile idiopathic arthritis (JIA) patients seen during the transition period in order to compare paediatric classification criteria with those for adults. Patients with JIA according to the ILAR classification and who had a consultation at transition between 2010 and 2017 were included in a retrospective bi-centre (Lyon, Lausanne) study. JIA classification criteria were compared to ACR/EULAR 2010 criteria for rheumatoid arthritis (RA), Yamaguchi criteria for adult-onset Still's disease (AOSD), ASAS criteria for spondyloarthritis and CASPAR criteria for psoriatic arthritis. One hundred and thirty patients were included: 13.9% with systemic JIA, 22.3% with polyarticular JIA, 22.3% with oligoarticular JIA, 34.6% with enthesitis-related arthritis (ERA) and 6.9% with psoriatic arthritis; 13.1% had suffered from uveitis; 14.5% of patients had erosions or carpitis, mainly those with psoriatic arthritis, polyarticular or systemic JIA; 37.5% of patients with ERA displayed radiological sacroiliitis. When comparing paediatric JIA criteria with adult classifications, we found that: 66.6% of patients with systemic JIA fulfilled the criteria for AOSD, 87.5% of rheumatoid factor-positive polyarticular JIA and 9.5% of rheumatoid factor-negative polyarticular JIA met the criteria for RA, and 34.5% of oligoarticular JIA fulfilled the criteria for spondyloarthritis. Finally, 77.7% of patients with ERA met the criteria for spondyloarthritis, and 100% of patients with psoriatic arthritis JIA met the criteria for psoriatic arthritis. Oligoarticular JIA and rheumatoid factor-negative polyarticular JIA seem to be paediatric entities, whereas the other types of JIA tended to meet the respective adult classification criteria.

Characteristics of coexisting localized scleroderma and inflammatory arthritis.

Localized scleroderma (LS), including morphea and linear scleroderma, is an autoimmune disease where excessive subcutaneous collagen deposits lead to thickening, scarring, and fibrosis of the tissues. LS coexisting with inflammatory arthritis is less well-described but has been reported in as many as 20% of 53 LS patients in a recent cohort. Herein, we describe a cohort of 8 children with both LS and inflammatory arthritis. The objective of this study is to determine the characteristics of inflammatory arthritis in children with LS and their response to treatment regimens. A retrospective chart review was completed on patients less than 19 years of age who were diagnosed with either morphea or linear scleroderma at the Children of Alabama center from 2004-2018. Patients were identified using ICD-9 and ICD-10 diagnostic codes. Records were reviewed for additional diagnostic codes, exams, and laboratory findings confirming coexisting inflammatory arthritis and LS. A total of 87 patients with a diagnosis of either morphea or linear scleroderma were identified. Eight (9%) had coexisting inflammatory arthritis according to the diagnostic codes with documented active arthritis. Median age of initial rheumatic disease diagnosis was 7.5 years. A majority of patients with both LS and inflammatory arthritis were female (62.5%). Half of the patients (n=4, 50%) had LS lesions over arthritic joints. All of the identified patients were diagnosed with a form of juvenile idiopathic arthritis (JIA). The JIA diagnoses varied widely in 3 (37.5%) patients with rheumatoid factor (RF) negative polyarticular JIA, 2 (25%) with oligoarticular JIA, 2 (25%) with psoriatic JIA, and 1 (12.5%) with enthesitis-related JIA. The timing of onset of LS and inflammatory arthritis varied widely. Three (37.5%) patients had LS lesions preceding clinical arthritis, and three (37.5%) had arthritis before the appearance of LS. Two (25%) patients had both LS and arthritis at the time of diagnosis. All patients received methotrexate (MTX) during their disease course with only 3 (37.5%) receiving systemic steroids during treatment. All 8 patients showed resolution of LS lesions. However, 6 of the 8 patients demonstrated active arthritis on combination MTX and TNFi therapy. In this cohort of pediatric LS, 9% of patients had coexisting inflammatory arthritis. The characteristics of this cohort varied widely. All patients received MTX initially and showed a resolution of LS lesions. However, in the majority of patients, the arthritis failed to respond to MTX and TNFi combination therapy. These results suggest that inflammatory arthritis coexisting with LS may be less likely to respond to traditional inflammatory arthritis or JIA therapies.

