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Additional file 1: Supplementary Material S1. CAPTURE-JIA Agileware Solution Security Information.

Funder: Versus Arthritis UK, Funder: LUPUS UK; FundRef: https://doi.org/10.13039/501100018817, Introduction: Juvenile-onset systemic lupus erythematosus (JSLE) is a rare autoimmune/inflammatory disease with significant morbidity and mortality. Neuropsychiatric (NP) involvement is a severe complication, encompassing a heterogeneous range of neurological and psychiatric manifestations. Methods: Demographic, clinical, and laboratory features of NP-SLE were assessed in participants of the UK JSLE Cohort Study, and compared to patients in the same cohort without NP manifestations. Results: A total of 428 JSLE patients were included in this study, 25% of which exhibited NP features, half of them at first visit. Most common neurological symptoms among NP-JSLE patients included headaches (78.5%), mood disorders (48.6%), cognitive impairment (42%), anxiety (23.3%), seizures (19.6%), movement disorders (17.7%), and cerebrovascular disease (14.9%). Peripheral nervous system involvement was recorded in 7% of NP-SLE patients. NP-JSLE patients more frequently exhibited thrombocytopenia (

Supplemental material, sj-pdf-1-lup-10.1177_0961203320984251 for Clinical and laboratory phenotypes in juvenile-onset Systemic Lupus Erythematosus across ethnicities in the UK by Joseph S Massias, Eve MD Smith, Eslam Al-Abadi, Kate Armon, Kathryn Bailey, Coziana Ciurtin, Joyce Davidson, Janet Gardner-Medwin, Kirsty Haslam, Dan P Hawley, Alice Leahy, Valentina Leone, Flora McErlane, Devesh Mewar, Gita Modgil, Robert Moots, Clarissa Pilkington, Athimalaipet V Ramanan, Satyapal Rangaraj, Phil Riley, Arani Sridhar, Nick Wilkinson, Michael W Beresford and Christian M Hedrich in Lupus

Supplemental material, sj-pdf-1-lup-10.1177_0961203320984251 for Clinical and laboratory phenotypes in juvenile-onset Systemic Lupus Erythematosus across ethnicities in the UK by Joseph S Massias, Eve MD Smith, Eslam Al-Abadi, Kate Armon, Kathryn Bailey, Coziana Ciurtin, Joyce Davidson, Janet Gardner-Medwin, Kirsty Haslam, Dan P Hawley, Alice Leahy, Valentina Leone, Flora McErlane, Devesh Mewar, Gita Modgil, Robert Moots, Clarissa Pilkington, Athimalaipet V Ramanan, Satyapal Rangaraj, Phil Riley, Arani Sridhar, Nick Wilkinson, Michael W Beresford and Christian M Hedrich in Lupus

Extended methodology from publication on AMRC of Prior elicitation of the efficacy and tolerability of Methotrexate and Mycophenolate Mofetil in Juvenile Localised SclerodermaSpecifically:Appendix S1 Participants in prior elicitation meetingAppendix S2 – Inclusion criteria for a clinical trial of MTX and MMF defined before elicitation exerciseAppendix S3 Detailed methodology of elicitation process

Additional file 1: Supplementary Table S1. Patient Reported Outcome and Experience Measures for Children Aged < 11 Years. Supplementary Table S2. Patient Reported Outcome and Experience Measures for Young People Aged ≥ 11 years.

