Juvenile idiopathic arthritis (JIA) used to be a joint-destroying disease, but thanks to modern treatment strategies and medications, many patients with JIA today reach inactive disease. However, once disease remission is achieved, there is a lack of knowledge and recommendations regarding maintenance therapy. Drug-free remission is the ultimate goal in JIA, but withdrawal of medications increases the risk of disease flare. Clinical approaches vary widely, underscoring a need for knowledge about maintenance treatment strategies that allow for safe tapering and withdrawal of medications in JIA patients in sustained remission. The MOVE-JIA study is a randomized, controlled trial with the primary objective to compare the effect of two different treatment withdrawal strategies, to a stable dose of methotrexate (MTX) and tumor necrosis factor inhibitors (TNFi), based on the risk of flares in children and adolescents with JIA with sustained inactive disease. A key secondary objective is the proportion of children with disease flare compared between the two withdrawal groups. In this investigator-initiated multicenter, randomized, 3-grouped, parallel, open-label, noninferiority trial, treating physicians at seven Norwegian pediatric rheumatology hospital centers will include 150 patients with JIA. Key eligibility criteria are as follows: Fulfilment of the International League of Associations for Rheumatology (ILAR) classification criteria for non-systemic JIA, inactive disease for ≥ 12 months documented at a minimum of 2 consecutive visits, and no active uveitis for ≥ 24 months under treatment with stable doses of MTX and TNFi. They will be randomized in a 1:1:1 ratio to (A) stable treatment, (B) methotrexate withdrawal, or (C) TNFi withdrawal. Randomization will be stratified for JIA subtype and study center. For patients in group B and C who are still in remission after 12 months, a new randomization will be performed for complete medication withdrawal for the next 12 months. After 24 months, medication adjustments will be done with shared decision-making. The primary endpoint is the rate of disease flare compared between the drug withdrawal groups and the stable treatment group between baseline and 12 months follow-up. The key secondary endpoint is the proportion with disease flare compared between the two withdrawal groups. Incidence and severity of adverse events will be monitored. The results from the MOVE-JIA trial will present an evidence-based treatment strategy for JIA patients with inactive disease. The trial will also give us knowledge about regaining disease remission after flares and possibilities of drug-free remission. All outcomes from the trial will provide a scientific basis for optimized JIA care and result in new treatment recommendations. EU CT 2024–512017–12–00. Registered on October 24th, 2024; ClinicalTrials.gov NCT06653634. Registered on October 24th, 2024. URL: Study Details | NCT06653634 | Optimizing Treatment for Patients With Juvenile Idiopathic Arthritis in Sustained Remission: The MOVE–JIA Trial | ClinicalTrials.gov. Date of first recruitment: October 24th, 2024.

Proinflammatory cytokines are central to disease mechanisms and important therapeutic targets in inflammatory chronic diseases. This exploratory study aimed to compare cytokine concentrations in saliva, serum, and temporomandibular joint (TMJ) synovial fluid in children with juvenile idiopathic arthritis (JIA) and controls. In this cross-sectional study, we included consecutive children with JIA and TMJ arthritis, planned for a TMJ corticosteroid injection, and non-JIA controls from three different centers in Norway. Data on demographics, disease activity, presence of TMJ arthritis, and medication were obtained. Samples of unstimulated saliva, serum, and TMJ synovial fluid were collected. The amount of recovered synovial fluid in each sample, collected by the push-and-pull technique, was quantified with the hydroxocobalamin method. Cytokine levels were analyzed using Luminex xMAP technology. Fifteen patients with JIA and TMJ arthritis (JIA-TMJ) (median age 15.0 (interquartile range (IQR) 11.0–16.0) years) and 34 controls (median age 13.0 (IQR 9.8–15.0) years) were consecutively recruited. Samples of saliva (JIA-TMJ, n = 13, and controls, n = 28), serum (JIA-TMJ, n = 11, and controls, n = 16), and TMJ synovial fluid (JIA-TMJ, n = 8) were collected. In saliva from JIA-TMJ, we found significantly higher levels of the cytokines IL-1β, IL-4, IL-5, IL-9, IL-10, IL-12, IL-13, IL-17, Eotaxin, FGF basic, GM CSF, PDGF bb, TNF, and RANTES, while IP-10 was found in significantly lower concentration compared to controls. In serum, there were no significant differences in these cytokine concentrations between JIA-TMJ and controls. Three TMJ synovial samples fulfilled the strict sampling criteria and were included in the analysis. The level of detected cytokines in TMJ synovial samples was higher in JIA-TMJ compared to controls, as described in a previous Nordic study. In this exploratory study, several proinflammatory cytokines were found in higher concentrations in saliva in JIA-TMJ compared to saliva from the controls. No differences were seen in serum between the groups. Some pro- and anti-inflammatory cytokines detected in JIA-TMJ synovial fluid were found in higher concentrations compared to TMJ synovial fluid from healthy adult reference data.

