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Purpose To assess the long-term outcome of uveitis in juvenile idiopathic arthritis (JIA). Design Population-based, multicenter, prospective JIA cohort, with a cross-sectional assessment of JIA-associated uveitis (JIA-U) 18 years after the onset of JIA. Participants A total of 434 patients with JIA, of whom 96 had uveitis, from defined geographic areas of Denmark, Finland, Norway, and Sweden. Methods 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. Main Outcome Measures 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. Results 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) Conclusions 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.

ObjectiveDistinction on clinical grounds between acute lymphoblastic leukaemia presenting with arthropathy (ALLarthropathy) and juvenile idiopathic arthritis (JIA) is difficult, as the clinical and paraclinical signs of leukaemia may be vague. The primary aim was to examine the use of lectin complement pathway proteins as markers to differentiate ALLarthropathy from JIA. The secondary aims were to compare the protein levels at baseline and follow-up in a paired number of children with ALL and to examine the correlation with haematology counts, erythrocyte sedimentation reaction (ESR), C-reactive protein (CRP), blasts, relapse and death.Study designIn this observational study, we measured M-ficolin, CL-K1 and MASP-3 in serum from children with ALL (n=151) and JIA (n=238) by time-resolved immunofluorometric assays. Logistic regression was used for predictions of ALL risk, considering the markers as the respective exposures. We performed internal validation using repeated ‘10-fold cross-validation’ with 100 repetitions computing the area under the curve (AUC) as well as positive and negative predictive values in order to evaluate the predictive performance.ResultsThe level of M-ficolin was higher in JIA than ALLtotal and the ALLarthropathy subgroup. The M-ficolin level normalised after remission of ALL. M-ficolin could differentiate ALL from JIA with an AUC of 94% and positive predictive value (PPV) of 95%, exceeding CRP and haemoglobin. In a dichotomised predictive model with optimal cut-offs for M-ficolin, platelets and haemoglobin, AUC was 99% and PPV 98% in detecting ALL from JIA.ConclusionM-ficolin is a valuable marker to differentiate the child with ALL from JIA.

Background Children and adolescents with juvenile idiopathic arthritis (JIA) may suffer pain from temporomandibular disorder (TMD). Still, routines for the assessment of temporomandibular joint (TMJ) pain in health and dental care are lacking. The aims of this study were to examine the prevalence of TMD in children and adolescents with JIA compared to their healthy peers and to investigate potential associations between JIA and TMD. Methods This comparative cross-sectional study is part of a longitudinal multicentre study performed during 2015–2020, including 228 children and adolescents aged 4–16 years with a diagnosis of JIA according to the ILAR criteria. This particular substudy draws on a subset of data from the first study visit, including assessments of TMD as part of a broader oral health examination. Children and adolescents with JIA were matched with healthy controls according to gender, age, and centre site. Five calibrated examiners performed the clinical oral examinations according to a standardised protocol, including shortened versions of the diagnostic criteria for TMD (DC/TMD) and the TMJaw Recommendations for Clinical TMJ Assessment in Patients Diagnosed with JIA. Symptoms were recorded and followed by a clinical examination assessing the masticatory muscles and TMJs. Results In our cohort of 221 participants with JIA and 221 healthy controls, 88 (39.8%) participants with JIA and 25 (11.3%) healthy controls presented with TMD based on symptoms and clinical signs. Painful TMD during the last 30 days was reported in 59 (26.7%) participants with JIA vs. 10 (5.0%) of the healthy controls (p Conclusion Symptoms and clinical signs of TMD were seen in approximately half of the JIA patients compared to about one fourth of their healthy peers. Painful palpation to masticatory muscles and decreased vertical unassisted jaw movement were more frequent in participants with JIA than among healthy controls and should be part of both medical and dental routine examinations in patients with JIA.

Objective To describe risk factors for IBD development in a cohort of children with JIA. Methods JIA patients who developed IBD were identified from the international Pharmachild register. Characteristics were compared between IBD and non-IBD patients and predictors of IBD were determined using multivariable logistic regression analysis. Incidence rates of IBD events on different DMARDs were calculated, and differences between therapies were expressed as relative risks (RR). Results Out of 8942 patients, 48 (0.54% ) developed IBD. These were more often male (47.9% vs 32.0%) and HLA-B27 positive (38.2% vs 21.0%) and older at JIA onset (median 8.94 vs 5.33 years) than patients without IBD development. They also had more often a family history of autoimmune disease (42.6% vs 24.4%) and enthesitis-related arthritis (39.6% vs 10.8%). The strongest predictors of IBD on multivariable analysis were enthesitis-related arthritis [odds ratio (OR): 3.68, 95% CI: 1.41, 9.40] and a family history of autoimmune disease (OR: 2.27, 95% CI: 1.12, 4.54). Compared with methotrexate monotherapy, the incidence of IBD on etanercept monotherapy (RR: 7.69, 95% CI: 1.99, 29.74), etanercept with methotrexate (RR: 5.70, 95% CI: 1.42, 22.77) and infliximab (RR: 7.61, 95% CI: 1.27, 45.57) therapy was significantly higher. Incidence on adalimumab was not significantly different (RR: 1.45, 95% CI: 0.15, 13.89). Conclusion IBD in JIA was associated with enthesitis-related arthritis and a family history of autoimmune disease. An increased IBD incidence was observed for etanercept therapy regardless of concomitant methotrexate use.

Background To determine the serum levels of the lectin pathway proteins early in the disease course and 17 years after disease onset and to correlate the protein levels to markers of disease activity in participants from a population-based Nordic juvenile idiopathic arthritis (JIA) cohort. Additionally, to assess the predictive value of lectin pathway proteins with respect to remission status. Methods A population-based cohort study of consecutive cases of JIA with a disease onset from 1997 to 2000 from defined geographical areas of Finland, Sweden, Norway and Denmark with 17 years of follow-up was performed. Clinical characteristics were registered and H-ficolin, M-ficolin, MASP-1, MASP-3, MBL and CL-K1 levels in serum were analyzed. Results In total, 293 patients with JIA were included (mean age 23.7 ± 4.4 years; mean follow-up 17.2 ± 1.7 years). Concentrations of the lectin protein levels in serum were higher at baseline compared to the levels 17 years after disease onset (p ≤ 0.006, n = 164). At baseline, the highest level of M-ficolin was observed in systemic JIA. Further, high M-ficolin levels at baseline and at 17-year follow-up were correlated to high levels of ESR. In contrast, high MASP-1 and MASP-3 tended to correlate to low ESR. CL-K1 showed a negative correlation to JADAS71 at baseline. None of the protein levels had prognostic abilities for remission status 17 years after disease onset. Conclusion We hypothesize that increased serum M-ficolin levels are associated with higher disease activity in JIA and further, the results indicate that MASP-1, MASP-3 and CL-K1 are markers of inflammation.

Background We aimed to examine psychosocial function, suicidality and school dropout in a clinical psychiatric population over a 3-year period from adolescence to young adulthood and explore associations with negative life events. Methods This study is part of the Health Survey in Department of Children and Youth, St. Olavs hospital, Norway. In the first study visit (T1), 717 (43.5% of eligible) participated, aged 13–18 years (2009–2011), and 3 years later (T2), 570 answered a questionnaire (school functioning and negative life events), and 549 completed Kiddie SADS as telephone interview assessing DSM-IV diagnoses, psychosocial functioning and suicidality. Results Suicidal ideation was more frequent among girls (17.9%) than among boys (5.4%) (risk difference; RD = 12.5%, CI (7.2 to 17.7), p p p p = 0.006). For those with a psychiatric disorder, 24.8% of girls had suicidal ideation and 30.0% suicidal behavior, which was larger than for boys (RD = 18.0%, CI (10.8 to 24.7), p p p p p p p = 0.011). Conclusions The high frequency of suicidality and school dropout confirms the severity of adolescent psychiatric disorders, especially among girls. Specific life events were associated risk factors and should be target points for prevention and intervention.

Background We aimed to examine symptom load in a clinical adolescent population at three-year follow-up and explore associations with standard care treatment procedures and resilience factors upon first presenting at Child and Adolescent Mental Health Services. Methods This study is part of a prospective longitudinal cohort study: The Health Survey in Department of Children and Youth, St. Olavs hospital, Norway. A clinical population of 717 (43.5% of eligible) adolescents aged 13–18 years participated in the first study visit (T1, 2009–2011). Of these, 447 adolescents with psychiatric disorders, with treatment history from medical records and self-reported resilience factors (Resilience Scale for Adolescents; READ) at T1, reported symptom load (Achenbach System of Empirically Based Assessment - Youth Self Report; YSR) three years later aged 16–21 years (T2). Result At T1, 93.0% received individual treatment. The frequency of psychotherapy and medication varied by disorder group and between genders. Overall, psychotherapy was more frequent among girls, whereas medication was more common among boys. Total READ mean value (overall 3.5, SD 0.8), ranged from patients with mood disorders (3.0, SD 0.7) to patients with Attention Deficit Hyperactivity disorder (3.7, SD 0.7), and was lower for girls than boys in all diagnostic groups. At T2, the YSR Total Problem mean T-score ranged across the diagnostic groups (48.7, SD 24.0 to 62.7, SD 30.2), with highest symptom scores for those with mood disorders at T1, of whom 48.6% had T-scores in the borderline/clinical range (≥60) three years later. Number of psychotherapy sessions was positively associated and Total READ score was negatively associated with the YSR Total Problems T-score (regression coefficient β = 0.5, CI (0.3 to 0.7), p Conclusions Self-reported symptom load was substantial after three years, despite comprehensive treatment procedures. Higher self-reported resilience characteristics were associated with lower symptom load after three years. These results highlight the burden of adolescent psychiatric disorders, the need for extensive interventions and the importance of resilience factors for a positive outcome.