Objectives: To investigate early vertical growth patterns and factors associated with poor growth in a modern inception cohort of UK children with juvenile idiopathic arthritis (JIA) using data from the Childhood Arthritis Prospective Study (CAPS). Methods: A study period of 3 years was chosen. Children included in this analysis had a physician diagnosis of JIA and had height measurements available at both baseline and at three-years of follow-up. Height is presented as z-scores calculated using World Health Organisation growth standards for age and gender. Growth over the three-year period was assessed using change in z-score and height velocity. Univariable and multivariable linear regressions were used to identify factors associated with height z-score at baseline and change of height z-score at 3 years. Results: 568 patients were included; 65% female, median baseline age 7.4 years [interquartile range (IQR) 3.6, 11.2], median symptom duration at presentation 5.5 months [IQR 3.1, 11.6]. Height z-score decreased significantly from baseline to three years (p≤0.0001); baseline median height z-score was -0.02(IQR -0.71, 0.61), decreasing to -0.47(IQR -1.12, 0.24) at three years. Growth restriction, defined as change of height z-score ≤-0.5, was observed in 39% of patients. At 3 years, higher baseline height z-score was the strongest predictor for a negative change in height z-score [-0.3 per unit of baseline height z-score (95% CI -0.36, -0.24), p

Additional file 1. Supplementary Methods, Supplementary Results, Supplementary Fig. S1, Supplementary Fig. S2, Supplementary Table S1, Supplementary References.

Objectives. The medical management of JIA has advanced significantly over the past 10 years. It is not known whether these changes have impacted on outcomes. The aim of this analysis was to identify and describe trends in referral times, treatment times and 1-year outcomes over a 10-year period among children with JIA enrolled in the Childhood Arthritis Prospective Study. Methods. The Childhood Arthritis Prospective Study is a prospective inception cohort of children with new-onset inflammatory arthritis. Analysis included all children recruited in 2001–11 with at least 1 year of follow-up, divided into four groups by year of diagnosis. Median referral time, baseline disease pattern (oligoarticular, polyarticular or systemic onset) and time to first definitive treatment were compared between groups. Where possible, clinical juvenile arthritis disease activity score (cJADAS) cut-offs were applied at 1 year. Results. One thousand and sixty-six children were included in the analysis. The median time from symptom onset and referral to first paediatric rheumatology appointment (22.7–24.7 and 3.4–4.7 weeks, respectively) did not vary significantly (∼20% seen within 10 weeks of onset and ∼50% within 4 weeks of referral). For oligoarticular and polyarticular disease, 33.8–47 and 25.4–34.9%, respectively, achieved inactive disease by 1 year, with ∼30% in high disease activity at 1 year. A positive trend towards earlier definitive treatment reached significance in oligoarticular and polyarticular pattern disease. Conclusion. Children with new-onset JIA have a persistent delay in access to paediatric rheumatology care, with one-third in high disease activity at 1 year and no significant improvement over the past 10 years. Contributing factors may include service pressures and poor awareness. Further research is necessary to gain a better understanding and improve important clinical outcomes.

Background: Potential targets for treat-to-target strategies in JIA are minimal disease activity (MDA) and clinically inactive disease (CID). Short and long-term outcomes following achievement of MDA and CID on the cJADAS10 and CID on Wallace’s preliminary criteria were compared. Methods: Children recruited to the Childhood Arthritis Prospective Study, a UK multicentre inception cohort, were selected if recruited prior to January 2011 and diagnosed with oligoarthritis or rheumatoid factor negative or positive polyarthritis. At one year following diagnosis, children were assessed for MDA on the cJADAS10 and CID on both Wallace’s preliminary criteria and the cJADAS10. Associations were tested between these disease states and i) functional ability, ii) absence of limited joints, iii) psychosocial health and v) pain at one year and annually to five years.Results: Of 832 children, 70% were female and the majority had oligoarthritis (68%). At one year, 21% had achieved CID according to both definitions, 7% on Wallace’s preliminary criteria only, 16% on cJADAS10 only and 56% on neither. Only 10% of children in the entire cohort achieved MDA without also having CID. Achieving either early CID state was associated with greater absence of limited joints. However, only CID on cJADAS10 was associated with improved functional ability and psychosocial health. Achieving CID was superior to MDA in terms of short and long-term pain and the absence of limited joints.Conclusion: CID on the cJADAS10 may be a preferable treatment target to CID on Wallace’s preliminary criteria in terms of both feasibility of application and long-term outcomes.