P08 Beliefs about pain in juvenile idiopathic arthritis are significantly associated with higher reported pain and more negative affect in children and young people

Abstract Background Persistent, unpredictable pain is one of the most burdensome features of juvenile idiopathic arthritis (JIA) which is a relapsing-remitting inflammatory musculoskeletal condition presenting in children and young people under 16 years. Recent research into perceptions of illness in young people has found that conceptualisations of long-term disease are mainly about the most salient features and symptoms, not of the illness as a whole. Particularly in young people with JIA, perceptions of pain are central to the individuals’ beliefs about the chronic condition. In paediatric pain research in general, the study of pain beliefs has mainly focused on pain catastrophising. Associations between other potentially important pain beliefs and pain outcomes (e.g. reporting pain or well-being) have not been explored to date. The aim of this study was to explore a wide range of pain perceptions in young people with JIA and to investigate whether these pain beliefs were associated with pain severity or well-being. Methods Cross-sectional data from the largest UK cohort study of children with JIA were analysed (the Childhood Arthritis Prospective Study- CAPS). Participants aged over 11 years completed ‘The Pain Perceptions Questionnaire for Young People (PPQ–YP)’ which assesses pain beliefs corresponding to the Common Sense-Self Regulatory framework first proposed by Leventhal and colleagues in the 1980s. Self-reported pain severity scores were captured using a Visual Analogue Scale (VAS). Emotional well-being was assessed using the Positive and Negative Affect Scale (PANAS). Associations were explored using Spearman’s Rank Correlation. Results 223 participants (64% female) completed the PPQ-YP. 38.2% of participants had a diagnosis of oligoarthritis, 29.6% had polyarthritis and equal proportions of participants had systemic, psoriatic or enthesitis related JIA (7.3%). Higher reported pain severity was significantly correlated with stronger beliefs about the greater impact of pain on life (rho=0.494, p < 0.001), pain unpredictability (rho=0.369, p < 0.001) and pain persistence (rho=0.278,p<0.001). Higher pain controllability beliefs (that pain was controllable) were significantly associated with lower self-reported pain severity (rho=-0.526, p < 0.001). Stronger beliefs about the impact of pain on life (rho=0.425,p=0.001) and pain coherence (poorer understanding about own pain: rho=-0.295,p=0.021) were significantly associated with more negative mood. Conclusion Pain beliefs (particularly about consequences, unpredictability, persistence and understanding) are linked to levels of pain severity and well-being in children with JIA. Adding to the evidence base on the link between pain catastrophising and illness outcomes, this research enhances our understanding of long-term illness and pain perceptions in young people through the implication of a wider range of pain beliefs and cognitions on illness outcomes. The PPQ-YP identified salient pain beliefs which should be considered in the design of potential interventions to improve pain outcomes in this group. Future research should aim to explore whether these perceptions can predict other important illness outcomes. Conflicts of Interest The authors declare no conflicts of interest.

Dermatological findings in common rheumatologic diseases in children

The aim of this study is to outline the common dermatological findings in pediatric rheumatologic diseases. A total of 45 patients, nineteen with juvenile idiopathic arthritis (JIA), eight with Familial Mediterranean Fever (FMF), six with scleroderma (SSc), seven with systemic lupus erythematosus (SLE), and five with dermatomyositis (DM) were included. Control group for JIA consisted of randomly chosen 19 healthy subjects of the same age and gender. The age, sex, duration of disease, site and type of lesions on skin, nails and scalp and systemic drug use were recorded. χ2 test was used. The most common skin findings in patients with psoriatic JIA were flexural psoriatic lesions, the most common nail findings were periungual desquamation and distal onycholysis, while the most common scalp findings were erythema and scaling. The most common skin finding in patients with oligoarthritis was photosensitivity, while the most common nail finding was periungual erythema, and the most common scalp findings were erythema and scaling. We saw urticarial rash, dermatographism, nail pitting and telogen effluvium in one patient with systemic arthritis; and photosensitivity, livedo reticularis and periungual erythema in another patient with RF-negative polyarthritis. Vascular skin lesions like RaynaudÂ’s phenomenon, livedo reticularis, palmar erythema, periungual telangiectasia and nailfold abnormalities were common in SLE, DM and SSc. Patients with FMF displayed signs of atopy. Specific skin lesions can be the peculiar features of rheumatologic diseases in pediatric population. Since it is not always easy to perform biopsy in children to confirm skin involvement of a rheumatologic disease, skin findings can help both dermatologists and rheumatologists in diagnosis.