Background To study fatigue in young adults with juvenile idiopathic arthritis (JIA) 18 years after disease onset, and to compare with controls. Methods Consecutive children with onset of JIA between 1997 and 2000, from geographically defined areas of Norway, Sweden, Denmark and Finland were followed for 18 years in a close to population-based prospective cohort study. Clinical features, demographic and patient-reported data were collected. Inclusion criteria in the present study were a baseline visit 6 months after disease onset, followed by an 18-year follow-up with available self-reported fatigue score (Fatigue Severity Scale (FSS), 1–7). Severe fatigue was defined as FSS ≥4. For comparison, Norwegian age and sex matched controls were used. Results Among 377 young adults with JIA, 26% reported severe fatigue, compared to 12% among controls. We found higher burden of fatigue among participants with sleep problems, pain, poor health, reduced participation in school/work, physical disability, active disease, or use of disease-modifying anti-rheumatic drugs (DMARDs)/biologics/systemic steroids. In contrast, participants without these challenges, had fatigue scores similar to controls. Active disease assessed at all three time points (baseline, 8-year and 18-year follow-up) was associated with higher mean fatigue score and higher percentage of severe fatigue compared to disease courses characterized by periods of inactive disease. Predictors of fatigue at the 18-year follow-up were female sex and diagnostic delay of ≥6 months at baseline, and also pain, self-reported poor health, active disease, and previous/ongoing use of DMARDs/biologics at 8 years. Conclusions Fatigue is a prominent symptom in young adults with JIA, with higher fatigue burden among participants with poor sleep, pain, self-reported health problems, active disease, or use of DMARDs/biologics. Participants without these challenges have results similar to controls. Patient- and physician-reported variables at baseline and during disease course predicted fatigue at 18-year follow-up.

Background Optimal utilization of dental caries data is crucial in epidemiological research of individuals with juvenile idiopathic arthritis (JIA). The aims were to: explore whether caries is more prevalent among children and adolescents with JIA compared to controls; examine presence of caries according to JIA group, socio-behavioral and intraoral characteristics, and the extent to which surface-specific caries varies between and within individuals; assess whether surface-specific caries varies according to JIA group and dentition; and investigate whether disease-specific clinical features of JIA are associated with presence of caries. Methods In this comparative cross-sectional study, calibrated dentists examined index teeth (primary 2. molars, 1. permanent molars) of 4–16-year-olds with JIA (n = 219) and matched controls (n = 224), using a detailed caries diagnosis system (including enamel caries). JIA-specific characteristics were assessed by pediatric rheumatologists and socio-behavioral information collected by questionnaires. Multilevel mixed-effect logistic regressions reporting odds ratios (OR) with 95% confidence interval (CI) were applied (caries at surface level as outcome variable). Potential confounders were adjusted for, and the effect of dependency of surface-specific caries data was estimated by calculating intra-class correlation coefficients (ICC). Results At individual level, no significant difference in caries prevalence was found between individuals with JIA and controls, regardless of inclusion of enamel caries. Proportion of enamel lesions exceeded dentine lesions. JIA was not associated with presence of caries, but in both groups, low maternal educational level was associated with presence of caries (OR: 2.07, 95% CI: 1.24–3.46). Occlusal and mesial surfaces, compared to buccal surfaces, had generally higher OR according to presence of caries than distal and lingual surfaces (ICC = 0.56). Surface-specific caries in the permanent dentition differed significantly according to group affiliation. Some JIA disease-specific variables were suggested to associate with presence of caries. Conclusions No overall difference in caries prevalence between individuals with JIA and controls was observed, but for both groups, low maternal educational level and tooth surface associated with presence of caries. Associations between JIA disease-specific variables and presence of caries cannot be excluded. Due to predominance of enamel lesions, the potential of preventative dental strategies is considerable.

Background Observational studies examining the association between oral health and juvenile idiopathic arthritis (JIA) among children and adolescents have reported inconsistent findings. The aims of this systematic review and meta-analysis were to ascertain a potential difference in oral health and oral health-related quality of life (OHRQoL) among children and adolescents with JIA and healthy peers, and to assess the association of prevalence of oral diseases/conditions, temporomandibular disorders (TMD), including temporomandibular joint (TMJ) diseases, in relation to activity and severity of JIA. Method Medline Ovid, Embase, CINAHL, SweMed+ and Cochrane Library were searched up to 25 November 2018. All articles published in English, German and Scandinavian languages focusing on children and adolescents with JIA and without JIA in relation to oral health measures, were considered. Two authors independently evaluated observational studies for inclusion. The study quality was assessed using modified Newcastle Ottawa Scale. Meta-analysis was performed for studies focusing on dental caries as an outcome. Results Nineteen articles met the inclusion criteria, covering a range of oral diseases/conditions and OHRQoL. Eighteen studies had cross-sectional design. No mean difference of dmft/DMFT indices (decayed/missed/filled teeth) was observed between the JIA - and healthy group. None of the oral health measures including dental erosive wear, enamel defects, dental maturation and OHRQoL, indicated better oral health among children and adolescents with JIA compared to healthy group. However, periodontal conditions and TMD were more predominant among children and adolescents with JIA compared to healthy peers. Conclusions Based on the cross-sectional studies, periodontal diseases and TMD were found to be more frequent in children and adolescents with JIA compared to healthy peers. Furthermore, more high-quality studies with large sample size are needed before we infer any concrete conclusion regarding the association between the prevalence of oral and TMJ diseases or oral conditions in relation to activity and severity of JIA.

This is a pre-copyediting, author-produced PDF of an article accepted for publication in The Journal of Rheumatology following peer review. The definitive publisher-authenticated version Hetlevik, S.O., Flatø, B., Aaløkken, T.M., Lund, M.B., Reiseter, S., Mynarek, G.K. ... Lilleby, V. (2019). Pulmonary Manifestations and Progression of Lung Disease in Juvenile-onset Mixed Connective Tissue Disease. The Journal of Rheumatology, 46(1), 93-100, is available online at: https://doi.org/10.3899/jrheum.180019. Objective - To assess the occurrence and extent of interstitial lung disease (ILD) in patients with juvenile mixed connective tissue disease (JMCTD), compare pulmonary function in patients and matched controls, study associations between ILD and disease-related variables, and examine progression of pulmonary manifestations over time. Methods - A cohort of 52 patients with JMCTD were examined in a cross-sectional study after a mean 16.2 (SD 10.3) years of disease duration with high-resolution computed tomography (HRCT) and pulmonary function tests (PFT) comprising spirometry, DLCO, and total lung capacity (TLC). Matched controls were examined with PFT. Previous HRCT and PFT were available in 37 and 38 patients (mean 8.8 and 10.3 yrs before study inclusion), respectively. Results - Compared to controls, patients with JMCTD had lower forced vital capacity (FVC), DLCO, and TLC (p < 0.01). The most frequent abnormal PFT was DLCO in 67% of patients versus 17% of controls (p < 0.001). Fourteen patients (27%) had ILD on HRCT. Most had ILD in < 10% of their lungs. ILD was associated with low values for FVC and TLC, but not with DLCO. HRCT findings did not progress significantly over time, but FVC declined (p < 0.01). Conclusion - Compared to controls, patients with JMCTD had impaired pulmonary function. ILD was present in 27% of patients after a mean 16 years of disease duration, mostly as mild disease, and did not progress. ILD seems to be less common in juvenile-onset than in adult-onset MCTD, and ILD in JMCTD seems mostly mild and stable over time.

Objectives. To evaluate the demographic, disease activity, disability and health-related quality of life (HRQoL) differences between children with juvenile idiopathic arthritis (JIA) and their healthy peers, and between children with JIA with and without clinical temporomandibular joint (TMJ) involvement and its determinants. Methods. This study is based on a cross-sectional cohort of 3343 children with JIA and 3409 healthy peers, enrolled in the Pediatric Rheumatology International Trials Organisation (PRINTO) health-related quality of life (HRQoL) study or in the methotrexate trial. Potential determinants of TMJ involvement included demographic, disease activity, disability and HRQoL measures selected through univariate and multivariable logistic regression. Results. Clinical TMJ involvement was observed in 387/3343 (11.6%) JIA children. Children with TMJ involvement, compared to those without, more often had polyarticular disease course (95% versus 70%), higher Juvenile Arthritis Disease Activity Score (JADAS) (odds ratio (OR) 4.6), more disability and lower HRQoL. Children with TMJ involvement experienced clearly more disability and lower HRQoL compared to their healthy peers. The multivariable analysis, showed that cervical spine involvement (OR 4.6), disease duration > 4.4 years (OR 2.8), and having more disability (Childhood Health Assessment Questionnaire Disability Index >0.625) (OR 1.6) were the most important determinants for TMJ involvement. Conclusion. Clinical TMJ involvement in JIA is associated with higher disease activity, higher disability and impaired HRQoL. Our findings indicate the need for dedicated clinical and imaging evaluation of TMJ arthritis especially in children with cervical spine involvement, polyarticular course and longer disease duration. This article is protected by copyright. All rights reserved.