ObjectivesPain is a very common symptom of juvenile idiopathic arthritis (JIA). Disease-activity alone cannot explain symptoms of pain in all children, suggesting other factors may be relevant. The objectives of this study were to describe the different patterns of pain experienced over time in children with JIA and to identify predictors of which children are likely to experience on-going pain.MethodsThis study used longitudinal-data from patients (aged 1-16 years) with new-onset JIA. Baseline and up to 5-year follow-up pain data from the Childhood Arthritis Prospective Study (CAPS) were used. A two-step approach was adopted. First, pain trajectories were modelled using a discrete mixture-model. Second, multinomial logistic regression was used to determine the association between variables and trajectories.ResultsData from 851 individuals were included (four years, median follow-up). A three-group trajectory model was identified: consistently-low pain (n=453), improved-pain (n=254) and consistently-high pain (n=144). Children with improved-pain or consistently-high pain differed on average at baseline from consistently-low pain. Older age at onset, poor function/disability and longer disease-duration at baseline were associated with consistently-high pain compared to consistently-low pain. Early increases in pain and poor function/disability were also associated with consistently-high pain compared to consistently-low pain. ConclusionsThis study has identified routinely collected clinical factors, which may indicate those individuals with JIA at risk of poor pain-outcomes earlier in disease. Identifying those at highest risk of poor pain-outcomes at disease onset may enable targeted pain management strategies to be implemented early in disease thus reducing the risk of poor pain-outcomes.Funding: ARUK Grant Reference number 20542

Appendix A. Topic guide for parentsâ qualitative interviews. (DOCX 28 kb)

Objectives: Timely access to holistic multidisciplinary care is the core principle underpinning management of juvenile idiopathic arthritis (JIA). Data collected in national clinical audit programmes fundamentally aim to improve health outcomes of disease, ensuring clinical care is equitable, safe and patient-centred. The aim of this study is to develop a tool for national audit of JIA in the UK.Methods: A staged and consultative methodology was used across a broad group of relevant stakeholders to develop a national audit tool, with reference to pre-existing standards of care for JIA. The tool comprises key service delivery quality measures assessed against two aspects of impact, namely disease related outcome measures and patient/carer reported outcome (PROM) and experience measures (PREM).Results: Eleven service-related quality measures were identified, including those that map to current standards for commissioning of JIA clinical services in the UK. The 3-variable JADAS composite disease activity score and presence/absence of sacro-iliitis in patients with enthesitis related arthritis were identified as the primary disease-related outcome measures, with presence/absence of uveitis a secondary outcome. Novel PROMs and PREMs were developed and face validity confirmed by relevant patient/carer groups.Conclusion: A tool for national audit of JIA has been developed with the aim of benchmarking current clinical practice and setting future standards and targets for improvement. Staged implementation of this national audit tool should facilitate investigation of variability in levels of care and drive quality improvement. This will require engagement from patients and carers, clinical teams and commissioners of JIA services.