Juvenile idiopathic arthritis in a center in the Western Anatolia region in Turkey.

Aim:To demonstrate the demographic data, subgroup distributions, responses to treatment and outcomes of long-term follow-up in patients who were followed up and treated in our clinics with a diagnosis of juvenile idiopathic arthritis, and to compare these data with national and international data.Material and Methods:The files of 116 patients who had been diagnosed as having juvenile idiopathic arthritis, were initiated on treatment and presented for regular follow-up visits between January 2012 and January 2018, were examined. Their demographic findings, treatments, active/inactive disease states (on-medication and off-medication) and treatment response states were evaluated.Results:According to the International League of Associations for Rheumatology criteria, the subtypes were specified as enthesitis-related arthritis (n=38), oligoarticular (n=37), rheumatoid factor (-) polyarticular (n=17), systemic (n=15), rheumatoid factor (+) polyarticular (n=5), and psoriatic juvenile idiopathic arthritis (n=4). In total, the female/male ratio was found to be 1.5. The mean delay time between the first complaint and the diagnosis was found to be 5.7±5.2 months. The patients with systemic type were diagnosed at the earliest, while the patients with polyarticular and enthesitis-related subtypes were diagnosed at the latest. Thirty-two percent of the patients were treated with methotrexate alone, and 38% were given additional biologic drugs. In both treatment groups, the time to achieve inactive disease was the shortest in the oligoarticular group and the longest in the enthesitis-related arthritis group. In the study period, 38 patients were in remission off-medication (the highest rate (53.3%) was observed in the systemic group) and 71 patients were in remission on-medication (the highest rate (70.2%) was observed in the oligoarticular group). Remission was obtained in 94% of the patients.Conclusion:Enthesitis which is the remarkable finding of enthesitis-related arthritis, should not be overlooked in routine physical examination. Awareness of enthesitis can contribute to the prevention of diagnostic delay in children with enthesitis-related arthritis.

Expressions and significance of Notch signal pathway and target gene in children with juvenile idiopathic arthritis