Supplemental Digital Content is available in the text., Objective: To investigate self-reported pain in young adults with a low birth weight. Materials and Methods: This study was a part of a long-term follow-up study of preterm very low birth weight (VLBW; birth weight ≤1500 g), term small for gestational age (SGA; birth weight 6 months and being moderate, severe, or very severe during the past 4 weeks. Results: The prevalence of chronic pain at 26 years was 16% in the VLBW group, 21% in the term SGA group, and 7% in the control group. The VLBW and the term SGA groups had higher odds ratios for chronic pain (crude OR, 2.6; 95% CI, 0.9-7.6 for the VLBW group and crude OR, 3.6; 95% CI, 1.3-9.9 for the term SGA group vs. controls). The main results remained after adjusting for potential confounding factors. Some attenuation was observed when adjusting for anxiety and depressive problems. Moderate to very severe pain increased from 16% to 41% in the term SGA group from 19 to 26 years, whereas less changes were seen in the VLBW and the control groups. Discussion: Results of our study imply that pain should be in focus when conducting long-term follow-up programs of individuals with a low birth weight.

To test the hypothesis that non-HLA single-nucleotide polymorphisms (SNPs) associated with the risk of juvenile idiopathic arthritis (JIA) are risk factors for an unfavourable disease outcome at long-term follow-up.The Nordic JIA cohort is a prospective multicentre study cohort of patients from the Nordic countries. In all, 193 patients met the inclusion criteria of having an 8 year follow-up assessment and available DNA sample. Seventeen SNPs met the inclusion criteria of having significant associations with JIA in at least two previous independent study cohorts. Clinical endpoints were disease remission, actively inflamed joints and joints with limitation of motion (LOM), articular or extra-articular damage, and history of uveitis.Evidence of associations between genotypes and endpoints were found for STAT4, ADAD1-IL2-IL21, PTPN2, and VTCN1 (p = 0.003-0.05). STAT4_rs7574865 TT was associated with the presence of actively inflamed joints [odds ratio (OR) 20.6, 95% confidence interval (CI) 2.2- 100, p = 0.003] and extra-articular damage (OR 7.9, 95% CI 1-56.6, p = 0.057). ADAD1_rs17388568 AA was associated with a lower risk of having joints with LOM (OR 0.1, 95% CI 0-0.55, p = 0.016). PTPN2_rs1893217 CC was associated with a lower risk of having joints with LOM (OR 0.2, 95% CI 0-0.99, p = 0.026), while VTCN1_rs2358820 GA was associated with uveitis (OR 3.5, 95% CI 1-12.1, p = 0.029).This exploratory study, using a prospectively followed JIA cohort, found significant associations between long-term outcome and SNPs, all previously associated with development of JIA and involved in immune regulation and signal transduction in immune cells.

This is a pre-copyediting, author-produced PDF of an article accepted for publication in The Journal of Rheumatology following peer review. The definitive publisher-authenticated version Glerup, M., Stoustrup, P., Matzen, L.H., Rypdal, V., Nordal, E., Frid, P. ... Klit Pedersen, T. (2020). Longterm Outcomes of Temporomandibular Joints in Juvenile Idiopathic Arthritis: 17 Years of Followup of a Nordic Juvenile Idiopathic Arthritis Cohort is available online at: https://doi.org/10.3899/jrheum.190231 in The Journal of Rheumatology, 47(5), 730-738: https://www.jrheum.org/. Objective - To determine the prevalence of orofacial symptoms, dysfunctions, and deformities of the temporomandibular joint (TMJ) in juvenile idiopathic arthritis (JIA) 17 years after disease onset. Methods - Drawn from a prospective, population-based Nordic JIA cohort with disease onset from 1997 to 2000, 420 consecutive cases were eligible for orofacial evaluation of TMJ involvement. The followup visit included demographic data, a standardized clinical orofacial examination, and full-face cone-beam computed tomography (CBCT). For comparison, 200 age-matched healthy controls were used. Results - Of 420 eligible participants with JIA, 265 (63%) were included (mean age 23.5 ± 4.2 yrs) and completed a standardized clinical orofacial examination. Of these, 245 had a full-face CBCT performed. At least 1 orofacial symptom was reported by 33%. Compared to controls, the JIA group significantly more often reported TMJ pain, TMJ morning stiffness, and limitation on chewing. Further, among participants reporting complaints, the number of symptoms was also higher in JIA. The mean maximal incisal opening was lower in the JIA group (p < 0.001), and TMJ pain on palpation was more frequent. Condylar deformities and/or erosions were observed in 61% as assessed by CBCT, showing bilateral changes in about 70%. Risk factors of condylar deformities were orofacial dysfunction or biologic treatment; enthesitis-related arthritis was protective. Conclusion - This study of the longterm consequences of TMJ involvement in a population-based JIA cohort reports persistence of comprehensive symptoms, dysfunctions, and damage of the TMJ into adulthood. We suggest interdisciplinary followup of JIA patients also in adulthood.

Background Juvenile idiopathic arthritis (JIA) has a reported prevalence varying from 16 to 150 per 100,000 patients and it thereby is the most frequent chronic rheumatologic disease presenting in childhood. Inflammatory bowel disease (IBD) is an auto-inflammatory disease that can develop in patients with JIA. Results from multiple studies suggest an association between therapy with Etanercept (ETN) and occurrence of IBD in patients with JIA, which may or may not be counteracted by methotrexate. Objectives The aim of this study was to describe characteristics of JIA patients who developed IBD and to evaluate a possible relationship between IBD onset and medication use at IBD onset. Methods Pharmachild, the largest international JIA registry, was used for this study. Patients were enrolled via members of the Paediatric Rheumatology INternational Trials Organisation (PRINTO). The registry contains both retrospective and prospective data. Results A total of 8,309 patients were included in this study. 290 gastrointestinal disorders were reported in 260 patients. 50 cases in 47 patients were classified as IBD or suspected IBD. Age at JIA onset was significantly higher in patients who developed IBD (9.1 vs 7.1 years p=0.002), and female predominance was lower (48.9% versus 67.6% p=0.011). Enthesitis related arthritis (ERA) was the JIA subtype in 40.4% of the patients who developed IBD. In 14 out of 47 patients more detailed information about IBD disease onset was available, such as date of disease onset and medication used at that time. 71.4% of all 14 patients used etanercept and 50.0% used MTX 3 months prior or at IBD onset. Etanercept exposure prior to IBD onset varied from no exposure to 6.39 years with a median of 1.3 years. Methotrexate exposure prior to disease onset varied from no exposure to 8.10 years with a median of 2.9 years. Information regarding quality of life was available for 2,752 patients of which 17 IBD patients. Quality of life, both physical and psychosocial, was significantly lower in patients who developed IBD patients (p=0.018 and p=0.046 respectively). Conclusion In this study ERA patients were more at risk of developing IBD and 71.4% used etanercept while developing IBD. We did not find a protective role of MTX since 50.0% of patients with available data developed IBD while using MTX. Lastly, IBD has an important physical and psychosocial impact on quality of life. Reference [1] Swart JF; Giancane G; Horneff G; Magnusson B; Hofer M; Alexeeva E; Panaviene V; Bader-Meunier B; Anton J; Nielsen S; De Benedetti F; Kamphuis S; Stanevicha V; Trachana M; Ailioaie LM; Tsitsami E; Klein A; Minden K; Foeldvari I; Haas JP; Klotsche J; Horne. Pharmacovigilance in juvenile idiopathic arthritis patients treated with biologic or synthetic drugs: combined data of more than 15,000 patients from pharmachild and national registries. Arthritis Res Ther. 2018; Disclosure of Interests Roline Krol: None declared, Joost F. Swart: None declared, Gabriella Giancane: None declared, Sytze De Roock: None declared, Troels Herlin: None declared, Pavla Dolezalova: None declared, Helga Sanner: None declared, Gordana Susic: None declared, Flavio R. Sztajnbok: None declared, D Maritsi: None declared, Tamas Constantin: None declared, V Vargova: None declared, Sujata Sawhney: None declared, Marite Rygg: None declared, SHEILA KNUPP DE OLIVEIRA: None declared, Marco Cattalini: None declared, Ellen Nordal: None declared, Claudia Magalhaes: None declared, Alberto Martini Consultant for: I do not have any conflict of interest to declare since starting from 1 March 2016 I became the Scientific Director of the G. Gaslini Hospital; therefore, my role does not allow me to render private consultancies resulting in personal income. I perform consultancy activities on behalf of the Gaslini Institute for the companies listed below: AbbVie, Biogen, Boehringer Ingelheim, Bristol-Myers Squibb, EMD Serono, Janssen, Novartis, Pfizer, R-Pharm. The money received for these activities are directly transferred to the Gaslini Institute’s bank account. Before March 2016, I was the head of the Pediatric Rheumatology Department at the G. Gaslini Hospital, where the PRINTO Coordinating Centre is located. For the coordination activity of the PRINTO network, the Gaslini Hospital received contributions from the industries listed in this section. This money has been reinvested for the research activities of the hospital in fully independent manners besides any commitment with third parties., Nico Wulffraat: None declared, Nicolino Ruperto Grant/research support from: The Gaslini Hospital, where NR works as full-time public employee, has received contributions (> 10.000 USD each) from the following industries in the last 3 years: BMS, Eli-Lilly, GlaxoSmithKline, F Hoffmann-La Roche, Janssen, Novartis, Pfizer, Sobi. This funding has been reinvested for the research activities of the hospital in a fully independent manner, without any commitment with third parties., Consultant for: Received honoraria for consultancies or speaker bureaus (