Objective To assess the validity of an ultrasonographic scoring system in juvenile idiopathic arthritis (JIA) by comparing ultrasound detected synovitis with whole-body MRI and clinical assessment of disease activity.Methods In a cross-sectional study, 27 patients with active JIA underwent clinical 71-joints examination, non-contrast enhanced whole-body MRI and ultrasound evaluation of 28 joints (elbow, radiocarpal, midcarpal, metacarpophalangeal 2–3, proximal interphalangeal 2–3, hip, knee, tibiotalar, talonavicular, subtalar and metatarsophalangeal 2–3). One rheumatologist, blinded to clinical findings, performed ultrasound and scored synovitis (B-mode and power Doppler) findings using a semiquantitative joint-specific scoring system for synovitis in JIA. A radiologist scored effusion/synovial thickening on whole-body MRI using a scoring system for whole-body MRI in JIA. At patient level, associations between ultrasound synovitis sum scores, whole-body MRI effusion/synovial thickening sum scores, clinical arthritis sum scores, and the 71-joints Juvenile Arthritis Disease Activity Score (JADAS71) were calculated using Spearman’s correlation coefficients (rs). To explore associations at joint level, sensitivity and specificity were calculated for ultrasound using whole-body MRI or clinical joint examination as reference.Results Ultrasound synovitis sum scores strongly correlated with whole-body MRI effusion/synovial thickening sum scores (rs=0.74,p<0.01) and the JADAS71 (rs=0.71,p<0.01), and moderately with clinical arthritis sum scores (rs=0.57,p<0.01). Sensitivity/specificity of ultrasound in detecting synovitis were 0.57/0.96 and 0.55/0.96 using whole-body MRI or clinical joint examination as reference, respectively.Conclusion Our findings suggest that ultrasound is a valid instrument to detect synovitis, and that ultrasound synovitis sum scores can reflect disease activity and may be an outcome measure in JIA.

Background: Rheumatic diseases are associated with reduced bone mineral density (BMD) as measured by Dual Energy x-ray absorptiometry (DXA). Mixed connective tissue disease (MCTD) is a rare connective tissue disease, and about 25% of all cases have a pediatric presentation. One study has implied that osteoporosis is common in postmenopausal women with MCTD [1], but data regarding BMD and osteopenia are lacking in younger patients with the disease. Objectives: To assess the frequency of osteopenia in a cohort of juvenile mixed connective tissue disease (JMCTD) patients compared to a matched control group and to explore associations between lower BMD and disease related variables. Methods: BMD was measured in a cohort of 52 JMCTD patients with mean age 28.2 years (SD 10.3) and mean disease duration 16.2 years (SD 10.3), and 52 controls matched for age and gender. Forty-four (85%) were female, and Z-score was estimated in the 35 patients and 35 controls over 20 years of age. Inclusion criteria were fulfilment of the Kasukawa- or the Alarcon-Segovia criteria and symptom debut before the age of 18 years. Results: Osteopenia in at least one area of measurement defined as Z-score ≤ -1.0 was found in 23 (61%) of patients and 15 (43%) of controls over 20 years of age (p <0.01) (Table 1). The most common location of osteopenia was the distal radius (49% of patients and 23% of controls, p <0.01). Osteopenic values were found more often in patients than controls also in the lumbal columna L2-L4 (20% vs 9%, p <0.01), in the hip (32% vs 6%, p <0.01) and in the proximal radius (43% vs 17%, p <0.01). Osteoporotic values defined as Z-score ≤ -2.5 were found in four patients (11%) and one control. None had osteoporotic fractures.We found significant correlations between lower BMD and a high number of joints with active arthritis at diagnosis (spearman -0.36, p 0.03 in proximal radius, -0.38, p 0.03 in distal radius, -0,36, p 0.03 in the hip). A high number of joints with active arthritis at examination was significantly associated with lower BMD in the proximal radius (spearman r -0.40, p 0.02) and L2-L4 (spearman r -0.37, p 0.03). Low age at diagnosis was associated with lower BMD in proximal radius (spearman r - 0.46, p <0.01). We could not identify significant correlations between number of months treated with glucocorticosteroids and BMD values, nor did we find associations between prednisolone dose at time of diagnosis, 6 months after diagnosis or one year after diagnosis and BMD values after mean 16.2 years. No significant correlations between different measurements of disease activity and BMD were observed (physician global disease activity score, systemic lupus erythematosus disease activity index, Rodnan skin score). In multiple linear regression analyses the BMD scores for proximal radius were used as dependent variable, as this was the area with the most consequent low values in patients. Age at examination, age at disease debut, gender, and number of joints with active arthritis at diagnosis and at examination were treated as independent variables. Active joints at diagnosis (B -0.01, p<0.01) and age (B 0.004, p 0.02) was associated with low BMD score at time of examination. Conclusion: Osteopenia is common in JMCTD patients after long-term disease duration compared to matched controls. Lower BMD after long-term disease duration seems to be associated with a high number of joints with active arthritis. REFERENCES: [1] Bodolay E et. Al, Osteoporosis in MCTD, Clin Rheum. 2003. Acknowledgements: NIL. Disclosure of Interests: None declared.Table 1Osteopenia and osteoporotic values in JMCTD patients compared to controlsPatients >20 years (35)Controls >20 years (35)POsteopenia L2-L47 (20)3 (9)<0.01Osteopenia hip12 (32)2 (6)<0.01Osteopenia proximal radius15 (43)6 (17)<0.01Osteopenia distal radius17 (49)8 (23)<0.01Osteopenia in at least one location23 (66)15 (43)<0.01Osteoporotic values L2-L41 (3)0Osteoporotic values hip01 (3)Osteoporotic values distal radius4 (11)1 (3)Osteoporotic values proximal radius00Osteoporotic values in at least one location4 (11)1 (3)Numbers are in n(%)