Objective To analyze the relationship between Notch signaling pathway and levels of lymphocytes and cytokines in children with juvenile idiopathic arthritis(JIA), and to explore its role in the pathogenesis of JIA. Methods Thirty-five pediatric patients with JIA [males 20 cases, females 15 cases; aged (6.5±4.0) years old, (0.83-15.00 years old)]and 15 healthy children [males 6 cases, females 9 cases; aged (5.0±2.9) years old, (1.0-11.0 years old)]from November 2015 to February 2016 in Guangzhou Women and Children′s Medical Center were included in the study. The JIA group were divided into the systemonset JIA(So-JIA) group (22 cases) and psoriatic JIA(p-JIA) group (13 cases, polyarthritis 7 cases and oligoarthritis 6 cases). The expressions of Notch signaling′s receptor, ligand and target gene mRNA in peripheral blood monouclear cells (PBMC) from the JIA group and the control group were determined by quantitative real-time PCR.The levels of cytokines interleukin (IL) -1β, IL-10, IL-6, IL-17, IL-4 and transforming growth factor-β (TGF-β) were detected by enzyme-linked immunosorbent assay. Results Compared with the healthy control group, the Notch2 receptors (1.6±3.2 vs. 0.4±0.3) expression level, Jagged1 ligand (44.0±79.0 vs. 11.3±1.2) expression levels and the levels of target gene HES1(0.4±0.3 vs. 0.1±0.1) mRNA in the JIA group showed a significant increase, and the differences were all statistically significant (all P<0.05 ). Compared with the healthy control group, the JIA group showed an increased level of IL-1β [(182.22±309.13) ng/L vs. (54.71±20.33) ng/L], IL-10 [(32.99±34.28) ng/L vs. (22.68±4.56) ng/L], IL-6 [(100.48±305.57) ng/L vs. (13.98±2.78) ng/L], IL-17 [(9.11±17.57) ng/L vs. (2.42±0.29) ng/L] and TGF-β [(14.37±9.33) ng/L vs. (5.49±4.49) ng/L], and there were statistically significant differences (all P<0.05) .The expression level of HES1 mRNA was positively correlated with STAT3 mRNA in the So-JIA group (r=0.573, P<0.05). The expression level of HES1 mRNA was positively correlated with CD3+ T(r=0.528), CD19+B(r=0.480), CD3+ CD4+ TH(r=0.457) and CD16+CD56+NK(r=0.598) cell absolute count in the So-JIA group (all P<0.05). The expression level of HES1 mRNA was positively correlated with CD3+ T cell absolute count in the p-JIA group(r=0.577, P<0.05). Conclusion Imbalance between Notch pathway and lymphocytes and cytokines in children with JIA may play an important role in the pathogenesis of JIA. Key words: Juvenile idiopathic arthritis; Notch pathway; Lymphocytes; Cytokines

A Family History of Psoriasis in a First-degree Relative in Children with JIA: to Include or Exclude?

To determine the consequences of disregarding first-degree relatives with psoriasis (FRP) as a classification criterion in juvenile idiopathic arthritis (JIA). Criteria were examined in children from a prospective cohort with unclassified and psoriatic JIA. FRP was the most common reason children were unclassified (57/85, 67%); all 57 children could be classified if FRP were disregarded as an exclusion criterion. FRP was a necessary inclusion criterion to classify 11/77 (14.3%) children with psoriatic JIA. Eliminating FRP as an exclusion criterion, but keeping it as an inclusion criterion in psoriatic JIA simplifies classification, though it is unclear whether the resulting classification would be better.

Implementation of musculoskeletal ultrasonography in detection of early juvenile idiopathic arthritis

AimTo determine whether early clinical, laboratory and musculoskeletal ultrasound (MSUS) characteristics can be used as early detectors of juvenile idiopathic arthritis. Patients and methodsForty (40) patients with juvenile idiopathic arthritis (JIA) diagnosed according to the ILAR criteria [1] and 20 healthy control children. All patients were subjected to the following assessment at base line and at follow up after 6 months: Clinical evaluation, MSUS examination and laboratory evaluation. ResultsOf the 40 patients, 6 patients (15%) had systemic onset subtype, 8 (20%) oligoarticular extended, 9 (22.5%) oligoarticular persistent, 5 (12.5%) polyarticular rheumatoid factor (RF) +ve, 6 (15%) polyarticular RF −ve, 5 (12.5%) enthesitis related subtype and only one patient (2.5%) had psoriatic JIA. MSUS detected more synovitis than clinical examination (subclinical synovitis) both at base line and at follow up. MSUS is highly sensitive for early detection of joint involvement in JIA when compared to physical examination. Significant decrease in the mean cartilage thickness of the patients measured at follow up as compared to measures at base line. ConclusionMSUS is highly sensitive for early detection of joint involvement in JIA when compared to physical examination

Enthesitis as a component of dactylitis in psoriatic juvenile idiopathic arthritis: histology of an established clinical entity