Background: Juvenile Arthritis Disease Activity Score (JADAS) is a 4 variable composite disease activity (DA) score for JIA (including active 10, 27 or 71 joint count (AJC), physician global (PGA), parent/child global (PGE) and ESR). The validity of JADAS for all ILAR subtypes in the routine clinical setting is unknown. We investigated the construct validity of JADAS in the clinical setting in all subtypes of JIA through application to a prospective inception cohort of UK children presenting with new onset inflammatory arthritis. Methods: JADAS 10, 27 and 71 were determined for all children in the Childhood Arthritis Prospective Study (CAPS) with complete data available at baseline. Correlation of JADAS 10, 27 and 71 with single DA markers was determined for all subtypes. All correlations were calculated using Spearman's rank statistic. Results: 262/1238 visits had sufficient data for calculation of JADAS (1028 (83%) AJC, 744 (60%) PGA, 843 (68%) PGE and 459 (37%) ESR). Median age at disease onset was 6.0 years (IQR 2.6-10.4) and 64% were female. Correlation between JADAS 10, 27 and 71 approached 1 for all subtypes. Median JADAS 71 was 5.3 (IQR 2.2-10.1) with a significant difference between median JADAS scores between subtypes (p < 0.01). Correlation of JADAS 71 with each single marker of DA was moderate to high in the total cohort (see Table 1). Overall, correlation with AJC, PGA and PGE was moderate to high and correlation with ESR, limited JC, parental pain and CHAQ was low to moderate in the individual subtypes. Correlation coefficients in the extended oligoarticular, rheumatoid factor negative and enthesitis related subtypes were interpreted with caution in view of low numbers. Conclusions: This study adds to the body of evidence supporting the construct validity of JADAS. JADAS correlates with other measures of DA in all ILAR subtypes in the routine clinical setting. Given the high frequency of missing ESR data, it would be useful to assess the validity of JADAS without inclusion of the ESR. Disclosure statement: All authors have declared no conflicts of interest. Table 1Spearman's correlation between JADAS 71 and single markers DA by ILAR subtype ILAR Subtype Systemic onset JIA Persistent oligo JIA Extended oligo JIA Rheumatoid factor neg JIA Rheumatoid factor pos JIA Enthesitis related JIA Psoriatic JIA Undifferentiated JIA Unknown subtype Total cohort Number of children 23 111 12 57 7 9 19 7 17 262 AJC 0.54 0.67 0.53 0.75 0.53 0.34 0.59 0.81 0.37 0.59 PGA 0.63 0.69 0.25 0.73 0.14 0.05 0.50 0.83 0.56 0.64 PGE 0.51 0.68 0.83 0.61 0.41 0.69 0.71 0.9 0.48 0.61 ESR 0.28 0.31 0.35 0.4 0.6 0.85 0.43 0.7 0.5 0.53 Limited 71 JC 0.29 0.51 0.23 0.37 0.14 -0.12 0.4 0.81 0.45 0.41 Parental pain 0.23 0.62 0.03 0.57 0.41 0.69 0.7 0.79 0.42 0.53 Childhood health assessment questionnaire 0.25 0.57 -0.07 0.36 -0.47 0.84 0.37 0.8 0.66 0.47

Objectives: Many criteria for clinically inactive disease (CID) and minimal disease activity (MDA) have been proposed for juvenile idiopathic arthritis (JIA). It is not known to what degree each of these criteria overlap within a single patient cohort. This study aimed to compare the frequency of MDA and CID across different criteria in a cohort of children with JIA at one year following presentation. Methods: The Childhood Arthritis Prospective Study recruits children at initial presentation to paediatric or adolescent rheumatology in seven UK centres. Children recruited between October 2001 and December 2013 were included. The proportions of children with CID and MDA at one year were calculated using four investigator-defined and eight published composite criteria. Missing data were accounted for using multiple imputation under different assumptions.Results: In a cohort of 1415 children and adolescents, 67% patients had no active joints at one year. Between 48% and 61% achieved MDA and between 25% and 38% achieved CID using published criteria. Overlap between criteria varied. Of 922 patients in MDA by either the original composite criteria, Juvenile Arthritis Disease Activity Score (JADAS) or clinical JADAS cut-offs, 68% were classified as in MDA by all 3 criteria. Similarly, 44% of 633 children with CID defined by either Wallace criteria or the JADAS cut-off were in CID according to both criteria.Conclusion: In a large JIA prospective inception cohort, a majority of patients have evidence of persistent disease activity after one year. Published criteria to capture MDA and CID do not always identify the same groups of patients. This has significant implications when defining and applying treat-to-target strategies.

Introduction: Many children with juvenile idiopathic arthritis (JIA) continue to have active disease into adulthood. Adults with JIA are a heterogeneous group and the effects of tumour necrosis factor-inhibitor (TNFi) therapies are not well described. This analysis aims to describe treatment outcomes among patients with JIA starting TNFi for the first time in adulthood. Methods: Patients with arthritis onset