OBJECTIVES:In long-term juvenile dermatomyositis (JDM), altered adipose tissue distribution and subclinical cardiac dysfunction have been described. Our aims were to compare adipokine levels in patients with JDM after long-term disease with controls, and explore associations between adipokines and (1) adipose tissue distribution and (2) cardiac function.METHODS:The study cohort included 59 patients with JDM (60% female, mean age 25.2 years, mean disease duration 16.9 years), and 59 age/sex-matched controls. Updated Pediatric Rheumatology International Trials Organization criteria for clinically inactive JDM were used to stratify patients into active (JDM-active) or inactive (JDM-inactive) disease groups. Lipodystrophy was clinically assessed in all patients. In all study participants, we measured adipose tissue distribution by dual-energy X-ray absorptiometry and cardiac function by echocardiography. Serum adipokines (adiponectin, apelin-12, lipocalin-2, leptin, visfatin and resistin) were analysed using ELISA.RESULTS:Patients with JDM had higher leptin levels compared with controls (p≤0.01). In JDM-active, apelin-12 and visfatin were higher compared with JDM-inactive (p≤0.05). In JDM-total and JDM-active, lower adiponectin correlated with lipodystrophy and total fat mass. Also, systolic dysfunction correlated with: lower adiponectin in JDM-total, JDM-inactive and JDM-active, and with lower apelin-12 in JDM-total and JDM-active and resistin in JDM-active (all p≤0.05). Lower adiponectin correlated with diastolic dysfunction in JDM-total and JDM-active.CONCLUSION:After long-term disease, leptin levels were unfavourably regulated in patients with JDM compared with controls, and apelin-12 and visfatin in JDM-active versus JDM-inactive. We found associations between adipokines and both adipose tissue distribution and cardiac systolic function in all patients with JDM, which was most prominent in patients with active disease.

Objectives Primary aims were to compare adipose tissue distribution in adult patients with juvenile-onset DM (JDM), with matched controls. Secondary aims were to explore how adipose tissue distribution is associated with cardio-metabolic status (cardiac dysfunction and metabolic syndrome) in patients. Methods Thirty-nine JDM patients (all aged >= 18 y, mean age 31.7 y and 51% female) were examined mean 22.7 y (s.d. 8.9 y) after disease onset and compared with 39 age/sex-matched controls. In patients, disease activity and lipodystrophy were assessed by validated tools and use of prednisolone noted. In all participants, dual-energy X-ray absorptiometry (DXA) and echocardiography were used to measure visceral adipose tissue (VAT)(g) and cardiac function, respectively. Risk factors for metabolic syndrome were measured and associations with adipose tissue distribution explored. For primary and secondary aims, respectively, P-values <= 0.05 and <= 0.01 were considered significant. Results Patients exhibited a 2.4-fold increase in VAT, and reduced HDL-cholesterol values compared with controls (P-values <= 0.05). Metabolic syndrome was found in 25.7% of the patients and none of the controls. Cardiac dysfunction (systolic and/or diastolic) was found in 23.7% of patients and 8.1% of controls (P = 0.07). In patients, VAT levels were correlated with age, disease duration and occurrence of metabolic syndrome and cardiac dysfunction. Occurrence of lipodystrophy (P = 0.02) and male sex (P = 0.04) tended to be independently associated with cardiac dysfunction. Conclusion Adults with JDM showed more central adiposity and cardio-metabolic alterations than controls. Further, VAT was found increased with disease duration, which was associated with development of cardio-metabolic syndrome.