Background A challenge with the present classification of JIA is the evolution of the disease over time. One category that is especially difficult to classify is enthesitis-related JIA (ERA). Objectives To longitudinally study radiologically diagnosed sacroiliitis (SI) developed during the first 18 years in an aim to gain knowledge about classification challenges posed by the proposed, new classification (Martini A. et al. J Rheumatol. 2018 Oct; Epub ahead of print). Methods 510 consecutive cases of JIA with disease onset 1997 to 2000 were prospectively included in a Nordic, longitudinal, close to population-based 18-year follow-up study, and 434 (85%) had at least two follow-up visits during disease course. At the 18-year follow-up visit; 329 (76%) attended a clinical visit, and 105 (24%) a telephone interview. The follow-up period was 17.5 ± 1.7 years (mean ± SD) after onset. Mean age of the study participants was 24.0 ± 4.4 years. Clinical data, collected at one, eight and 18 years after disease onset, were evaluated regarding variables for enthesitis/spondylitis-related arthritis compared to the other JIA categories. Results In 376 participants evaluated for SI, radiology was performed on clinical suspicion, 26 (16 males, 10 females) developed radiologically verified sacroiliitis (rad-SI) during the first 18 years of disease. Age at onset was significantly higher in this group compared to the other participants, median 9.9 (IQR 6.4-12.0) vs. 5.6 (IQR 2.6-9.5) years, (p=0.001). Only 3/26 had rad-SI at eight-year follow-up. Using the ILAR criteria 12/26 with rad-SI were classified as ERA after median 7 (IQR 6-10.2) months, 1/26 as juvenile psoriatic arthritis, 5/26 as undifferentiated JIA because of psoriasis-related variables, the remaining had oligo- or polyarticular arthritis. At 18-year follow-up, 18/26 fulfilled criteria for ERA, 2/26 juvenile psoriatic arthritis, and 3/26 the undifferentiated category because of psoriasis-related variables, 3/26 had polyarticular RF negative or oligoarticular extended disease. Enthesitis, inflammatory back pain and SI joint tenderness were more common with rad-SI (p Conclusion The majority of variables involved in the new proposed classification of enthesitis/spondylitis-related JIA were significantly more common during the first 18 years of disease in those that developed rad-SI. Psoriasis-related variables, no longer exclusion criteria for ERA, should be further evaluated as possible inclusion criteria, as well as higher age at onset. Disclosure of Interests Karin Qvarnstrom: None declared, Mia Glerup: None declared, Veronika Rypdal: None declared, Ellen Dalen Arnstad: None declared, Suvi Peltoniemi: None declared, Maria Ekelund: None declared, Anders Fasth: None declared, Susan Nielsen: None declared, Marek Zak: None declared, Kristiina Aalto: None declared, Ellen Nordal: None declared, Troels Herlin: None declared, Marite Rygg: None declared, Lillemor Berntson Consultant for: AbbVie, Speakers bureau: AbbVie

Background Fatigue is common in adults with rheumatic disease and has also been shown in adolescents with juvenile idiopathic arthritis (JIA). Knowledge on fatigue in JIA in long-term follow-up is limited. Objectives To study the prevalence and severity of fatigue 18 years after onset of JIA. Methods In this close to population-based cohort study from defined geographical areas of Norway, Sweden, Denmark and Finland, consecutive cases of JIA with disease onset in 1997 to 2000 were prospectively enrolled (1). At 18-year follow-up, fatigue was measured using Fatigue Severity Scale (FSS, range 0-7) (2), and severe fatigue was defined as FSS ≥4. General health status was measured with Health Assessment Questionnaire (HAQ) and 36-Item Short Form Health Survey (SF-36). Reduced health was defined as HAQ >0, and SF-36 0 = pain. Remission was defined according to the preliminary criteria described by Wallace. A Norwegian healthy cohort was used for comparison. Multivariable logistic regression analyses were performed. Results Among 434 eligible JIA participants 377 completed a Fatigue Severity Scale (FSS) measurement at the 18-year follow-up and were included. Of these 72% were girls, 53% had oligoarticular disease six months after onset, median age at onset was 5.6 (IQR 2.6-9.7) years, and age at the 18-year visit was 23.1 (IQR 20.3-27.2). Mean total FSS (±SD) was 3.2 (±1.5), and participants with active disease scored 3.6 (±1.6) compared to 2.9 (±1.4) for those in remission off medication. The highest total FSS was found in those with SF-36 PCS and/or MCS 0 (47%, OR 4.1) compared to HAQ score =0 (18%), SF-36 PCS/MCS 0 (36%, OR 3.8) compared to VAS pain =0 (13%). The proportion of severe fatigue in a healthy Norwegian control cohort was 12%. Conclusion At 18-year follow-up fatigue was a prominent symptom in JIA, and we found consistently higher fatigue burden and considerably more severe fatigue among participants with active disease, pain and self-reported health problems, compared to those without. We suggest fatigue to be measured at long-term follow-up both in clinical and research settings. References [1] Nordal E, et al. Arthritis Rheum 2011;63:2809-18 [2] Krupp LB, et al. Arch Neurol 1989;46:1121-23 Disclosure of Interests Ellen Dalen Arnstad: None declared, Mia Glerup: None declared, Veronika Rypdal: None declared, Suvi Peltoniemi: None declared, Maria Ekelund: None declared, Lillemor Berntson Consultant for: AbbVie, Speakers bureau: AbbVie, Anders Fasth: None declared, Susan Nielsen: None declared, Marek Zak: None declared, Kristiina Aalto: None declared, Ellen Nordal: None declared, Troels Herlin: None declared, Pal Richard Romundstad: None declared, Marite Rygg: None declared

Background Models predicting outcome in juvenile idiopathic arthritis (JIA) have recently been proposed by Guzman et al.1 and Rypdal et al.2 Guzman et al. constructed a model for predicting severe disease course derived from the ReACCh-Out study, and Rypdal et al. constructed models for prediction of non-remission, functional disability and joint damage. Objectives To validate methods for prediction of long-term outcome in JIA by testing the ability of Guzman’s model and Rypdal’s model to predict severe disease course (the ReACCh-Out outcome) in the Nordic cohort. Methods The Nordic cohort is a prospective longitudinal multicenter cohort from defined geographical areas of 4 Nordic countries. Children with a baseline and an 8-year study visit were included. Missing data were imputed using low rank matrix factorization3, and a K-medoids algorithm4 was used to identify clusters corresponding to severe disease course in the ReACCh-Out study. With this outcome, the prediction model of Guzman et al. was tested with no re-estimation of parameters. A Receiver operating characteristic (ROC) curve and the corresponding area under the curve (AUC) were computed. For the same outcome, prediction models were built using the method of Rypdal et al. on randomly sampled training sets, and tested on disjoint validation sets. Results In the Nordic cohort 98/440 (22%) patients were identified with a severe disease course. This ratio is similar to the 125/610 (20%) found in the ReACCh-Out study. Characteristics of groups of patients with severe and non-severe disease course are similar in the two cohorts. The model of Guzman et al. had an AUC of 0.85 for prediction of severe disease course and an AUC of 0.66 for predicting remission off medication. In repeated cross-validations, the model of Rypdal et al. had a median AUC of 0.90 (IQR 0.86-0.92) for prediction of severe disease course, and a median AUC of 0.78 (IQR 0.72–0.82) for remission off medication. Conclusion Tests in the Nordic cohort validate the ability of the model of Guzman et al. to predict severe disease course. Repeated cross-validations of the model of Rypdal et al. indicate that validation results are highly dependent of the chosen outcome, and that prediction of long-term remission status is more challenging than prediction of a severe disease course. References [1] Guzman, J. et al., Predicting Which Children with Juvenile Idiopathic Arthritis Will Have a Severe Disease Course: Results from the ReACCh-Out Cohort, J Rheumatol, 44, 230-240, 2017. [2] Rypdal, V. et al., Predicting unfavorable long-term outcome in juvenile idiopathic arthritis: results from the Nordic cohort study, Arthritis Res Ther, 20, 91, 2018. [3] Liu, G. et al., Robust subspace segmentation by low-rank representation, Proceedings of the 27th International Conference on International Conference on Machine Learning, 663-670, 2010. [4] Park, H.-S. et al., A simple and fast algorithm for K-medoids clustering, Expert Systems with Applications, 36, 3336-3341, 2009. Disclosure of Interests Veronika Rypdal: None declared, Jaime Guzman: None declared, Andrew Henrey: None declared, Thomas Loughin: None declared, Mia Glerup: None declared, Anders Fasth: None declared, Ellen Dalen Arnstad: None declared, Lillemor Berntson Consultant for: AbbVie, Speakers bureau: AbbVie, Susan Nielsen: None declared, Marek Zak: None declared, Marite Rygg: None declared, Kristiina Aalto: None declared, Troels Herlin: None declared, Martin Rypdal: None declared, Ellen Nordal: None declared

Background The aim of the study was to describe school attendance and participation in physical education in school among children with juvenile idiopathic arthritis (JIA). Methods Consecutive cases of JIA from defined geographical areas of Finland, Sweden and Norway with disease onset in 1997 to 2000 were followed for 8 years in a multi-center cohort study, aimed to be as close to population-based as possible. Clinical characteristics and information on school attendance and participation in physical education (PE) were registered. Results Participation in school and in PE was lowest initially and increased during the disease course. Eight years after disease onset 228/274 (83.2%) of the children reported no school absence due to JIA, while 16.8% reported absence during the last 2 months due to JIA. Full participation in PE was reported by 194/242 (80.2%), partly by 16.9%, and none by 2.9%. Lowest participation in PE was found among children with ERA and the undifferentiated categories. Absence in school and PE was associated with higher disease activity measures at the 8-year visit. School absence > 1 day at baseline predicted use of disease-modifying anti-rheumatic drugs, including biologics (DMARDs) (OR 1.2 (1.1–1.5)), and non-remission off medication (OR 1.4 (1.1–1.7) 8 years after disease onset. Conclusion School absence at baseline predicted adverse long-term outcome. In children and adolescents with JIA participation in school activities is mostly high after 8 years of disease. For the minority with low participation, special attention is warranted to promote their full potential of social interaction and improve long-term outcome. Open Access This article is distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.

