OBJECTIVE Juvenile dermatomyositis (JDM) is a rare disease in children that is treatable, but patients may suffer from long-term effects of the disease. Clinical trials are needed to find better treatments for affected patients. Among validated tools for evaluating disease activity clinically is the disease activity score (DAS), but it is not routinely collected in all clinics. We developed a modified DAS (DASmod) which can be scored using data routinely collected by our clinical staff, and has been used in previous studies. This study’s objective was to determine if our DASmod correlates with the validated DAS in patients with JDM. METHODS In this study, we used DASmod (scored 0-12) and DAS scores (scored 0-20) for patients with JDM in our clinic. We analysed the correlation between the DASmod and the validated DAS. RESULTS For 51 patients seen in our JDM clinic, the median (IQR) DASmod score was 2.0 (0, 4.0) and the median (IQR) DAS score was 3.0 (0, 5.5). Scores on the two tools were highly positively correlated (r = 0.94, p less then 0.001, 95% CI [0.89, 0.96]). The linear regression was significant (R2 = 0.88, F (1, 49) = 357.60, p less then 0.001) and in this dataset, the tools can be used interchangeably with the regression equation DAS score = -0.26 + 1.5*DASmod. CONCLUSION If the regression equation from this dataset is successfully tested against future datasets, then further research collaborations between centres that collect different data related to disease activity in children with JDM will be facilitated.OBJECTIVE To investigate an eight-year change in waist circumference (WC) with the risk of incident low physical function over one-year in adults with or at risk of knee osteoarthritis (OA). METHODS Data from the Osteoarthritis Initiative (OAI) were used. Change in WC was measured from study enrollment (0-month) to the 96-month visit and classified as Increase (≥ 5cm gain) or Maintain ( less then 5cm gain). We identified WHO risk category based on WC at study enrollment as Large WC (males ≥ 102cm, females ≥ 88cm) or Small WC (males less then 102cm, females less then 88cm). The outcome was incident low physical function (≥ 28 WOMAC physical function subscale) at the 108-month visit. To investigate the association of the 8-year change in WC with the risk of low physical function, we calculated risk ratios 95% confidence intervals [95%CI and adjusted for potential confounders. We repeated the analyses stratified by the WHO disease risk category. RESULTS The Increase WC group had 1.43 [1.04, 1.96] times the risk of incident low physical function compared to adults in the Maintain WC group. Adults with a Large at baseline WC, who increased WC, had 1.55 [1.00, 2.37] times the risk of incident low physical function compared to those who maintained WC. XAV-939 research buy Adults with a Small WC at baseline, who increased WC, had 1.97 [0.84, 4.63] times the risk compared to the who maintained WC. CONCLUSION Increasing WC increases the risk of incident low physical function in the following year. Maintaining WC may mitigate developing low physical function.OBJECTIVE The present study aimed to identify trajectories of physical activity (PA) components (frequency, duration, intensity, type) and screen-based sedentary behavior (SB) as well as baseline predictors of each trajectory in patients with hip and/or knee OA. METHODS We included 878 patients with a 5-year follow-up from the KHOALA cohort. PA and SB were measured by the Modifiable Activity Questionnaire. We used group-based trajectory analysis to identify the trajectories of PA components and screen-based SB, and multivariable logistic regression to determine predictors of the identified trajectories. RESULTS Two groups of trajectories were identified for each PA component and three for SB. High and decreasing PA duration was associated with female sex (odds ratio [OR]=0.3 [95% confidence interval [CI] 0.1-0.5]) as was low and stable than high and decreasing prevalence of weight-bearing activities (OR=0.6 [0.4-0.9]). Patients with impaired patient-reported outcome measure and obese patients often featured low versus high and decreasing prevalence of weightbearing activities. Predictors of moderate and high versus low and slightly increasing screenbased SB trajectories were male sex, age less then 60 years, single status (OR=1.5 [1.1-2.1]), obesity (OR=2.1 [1.4-3.1]), smoking (OR=2.0 [95% CI 1.1-3.7]), and less-physical jobs. Predictors of moderate and high versus low screen-based SB trajectories were all sociodemographic male sex, age less then 60 years, single status, obesity, smoking and less-physical jobs. CONCLUSION Sociodemographic and clinical predictors of trajectories vary between PA components they are mainly associated with PA frequency and type. No clinical characteristics were associated with screen-based SB.OBJECTIVE Scleroderma renal crisis (SRC) is a life-threatening complication of systemic sclerosis (SSc) strongly associated with anti RNA polymerase III antibody (ARA) autoantibodies. We explore genetic susceptibility and altered protein expression in renal biopsy specimens in ARA positive SRC. METHODS ARA-positive patients (n=99) with at least 5 years’ follow-up (49% with a history of SRC) were selected from a well-characterised SSc cohort (n=2254). Cases were genotyped using the Illumina Human Omni-express chip. Based on initial regression analysis, nine SNPs were chosen for validation in a separate cohort of 256 ARA+ patients (40 with SRC). Immunostaining of tissue sections from SRC or control kidney was used to quantify expression of candidate proteins based upon genetic analysis of the discovery cohort. RESULTS Analysis of 641,489 SNPs suggested association of POU2F1 (rs2093658; 1.98×10-5), CTNND2 (rs1859082; p=7.14 x 10-5), HECW2 (rs16849716; p=1.2 x 10-4) and GPATCH2L (rs935332; p=4.92 x 10-5) with SRC. Furthermore, the validation cohort showed an association between rs935332 within the GPATCH2L region, with SRC (p=0.025). Immunostaining of renal biopsy sections showed increased tubular expression of GPATCH2L (p=0.026), and glomerular expression of CTNND2 (p=0.026) in SRC samples (n=8) compared with normal human kidney controls (n=8), despite absence of any genetic replication for the associated SNP. CONCLUSION Increased expression of two candidate proteins GPATCH2L and CTNND2 in SRC compared with control kidney suggests a potential role in pathogenesis of SRC. For GPATCH2L this may reflect genetic susceptibility in ARA positive SSc based upon 2 independent cohorts.