ContextImaging of dactylitis in adult psoriatic arthritis suggests a pathophysiological role for enthesitis. However, histological definition of the dactylitic digit is unavailable.ObjectiveWe evaluated the role of enthesitis in dactylitis associated with psoriatic juvenile idiopathic arthritis (psJIA) in a child who underwent detailed imaging and histologic evaluation.DesignRadiographs, ultrasound and high-resolution magnetic resonance imaging were employed to define the locus of disease in two dactylitic digits from a 14-year-old girl with psJIA. Biopsies were stained with hematoxylin and eosin, CD3, CD20, CD4, CD8 and CD117 and examined via light microscopy.ResultsRadiologic features of dactylitis included enhanced signal at digital entheses without accompanying synovitis or tenosynovitis. Histologically, finger and toe tissue exhibited hypervascular tenosynovium with a fibromyxoid expansion of fibrous tissue. This was accompanied by sparse to moderate perivascular lymphocytic inflammation consisting predominantly of T cells, with occasional admixed B cells and mast cells. Neutrophils and plasma cells were absent. Fibrocartilage exhibited reactive features including increased extracellular myxoid matrix, binucleation, and focal necrosis, without cellular inflammation. Fibrinous synovitis and mild reactive synoviocyte hyperplasia were seen.ConclusionsDactylitis in psJIA bears a radiographic resemblance to the condition in adult psoriatic arthritis. Histologic hallmarks include an expanded mast cell-populated extracellular myxoid matrix, cartilage degeneration, and a T cell-rich perivascular inflammatory infiltrate. These findings help to define enthesitis as a clinicopathologic entity.Electronic supplementary materialThe online version of this article (doi:10.1186/s12969-015-0003-2) contains supplementary material, which is available to authorized users.

Clinical features and therapy of rheumatic diseases and vasculitides in childhood

Rheumatic diseases are the second most common autoimmune disease in childhood after diabetes mellitus. Because cutaneous findings are so common, a dermatologist is often the first physician these children and their parents see. The most common rheumatic disease is juvenile idiopathic arthritis. Except for the systemic form and psoriatic arthritis JIA usually does not show cutaneous symptoms. However, there are many rheumatic diseases that start with skin manifestations such as Henoch-Schönlein purpura, systemic lupus erythematosus and Kawasaki syndrome. Knowledge of these diseases and their symptoms is therefore of importance for the dermatologist.

Treatment of juvenile idiopathic arthritis: a revolution in care

A generation ago, children with arthritis faced a lifetime of pain and disability. Today, there are a multitude of treatment options, including a variety of biologics targeting key cytokines and other inflammatory mediators. While non-steroidal anti-inflammatory drugs and corticosteroids were once the mainstay of therapy, they are now largely used as bridge or adjunctive therapies. Among the conventional disease-modifying anti-rheumatic drugs, methotrexate remains first-line therapy for most children with juvenile idiopathic arthritis (JIA) due to its long track record of safety and effectiveness in the management of peripheral arthritis. Sulfasalazine and leflunomide may also have a secondary role. The tumor necrosis factor inhibitors (TNFi) have shown tremendous benefit in children with polyarticular JIA and likely in enthesitis-related arthritis and psoriatic JIA as well. There may be additional benefit in combining TNFi with methotrexate. Abatacept and tocilizumab also appear to benefit polyarticular JIA; the role of rituximab remains unclear. For the treatment of systemic JIA, while the TNFi are of less benefit, blockade of interleukin-1 or interleukin-6 is highly effective. Additionally, interleukin-1 blockade appears to be effective treatment of macrophage activation syndrome, one of the most dangerous complications of JIA; specifically, anakinra in combination with cyclosporine and corticosteroids may obviate the need for cytotoxic approaches. In contrast, methotrexate along with the TNFi and abatacept are effective agents for the management of uveitis, another complication of JIA. Overall, the biologics have demonstrated an impressive safety record in children with JIA, although children do need to be monitored for rare but potentially dangerous adverse events, such as tuberculosis and other infections; paradoxical development of additional autoimmune diseases; and possibly an increased risk of malignancy. Finally, there may be a window of opportunity during which children with JIA will demonstrate most optimal responses to aggressive therapy, underscoring the need for rapid diagnosis and initiation of treatment.

AB1129 Radiological cervical spine involvement in juvenile idiopathic arthritis in adulthood

BackgroundSeveral studies have assessed cervical spine structural involvement in juvenile idiopathic arthritis (JIA) but most of them included only symptomatic patients without differentiating subsets of JIA.ObjectivesTo evaluate the frequency and...