BackgroundModels to predict disease course and long-term outcome based on clinical characteristics at disease onset may guide early treatment strategies in juvenile idiopathic arthritis (JIA). Before a prediction model can be recommended for use in clinical practice, it needs to be validated in a different cohort than the one used for building the model. The aim of the current study was to validate the predictive performance of the Canadian prediction model developed by Guzman et al. and the Nordic model derived from Rypdal et al. to predict severe disease course and non-achievement of remission in Nordic patients with JIA.MethodsThe Canadian and Nordic multivariable logistic regression models were evaluated in the Nordic JIA cohort for prediction of non-achievement of remission, and the data-driven outcome denoted severe disease course. A total of 440 patients in the Nordic cohort with a baseline visit and an 8-year visit were included. The Canadian prediction model was first externally validated exactly as published. Both the Nordic and Canadian models were subsequently evaluated with repeated fine-tuning of model coefficients in training sets and testing in disjoint validation sets. The predictive performances of the models were assessed with receiver operating characteristic curves and C-indices. A model with a C-index above 0.7 was considered useful for clinical prediction.ResultsThe Canadian prediction model had excellent predictive ability and was comparable in performance to the Nordic model in predicting severe disease course in the Nordic JIA cohort. The Canadian model yielded a C-index of 0.85 (IQR 0.83–0.87) for prediction of severe disease course and a C-index of 0.66 (0.63–0.68) for prediction of non-achievement of remission when applied directly. The median C-indices after fine-tuning were 0.85 (0.80–0.89) and 0.69 (0.65–0.73), respectively. Internal validation of the Nordic model for prediction of severe disease course resulted in a median C-index of 0.90 (0.86–0.92).ConclusionsExternal validation of the Canadian model and internal validation of the Nordic model with severe disease course as outcome confirm their predictive abilities. Our findings suggest that predicting long-term remission is more challenging than predicting severe disease course.

Made available in DSpace on 2019-10-05T16:54:20Z (GMT). No. of bitstreams: 0 Previous issue date: 2019-04-01 IRCCS Istituto Giannina Gaslini Background To our knowledge, the characteristics and burden of childhood arthritis have never been studied on a worldwide basis. We aimed to investigate, with a cross-sectional study, the prevalence of disease categories, treatment methods, and disease status in patients from across different geographical areas and from countries with diverse wealth status. Methods In this multinational, cross-sectional, observational cohort study, we asked international paediatric rheumatologists from specialised centres to enrol children with a diagnosis of juvenile idiopathic arthritis, according to International League of Associations for Rheumatology criteria, who were seen consecutively for a period of 6 months. Each patient underwent retrospective and cross-sectional assessments, including measures of disease activity and damage and questionnaires on the wellbeing and quality of life of the children. We qualitatively compared the collected data across eight geographical areas, and we explored an association between disease activity and damage and a country's gross domestic product (GDP) with a multiple logistic regression analysis. Findings Between April 4, 2011, and Nov 21, 2016, 9081 patients were enrolled at 130 centres in 49 countries, grouped into eight geographical areas. Systemic arthritis (125 [33.0%] of 379 patients) and enthesitis-related arthritis (113 [29.8%] of 379) were more common in southeast Asia, whereas oligoarthritis was more prevalent in southern Europe (1360 [56.7%] of 2400) and rheumatoid factor-negative polyarthritis was more frequent in North America (165 [31.5%] of 523) than in the other areas. Prevalence of uveitis was highest in northern Europe (161 [19.1%] of 845 patients) and southern Europe (450 [18.8%] of 2400) and lowest in Latin America (54 [6.4%] of 849), Africa and Middle East (71 [5.9%] of 1209), and southeast Asia (19 [5.0%] of 379). Median age at disease onset was lower in southern Europe (3.5 years, IQR 1.9-7.3) than in other regions. Biological, disease-modifying antirheumatic drugs were prescribed more frequently in northern Europe and North America than in other geographical settings. Patients living in countries with lower GDP had greater disease activity and damage than those living in wealthier countries. Damage was associated with referral delay. Interpretation Our study documents a variability in prevalence of disease phenotypes and disparities in therapeutic choices and outcomes across geographical areas and wealth status of countries. The greater disease burden in lowerresource settings highlights the need for public health efforts aimed at improving equity in access to effective treatments and care for juvenile idiopathic arthritis. Copyright (C) 2019 Elsevier Ltd. All rights reserved. Univ Genoa, Ist Giannina Gaslini, IRCCS, Clin Paediat & Rheumatol, Genoa, Italy Univ Genoa, Ist Giannina Gaslini, IRCCS, Epidemiol & Biostat Serv, Genoa, Italy Univ Genoa, Ist Giannina Gaslini, IRCCS, Sci Directory, Genoa, Italy Univ Genoa, PRINTO, IRCCS, Ist Giannina Gaslini, Genoa, Italy Univ Genoa, Dept Neurosci Rehabil Ophthalmol Genet & Maternal, Genoa, Italy Wilhelmina Childrens Hosp, Dept Pediat Immunol & Rheumatol, Utrecht, Netherlands Sanjay Gandhi Postgrad Inst Med Sci, Lucknow, Uttar Pradesh, India Univ Hosp 12 Octubre, Dept Pediat Rheumatol, Madrid, Spain Alfaisal Univ, Dept Pediat Rheumatol, King Faisal Specialist Hosp, Riyadh, Saudi Arabia Alfaisal Univ, Res Ctr, Riyadh, Saudi Arabia Western Univ Childrens Hosp, Hlth Sci Ctr, London, ON, Canada Oslo Univ Hosp, Dept Rheumatol, Oslo, Norway Oslo Univ Hosp, Med Fac, Oslo, Norway Univ Oslo, Oslo, Norway Oslo Univ Hosp, Norwegian Natl Advisory Unit Rheumat Dis Children, Oslo, Norway Univ Hosp Munster, Dept Pediat Rheumatol & Immunol, Munster, Germany Hosp Sor Maria Ludovica, Rheumatol Serv, La Plata, Buenos Aires, Argentina Bucharest Emergency Hosp, Cluj Napoca, Romania Childrens Emergency Hosp, Cluj Napoca, Romania Cincinnati Childrens Hosp Med Ctr, Div Rheumatol, Cincinnati, OH 45229 USA Alberta Childrens Prov Gen Hosp, Div Pediat Rheumatol, Dept Pediat, Calgary, AB, Canada Sofiamed, Pediat Dept, Sofia, Bulgaria Rigshosp, Juliane Marie Ctr, Paediat Rheumatol Unit, Copenhagen, Denmark Natl Inst Rheumatism & Physiotherapy, Clin Immunol Adult & Paediat Rheumatol Dept, Budapest, Hungary Univ Latvia, Pediat Dept, Riga, Latvia Univ Latvia, Univ Childrens Hosp, Riga, Latvia Univ Estadual Paulista, Botucatu Fac Med, Botucatu, SP, Brazil Shariati Hosp, Rheumatol Res Ctr, Dept Pediat & Rheumatol, Tehran, Iran Inst Rheumatol Belgrade, Div Pediat Rheumatol, Belgrade, Serbia Thessaloniki Univ, Dept Pediat 1, Hippokrat Gen Hosp, Sch Med, Thessaloniki, Greece Univ Estadual Paulista, Botucatu Fac Med, Botucatu, SP, Brazil

ObjectivesTo describe the characteristics, outcome and predictive factors of juvenile mixed connective tissue disease (JMCTD) in a nationwide cohort of patients.MethodsWe examined 55 patients with JMCTD after a mean disease duration of 16.2 years (SD 10.0). Patients were registered according to Kasukawa's criteria. Remission criteria were defined according to those for juvenile idiopathic arthritis, plus absence of cytopenia, myositis, progressive sclerodactyly, lung and oesophageal manifestations. Organ damage was assessed with the Systemic Lupus International Collaborating Clinics (SLICC)/American College of Rheumatology (ACR) Damage Index and the Juvenile Arthritis Damage Index (JADI). Medical records were reviewed for early predictors for outcome, which were assessed by multivariate logistic regression analyses.ResultsThree patients developed systemic lupus erythematosus (SLE). Fifty-two patients had continuous JMCTD; the most common manifestations were: Raynaud (100%), arthritis (94%), puffy hands (77%) and pulmonary manifestations (58%). SLE-like, systemic sclerosis (SSc)-like and polymyositis (PM)-like findings were found in 98%, 77% and 48%, respectively. Over time, SLE-like and PM-like manifestations decreased, and SSc-like findings increased. At follow-up, 35 patients (67%) had active disease and 17 (33%) were in remission. In 34 patients (65%), SLICC or JADI≥1 assessments indicated organ damage. Active disease was associated with higher anti-ribonucleoprotein antibody titres at follow-up and positive rheumatoid factor (RF) at diagnosis and follow-up.ConclusionsMost patients with JMCTD had active disease and organ damage after a mean follow-up of 16.2 years. Active disease was associated with higher anti-ribonucleoprotein antibody levels and positive RF. The presence of RF at diagnosis predicted persistent disease activity.

Background Juvenile Idiopathic Arthritis (JIA) has a broad impact on the child and family life. There is an increasing interest in the importance and recognition of the value of parent/patient reported outcome (PRO). Juvenile Arthritis Multidimensional Assessment Report (JAMAR) assesses essential aspects of the child’s disease perception and Health Related Quality of Life (HRQoL). The intention is to enhance adequate medical decisions and to improve patient care in routine clinical setting. 1 Objectives To determine the level of agreement between patients with JIA and their parents on the quantitative items of the Norwegian version of JAMAR, the direction of potential differences and if sex, age, disease activity and duration influence the level of agreement. Methods 129 patient/parent dyads participated in the study. The patients, aged 12 to 18 years, were included consecutively at Oslo University Hospital, St. Olavs Hospital in Trondheim, and the University Hospital of Northern Norway in Tromso during 2012–2013. Both patients and parents completed the JAMAR questionnaire. Demographic and disease specific data were recorded. The study examined the level of agreement for the seven quantitative items in JAMAR. Pain, disease activity (DA) and wellbeing (WB) were measured by a 21-numbered circle VAS, physical function with Juvenile Arthritis Functional Score (JAFS) and HRQoL with Paediatric Rheumatology Quality of Life Scale (JQL) total score including the sub- dimensions Physical Health (JQLPhH) and Psychosocial Health (JQLPsH). Both the direction of the differences in scores and whether sex, age, disease activity (MDGlobal), and duration influenced the degree of agreement were investigated. Intraclass correlation coefficient ICC was used for analysis together with paired and independent t-test. Results Median age for patients were 15 years, 66% were girls, 37% had oligoarticular JIA, 25% had polyarticular RF negative JIA, 4% had systemic JIA, 34% belonged to other categories, and median MDGlobal was 1 (range 0–7). As a group, patients and parents median scores were similar, except for JQLPhH, where patients scored better than parents (p=0.002). Correlations between answers from patients and parents were significant for all items (p 2 Male patients scored better and female patients scored worse for WB than their parents (p=0.03). Patients with MDGlobal ≤1 scored less pain and patients with MDGlobal >1 scored more pain than their parents (p=0.01). Conclusions The Norwegian version of JAMAR is a PRO- and HRQoL-tool suitable for standard clinical care. The study shows high level of agreement between patients and parents as groups, but the strength of the agreement varies between the items. Comparing dyads showed substantial differences on all items. Thus it is important that both adolescent patients and parents complete the questionnaire, and that both reports are used to support adequate clinical assessment and care. References [1] Filocamo G, et al. The Journal of rheumatology2011May;38(5):938–53. [2] Vanoni F, et al. Pediatric Rheumatology 2016;14:2. Disclosure of Interest None declared

Background The aim was to develop prediction rules that may guide early treatment decisions based on baseline clinical predictors of long-term unfavorable outcome in juvenile idiopathic arthritis (JIA). Methods In the Nordic JIA cohort, we assessed baseline disease characteristics as predictors of the following outcomes 8 years after disease onset. Non-achievement of remission off medication according to the preliminary Wallace criteria, functional disability assessed by Childhood Health Assessment Questionnaire (CHAQ) and Physical Summary Score (PhS) of the Child Health Questionnaire, and articular damage assessed by the Juvenile Arthritis Damage Index-Articular (JADI-A). Multivariable models were constructed, and cross-validations were performed by repeated partitioning of the cohort into training sets for developing prediction models and validation sets to test predictive ability. Results The total cohort constituted 423 children. Remission status was available in 410 children: 244 (59.5%) of these did not achieve remission off medication at the final study visit. Functional disability was present in 111/340 (32.7%) children assessed by CHAQ and 40/199 (20.1%) by PhS, and joint damage was found in 29/216 (13.4%). Model performance was acceptable for making predictions of long-term outcome. In validation sets, the area under the curves (AUCs) in the receiver operating characteristic (ROC) curves were 0.78 (IQR 0.72–0.82) for non-achievement of remission off medication, 0.73 (IQR 0.67–0.76) for functional disability assessed by CHAQ, 0.74 (IQR 0.65–0.80) for functional disability assessed by PhS, and 0.73 (IQR 0.63–0.76) for joint damage using JADI-A. Conclusion The feasibility of making long-term predictions of JIA outcome based on early clinical assessment is demonstrated. The prediction models have acceptable precision and require only readily available baseline variables. Further testing in other cohorts is warranted. © The Author(s). 2018 Open Access This article is distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/)

The objective of this prospective long-term follow-up study was to investigate whether somatosensory function is altered among young adults born preterm with very low birth weight (VLBW; ≤1,500 g) or small for gestational age (SGA;10th percentile) at term. In a blinded quantitative sensory testing protocol, we determined thermal detection, thermal pain, and pressure pain thresholds and the response to prolonged supra-threshold heat among 51 VLBW, 66 term SGA, and 86 term-born controls (birth weight ≥10th percentile) at 28 years. Self-reported chronic pain was also investigated. Except for increased sensitivity to cool in the term SGA group versus controls, we found no significant group differences regarding thermal or pain thresholds. Overall, male participants had higher pain thresholds, and no significant interactions of group and sex were observed (P.14). Within the VLBW group, neonatal mechanical ventilation was associated with reduced sensitivity to cool, and length of mechanical ventilation correlated with lower pressure pain thresholds. The response to prolonged supra-threshold heat was similar between the groups, and the prevalence of self-reported chronic pain was not reliably different. In conclusion, low birth weight young adults were as sensitive to thermal and pain stimuli as term-born, normal birth weight controls, with the same sex differences.To our knowledge, this is the first report on thermal and pain sensitivity among young adults born preterm with VLBW or SGA at term. The negative results from a comprehensive quantitative sensory testing protocol oppose previous findings of altered sensory perception among children and adolescents born preterm.

The Juvenile Arthritis Multidimensional Assessment Report (JAMAR) is a new parent/patient-reported outcome measure that enables a thorough assessment of the disease status in children with juvenile idiopathic arthritis (JIA). We report the results of the cross-cultural adaptation and validation of the parent and patient versions of the JAMAR in the Norwegian language. The reading comprehension of the questionnaire was tested in 10 JIA parents and patients. Each participating centre was asked to collect demographic and clinical data and the JAMAR in 100 consecutive JIA patients or all consecutive patients seen in a 6-month period and to administer the JAMAR to 100 healthy children and their parents. The statistical validation phase explored descriptive statistics and the psychometric issues of the JAMAR: the 3 Likert assumptions, floor/ceiling effects, internal consistency, Cronbach’s alpha, interscale correlations, test–retest reliability, and construct validity (convergent and discriminant validity). A total of 301 JIA patients (3.3% systemic, 41.2% oligoarticular, 25.9% RF negative polyarthritis, and 29.6% other categories) and 74 healthy children were enrolled in three centres. The JAMAR components discriminated well healthy subjects from JIA patients. All JAMAR components revealed good psychometric performances. In conclusion, the Norwegian version of the JAMAR is a valid tool for the assessment of children with JIA and is suitable for use both in routine clinical practice and clinical research. © The Author(s) 2018 Open Access. This article is distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/)

Background Pulmonary manifestations in mixed connective tissue disease (MCTD) are common and a major cause of morbidity and mortality [1]. Data on lung involvement in patients with juvenile onset MCTD (JMCTD) are scarce. Objectives The aim of this study was 1) to compare pulmonary function abnormalities in a nationwide representative Norwegian JMCTD cohort with that of matched controls, 2) investigate occurrence of interstitial lung disease (ILD) in JMCTD and 3) to evaluate possible associations between pulmonary findings and disease related variables. Methods Inclusion criteria were fulfillment of the Kasukawa or Alarcon-Segovia criteria and symptom-onset before 18 years. The control group was randomly drawn from the national population register, after matching for age and gender. Fifty-two patients with JMCTD were examined after mean disease duration 16.2 years, 44 (85%) were female. Patients and controls performed pulmonary function tests (PFT) and a 6 min walk test (6MWT). The patients had a high-resolution CT (HRCT) of the lungs. Results Abnormal PFT were found in 24 patients (46%) and in 12 controls (23%) (p=0.01) (table 1). More patients than controls had abnormal FVC (11 patients and 0 controls, p Conclusions Patients with JMCTD had significantly reduced pulmonary function after mean 16.2 years disease duration compared to matched controls. However, overall lung function was only moderately reduced. The occurrence of ILD assessed with HRCT was 29%, but the majority of patients with ILD had mild disease. To our knowledge, this is the first systematic case-control study of pulmonary manifestations in JMCTD. References Gunnarsson R, Aalokken TM, Molberg O, et al. Prevalence and severity of interstitial lung disease in mixed connective tissue disease: a nationwide, cross-sectional study. Ann Rheum Dis 2012;71:1966–72. Disclosure of Interest None declared

The aim of this study was to examine a possible relationship between early puberty and chronic nonspecific pain in 13- to 18-year-old girls. All adolescents in Nord-Trøndelag County, Norway, were invited to participate in the Young-HUNT3 study (2006-2008). Of the invited girls, 81% answered the questionnaire and of these 3982 were 13 to 18 years of age. Menarche and perceived physical maturation were used as exposure measures. Early menarche was defined as

Background The aim of this study was to examine the associations of birthweight, gestation and 5-min Apgar score with self-reported chronic nonspecific pain in a large, unselected adolescent population. Methods The third population-based Nord-Trondelag Health Study (HUNT) included 8200 adolescents aged 13-19 years, constituting 78.2% of adolescents in Nord-Trondelag County. In the target age group, 13-18 years, data on pain frequency from 10 localizations were available from 7373 adolescents. Chronic nonspecific pain was defined as pain at least once a week during the last 3 months, not related to any known disease or injury. Chronic multisite pain was defined as chronic pain in at least three localizations, and chronic daily pain was defined as chronic pain almost every day. Perinatal data were retrieved from the Medical Birth Registry of Norway, and data were available for 7120 of the 7373 adolescents. Covariates included adolescent and maternal general health measures from the HUNT study. Results We found no consistent association between preterm birth and chronic pain and no clear association between birthweight and chronic pain complaints in adolescence. Post-term birth in boys and a low 5-min Apgar score in both sexes tended to increase the reporting of chronic pain in adolescence. Conclusions Perinatal factors, and especially preterm birth and low birthweight, did not seem to have a major impact on pain complaints in adolescence.

Objective Amyloid deposition in multiple myeloma (MM) may lead to an arthropathy resembling rheumatoid arthritis (RA). Since a systematic description of its natural history is lacking, we have performed a systematic analysis of all published cases. Methods Literature review featuring backward and forward database searches and direct inspection of reference lists. Inclusion criteria were as follows: publication between 1931 and 2012, diagnosis of multiple myeloma, and demonstration of light chain amyloid (AL) in any organ or in synovial fluid, arthritis, or synovitis. Results Overall, 101 cases were identified. Median age was 59 years and the male-to-female ratio was 1:1. A systemic manifestation of MM was reported in 88 cases. In 53 of these, characteristic physical findings (carpal tunnel syndrome, macroglossia, shoulder pad, and soft tissue swelling/masses) were present. Arthritis manifested before the diagnosis of MM in 63 cases, with 33 cases initially misdiagnosed as RA. There were 72 cases of poly-, 17 of oligo-, and three of monoarthritis. The shoulder joint was most commonly affected, followed by knees and small hand joints. Median synovial fluid leukocyte count was 2460 cells/mm 3 , and was normal in seven cases. Synovial histopathology often featured mild synovitis without plasma cell infiltration. Imaging revealed articular or periarticular inflammation in many cases and bone lesions near 22% of affected joints. Treatments varied but led to some improvement in the majority of cases. Conclusions These results solidify previous experience that MM arthropathy tends to feature a symmetric RF-negative nonerosive polyarthritis. However, the results also highlight the diversity of its presentations and stress the importance of arthropathy as a potentially under-recognized presenting manifestation of MM.

To evaluate whether C-reactive protein (CRP), including variation within the normal range, is predictive of long-term disease outcome in Juvenile Idiopathic Arthritis (JIA). Consecutive patients with newly diagnosed JIA were included prospectively from defined geographic areas of the Nordic countries from 1997 to 2000. Inclusion criteria were availability of a baseline serum sample within 12 months after disease onset and 8-year clinical assessment data. Systemic onset JIA was not included. CRP was measured by high-sensitive ELISA (detection limit of 0.2 mg/l). One hundred and thirty participants with a median follow-up time of 97 months (range 95-100) were included. At follow-up, 38% of the patients were in remission off medication. Absence of remission was associated with elevated level of CRP at baseline (odds ratio (OR) 1.33, confidence interval (CI) 1.08-1.63, p = 0.007). By applying a cutoff at the normal upper limit (>10 mg/l), the risk of not achieving remission was increased to an OR of 8.60 (CI 2.98-24.81, p

The aim of this study was to determine the prevalence of self-reported chronic idiopathic pain among adolescents in relation to age and gender, and to explore how pain interferes with daily activities. The study was performed in Nord-Trøndelag County, Norway in 2006-2008. All adolescents were invited to participate; the response rate was 78%. Participants completed a comprehensive questionnaire, including questions about pain and interference with everyday life. Chronic idiopathic pain was defined as pain at least once a week during the last 3 months, not related to any known disease or injury. The final study population, with complete pain questionnaires, consisted of 7373 adolescents aged 13-18 years. Chronic pain was reported by 44.4% of the participants, and 25.5% reported pain in at least 2 locations. Chronic idiopathic musculoskeletal pain was most prevalent (33.4%), and the neck/shoulder was most commonly affected. Musculoskeletal pain in 3 or more locations was reported by 8.5%. Pain almost daily was reported by 10.2%. More girls than boys reported pain. In girls, the prevalence of pain increased with age. A high number of pain-associated disabilities were reported, and 58.5% described difficulties doing daily activities in leisure time. Subjective disabilities were higher in girls, and increased with the frequency of pain and the number of pain locations, as shown by high disability in adolescents with musculoskeletal pain in 3 or more locations. Chronic idiopathic pain, especially multisite pain, is common among adolescents, and those suffering from it report a major impact on several areas of daily living.

The general movement assessment (GMA) method is used to predict cerebral palsy (CP) in infants with high risk of developing neurological dysfunctions. Most of the work on GMA has been performed from the same group of researchers. The aim of this study was to demonstrate to what extent GMA predicted CP in our hands.A prospective study was performed using the Prechtl classification system for GMA in the fidgety period to predict later cerebral palsy. The study population consisted of 74 term and preterm infants at low and high risk of developing neurological dysfunction. The absence or presence of CP was reported at 23 months median-corrected age by the child's physician and the parents.The GMA identified all 10 infants that later were classified as having CP. GMA also identified all the infants that did not develop CP except for one infant with abnormal GMA and no CP. Three infants had uncertain CP status at follow-up. The sensitivity of GMA with regard to later CP was 100% with 95% CI (0.73, 1.00) and the specificity was 98% with 95% CI (0.91, 0.99) when the three uncertain cases were excluded.Our study indicates that the GMA used in a clinical setting strongly predicts the development of CP. The work supports the results of previous studies and contributes to the validation of GMA. The qualitative nature of this method may be a problem for inexperienced observers. Larger clinical studies are needed.

Uveitis is the most common extraarticular manifestation of juvenile idiopathic arthritis (JIA). The nature of JIA-associated uveitis is often insidious and asymptomatic, and baseline predictors can aid early diagnosis of eye disease for prompt and adequate treatment. Antihistone antibodies (AHA) are among subtypes of antinuclear antibodies (ANA) identified in children with JIA. An association between early-onset JIA, oligoarthritis and uveitis is shown in some studies, and we have previously shown that AHA is a significant predictor of chronic uveitis in a Norwegian JIA cohort. New interest for histones has emerged because epigenetic alterations of these DNA-binding molecules may be involved in the pathological processes of autoimmunity.

The etiology and pathogenesis of JIA are believed to be influenced by both genetic and environmental factors. Although several associations between the risk of developing JIA and gene variants both inside and outside the HLA region have been confirmed, no studies have examined associations between non-HLA gene polymorphisms and the disease outcome.

Methods A population-based, cross-sectional study was carried out in Nord-Trondelag county, Norway, during the years 2006-2008. All adolescents aged 12-19 years were invited to participate in the youth part of the Nord-Trondelag Health Study [“Helseundersokelsen i Nord-Trondelag (HUNT)”]. Response rate was 78% (8200 responders out of 10.485 invited). The adolescents completed a comprehensive questionnaire including questions about whether they had experienced any headaches and/or any musculoskeletal pain during the last three months, not related to any known disease or injury. They were asked to indicate the location of pain (neck/shoulders, upper back, lower back/buttocks, chest, upper and/or lower extremities). The participants were also asked to indicate how often they had experienced pain in these areas (seldom or never, once a month, once a week, more than once a week, almost every day). The final study population, with complete pain questionnaires, consisted of 7373 adolescents aged 13-18 years.

Purpose Juvenile idiopathic arthritis (JIA) is the most common rheumatic disease in children. During the last decade international consensus on the ILAR (International League Against Rheumatism) classification of this heterogeneous group of chronic childhood arthritis has been reached. In an era of modern medical treatment, epidemiologic studies on outcome in unselected cohorts of children with JIA are important. The aim of the study was to describe disease characteristics, ILAR categories and remission status during the first eight years after disease onset in a cohort of Nordic children with JIA in a population-based setting.

The aim of this study was to assess the association of chronic pain with different lifestyle factors and psychological symptoms in a large, unselected adolescent population. Pain was evaluated as chronic non-specific pain, chronic multisite pain, and in additional analyses, chronic pain with high disability. The study was performed during 2006 to 2008 in Nord-Trondelag County, Norway. Adolescents aged 13 to 18 years were invited to participate. The response rate was 78%. The final study population consisted of 7,373. Sedentary behavior and pain were associated only in girls. In both sexes, overweight and obesity were associated with increased odds of pain. Whereas both smoking and alcohol intoxication showed strong associations with pain, the associations were attenuated after adjustments for psychosocial factors. Symptoms of anxiety and depression showed the strongest associations with pain (odds ratio 4.1 in girls and 3.7 in boys). The odds of pain increased gradually by number of unfavorable lifestyle factors reported. This study revealed consistent associations between lifestyle factors, anxiety and depression, and chronic pain, including multisite pain and pain with high disability. The consistency across the different pain categories suggests common underlying explanatory mechanisms, and despite the cross-sectional design, the study indicates several modifiable targets in the management of adolescent chronic pain. Perspective This study showed a clear and consistent relation between different lifestyle factors, anxiety and depression, and the pain categories chronic non-specific pain, multisite pain, and also pain with high disability. Independent of causality, it underlines the importance of a broad perspective when studying, preventing, and treating chronic pain in adolescents.

To obtain longitudinal data on growth/puberty in a large-scale, multi-national prospective cohort of juvenile systemic lupus erythematosus (SLE).Data from 331/557 (59.4%) patients ≤18 years old with juvenile SLE in active phase, with anthropometric data available at four follow-up visits, were studied.There was a significant reduction in parent-adjusted height z score with time in females and males (p0.0001), with a significant gender difference (p0.0001) and with male height being most affected. Median body mass index z score peaked at 6 months and was still significantly above baseline after 26 months (p0.01), with no gender difference. Standardised height reduction was inversely related to age at onset. Females with onset age12 years had a median parent-adjusted height z score of -0.87 with no catch-up growth. At the end of the study, growth failure was seen in 14.7% of the females and 24.5% of the males. Height deflection (less than -0.25/year) was found in 20.7% of the females and 45.5% of the males. Delayed pubertal onset was seen in 15.3% and 24% of the females and males, respectively, and delayed/absent menarche was seen in 21.9%, while 36.1% of the females and 44% of the males had some degree of delayed pubertal development. Growth failure baseline determinants were previous growth failure (OR: 56.6), age at first visit ≤13.4 years (OR: 4.2) and cumulative steroid dose426 mg/kg (OR: 3.6).The children at risk of having a negative effect on height and pubertal development are prepubertal and peripubertal children treated with400 mg/kg cumulative dose of corticosteroids.

Background Juvenile Arthritis Disease Activity Score (JADAS) is a recently developed composite tool for scoring disease activity in juvenile idiopathic arthritis (JIA). JADAS consists of four items; the joint count, the physician and the patient’s/parent’s global assessment and the erythrocyte sedimentation rate (ESR) as an inflammatory marker. Creactive protein (CRP) has been suggested as an alternative inflammatory marker [1].

Objective.To study the predictive value of antinuclear autoantibody (ANA) tests and antihistone antibodies (AHA) as risk factors for development of chronic asymptomatic uveitis of insidious onset in juvenile idiopathic arthritis (JIA).Methods.ANA by indirect immunofluorescence using HEp-2 cells (IF-ANA), ELISA for ANA (E-ANA), and AHA were analyzed in sera of 100 children with recent-onset JIA and in 58 control sera. Clinical features, including age at onset, JIA subgroup, and presence of uveitis, were recorded in this prospective population-based cohort study.Results.E-ANA was positive in 4 of the 100 sera, and was not associated with uveitis. Chronic uveitis developed in 16 children with JIA: in 14 of 68 positive for IF-ANA ≥ 80, and in 13 of 44 positive for AHA ≥ 8 U/ml. IgM/IgG AHA were found in higher proportions in children with uveitis (mean 12.4 U/ml) than in those with JIA and no uveitis (mean 6.9 U/ml) or in healthy controls (mean 4.3 U/ml).Conclusion.No association was found between E-ANA and uveitis, and most IF-ANA-positive sera were E-ANA-negative. E-ANA is not clinically relevant in this setting and should never be used to determine frequencies of eye examinations to detect new uveitis in JIA. AHA ≥ 8 U/ml, IF-ANA titer ≥ 320, and young age at onset of arthritis were significant predictors for development of chronic uveitis. The diagnostic value of AHA ≥ 8 U/ml as a biomarker of chronic uveitis in JIA is very similar to IF-ANA ≥ 80.

Objective:To assess whether the removal of aids/devices and/or help from another person in the Childhood Health Assessment Questionnaire (C-HAQ) leads to a significant change in the disability index (DI) score and responsiveness in juvenile idiopathic arthritis (JIA).Methods:Changes in the C-HAQ DI score in a cross-sectional sample of 2663 children with JIA and in 530 active patients with JIA in a trial of methotrexate (MTX) were compared.Results:Patients in the MTX trial had higher disease activity and disability than the cross-sectional sample. The frequency of aids/devices (range 1.2–10.2%) was similar between the two samples, while help (range 5.3–38.1%) was more frequently used in the MTX group. Correlation between disease severity variables and the two different C-HAQ DI scoring methods did not change substantially. There was a decrease in the C-HAQ DI score for both the cross-sectional (mean score from 0.64 with the original method to 0.54 without aids/devices and help, pConclusion:The removal of aids/devices and help from the C-HAQ does not alter the interpretation of disability at a group level. The simplified C-HAQ is a more feasible and valid alternative for the evaluation of disability in patients with JIA.

The aim of this study was to survey the occurrence of invasive group B streptococcus (GBS) disease in Norway and detect possible trends in characteristics of invasive GBS strains from 1996 to 2006. Data from national monitoring systems for infectious diseases in Norway were analysed. Of 638,452 live births in the period, 434 cases of invasive GBS disease in infants were reported. In adults and children older than 1 year of age, 969 cases were reported. The incidence of invasive GBS disease increased significantly in the elderly, while the incidence of neonatal early-onset disease was stable with 0.46 cases per 1,000 live births. The incidence of late-onset disease increased in 2005 and 2006. The lethality of GBS in infants increased from an average of 6.5% in 1996-2005 to 20% in 2006. Serotypes III and V were predominant in 839 invasive GBS strains characterized-type III in infants and type V in the elderly. The distribution of serotypes did not change throughout the period. The distribution of detected surface proteins was stable from 1996 to 2005, but the detection rates in types III and V were low. Molecular methods for GBS typing introduced in 2006 made characterization of nearly all strains possible and appear more applicable to epidemiological studies of GBS than conventional methods. Resistance to erythromycin and clindamycin increased significantly in 2006. The increased incidence in the elderly, the increased lethality in infants in 2006, and the increased resistance to erythromycin and clindamycin the same year might indicate changing characteristics of invasive GBS strains.

Group B streptococcus (GBS) is an important aetiological agent of serious neonatal infections. A rapid and sensitive method for the detection of GBS colonization in pregnant women at delivery could make intrapartum screening for GBS possible. A real-time PCR method targeting the sip gene of GBS in pregnant women at delivery has been evaluated. The performance of the real-time PCR was compared with optimized GBS culture. Separate vaginal and rectal swabs were collected from women hospitalized at the delivery department at St Olavs Hospital, Trondheim, Norway, from January 15 through May 2005. The specimens were cultured on selective blood agar plates and in selective broth and examined by real-time PCR. Of samples from 251 women, 87 (34.7%) were GBS positive by culture and 86 (34.3%) were positive by PCR. Using GBS culture as the 'gold standard', the sensitivity of real-time PCR was 0.97 (95% confidence interval 0.90-0.99) and specificity was 0.99 (95% confidence interval 0.97-1.00). In two women the PCR was positive and the culture negative. Additional analysis using cylE PCR substantiates that these two women were true GBS carriers with negative GBS culture. The rate of GBS colonization was lower in vaginal specimens than in rectal specimens both by culture and PCR. The real-time PCR assay is fast, highly sensitive and specific for detecting GBS colonization in pregnant women at delivery, and has the potential for intrapartum detection of GBS colonization. Both vaginal and rectal samples are required to achieve highest possible detection rate.

Background Juvenile Idiopathic Arthritis (JIA) is the most common rheumatic disease of childhood. Pain is a frequent complaint and an important reason for reduced quality of life for these children and adolescents. Knowledge of pain and its relevance for quality of life and disease outcome is limited. Objectives To study self-reported pain early in the disease course as predictor of persistent pain and disease outcome in a long-term perspective. Methods Consecutive cases of JIA with disease onset in 1997 to 2000 from defined geographical areas of Sweden, Denmark, Finland and Norway were prospectively included in a population-based cohort study (1). Self-reported pain was measured on a 10 cm visual analogue scale (VAS pain ). Remission at 8-year follow-up was defined according to the preliminary criteria described by Wallace (2). Finnish participants did not fill in the pain score and were excluded. The final study population consisted of 302 children, 204 of these had filled in the pain score both 6 months after disease onset and at 8-year follow-up. For multivariable analyses we used binomial regression in STATA. Results Among the 204 children, 69% were girls, median onset age was 5.9 years and 50% had oligoarticular disease 6 months after onset. Six months after disease onset 74% reported VAS pain >0. At 8-year follow-up 57% reported VAS pain >0, and 61% was either not in remission or in remission on medication. We found an association between early pain reports and outcome 8 years later. For participants reporting no pain at 6 months, the risk of reporting pain at 8-year follow-up was 28% and the risk of not being in remission or in remission on medication was 27%. On the contrary, for participants reporting VAS pain >0 at 6 months, the risk of reporting pain at 8 year was 68% and the risk of not being in remission or in remission on medication was 73%. The results were similar after adjustments for age and sex. Conclusions Early pain report appears to be predictive of both more severe disease outcome and more persistent pain in a long-term perspective. References Nordal E, et al. Arthritis Rheum 2011;63:2809-18 Wallace CA, et al. J Rheumatol 2004;31:2290-4 Disclosure of Interest None declared

Background Among pediatric rheumatologists there is an on-going discussion whether juvenile psoriatic arthritis is an entity of its own or not. It is recognized that psoriatic arthritis is a disease with both autoimmune and autoinflammatory features. The ILAR-classification of psoriatic arthritis is under debate. Objectives To study clinical signs, classification, and outcome in children with juvenile idiopathic arthritis (JIA) and psoriasis/psoriasis-like rash in a population-based cohort during the first eight years of disease. Methods In total 440 children with JIA were included prospectively in a population-based cohort study. The presence of psoriasis and psoriasis-like rash during an eight years follow-up period was assessed in relation to clinical characteristics, ILAR-classification and remission. Results - Of the 440 children 14 developed psoriasis and 13 psoriasis-like rash during the first eight years of disease. 8/14 children with psoriasis and JIA were classified as juvenile psoriatic arthritis (JPA). 6/14 children with psoriasis and JIA were classified as undifferentiated JIA. One was RF positive and the other five had concurrent signs of enthesitis-related arthritis. Children with dactylitis and/or nail-pitting were spread among many ILAR categories. Children with JIA and psoriasis/psoriasis-like rash had significantly higher number of cumulative active joints during the first eight years from disease onset than children with no psoriasis/psoriasis-like rash (p=0.02). Dactylitis, nail pitting and enthesitis were significantly more common (0.01, Children with and without psoriasis/psoriasis-like rash had the same outcome with regards to remission status after eight years. Conclusions ILAR-criteria exclude children with spondylitis and enthesitis-related features from the JPA category. We suggest that this is not clinically relevant. Our results indicate a more severe disease course in children with psoriasis/psoriatisis-like rash and JIA, but the number of children with psoriasis/psoriasis-like rash was limited. There is a need to study children with psoriasis and arthritis in more detail regarding clinical pattern and prognosis. Disclosure of Interest None declared