OBJECTIVE:To compare the effectiveness of tumor necrosis factor inhibitors (TNFi) ± comedication and methotrexate (MTX) monotherapy between patients with adult juvenile idiopathic arthritis (JIA) and patients with rheumatoid arthritis (RA).METHODS:Adult patients with JIA and RA were identified from the Norwegian Antirheumatic Drug Register (NOR-DMARD) register. Disease activity measurements at baseline, 3, 6, and 12 months were compared between patients with JIA and RA starting (1) TNFi and (2) MTX monotherapy, using age- and gender-weighted analyses. We calculated differences between JIA and RA in mean changes in Disease Activity Score in 28 joints (DAS28), Clinical Disease Activity Index (CDAI), and Simplified Disease Activity Index (SDAI), among other disease activity measures. DAS28, CDAI, SDAI, and American College of Rheumatology (ACR)/European Alliance of Associations for Rheumatology (EULAR) remission rates at 3, 6, and 12 months, as well as 6- and 12-month Lund Efficacy Index (LUNDEX)-corrected rates, were calculated.RESULTS:We identified 478 patients with JIA (TNFi/MTX monotherapy, n = 358/120) and 4637 patients with RA (TNFi/MTX monotherapy, n = 2292/2345). Patients with JIA had lower baseline disease activity compared to patients with RA across treatment groups. After baseline disease activity adjustment, there were no significant differences in disease activity change from baseline to 3, 6, and 12-months of follow-up between patients with JIA and RA for either treatment group. Twelve-month remission rates were similar between groups based on DAS28 (TNFi: JIA 55.2%, RA 49.5%; MTX monotherapy: JIA 45.3%, RA 41.2%) and ACR/EULAR remission criteria (TNFi: JIA 20.4%, RA 20%; MTX monotherapy: JIA 17%, RA 12.7%). Median drug survival (yrs) was similar for JIA and RA in both treatment groups (TNFi: JIA 1.2, RA 1.4; MTX monotherapy: JIA 1.3, RA 1.6).CONCLUSION:TNFi and MTX monotherapy are effective in adult JIA, with similar effectiveness to that shown in RA.

Objectives To describe power Doppler (PD) ultrasound findings in joint regions with B-mode (BM) synovitis using a standardised scanning protocol and scoring system in patients with juvenile idiopathic arthritis (JIA). Further, to examine associations between PD findings and BM synovitis, clinical arthritis, patient characteristics and disease activity.Methods In this cross-sectional study, one experienced ultrasonographer, blinded to clinical findings, performed ultrasound examinations in 27 JIA patients with suspected clinical arthritis. The elbow, wrist, metacarpophalangeal 2–3, proximal interphalangeal 2–3, knee, ankle and metatarsophalangeal 2–3 joints were assessed bilaterally and scored semiquantitatively (grades 0–3) for BM and PD findings using a joint-specific scoring system with reference atlas. Multilevel mixed-effects ordered regression models were used to explore associations between PD findings and BM synovitis, clinical arthritis, age, sex, JIA subgroups, disease duration and 10-joint Juvenile Arthritis Disease Activity Score (JADAS10).Results Twenty-one girls and six boys, median age (IQR) 8 years (6–12 years) were included. Overall, 971 joint regions were evaluated by ultrasound, 129 had BM synovitis and were assessed for PD. PD findings were detected in 45 joint regions (34.9%), most frequently in the parapatellar recess of the knee (24.4%). Increasing PD grades were associated with higher BM grades (OR=5.0,p<0.001) and with clinical arthritis (OR=7.4,p<0.001) but not with age, sex, JIA subgroups, disease duration or JADAS10.Conclusion Increasing severity of PD findings were significantly associated with BM synovitis and with clinical arthritis. This suggests that PD signals detected using a standardised ultrasound examination and scoring system can reflect active disease in JIA patients.

BackgroundRheumatic diseases are associated with reduced bone mineral density (BMD) as measured by Dual Energy x-ray absorptiometry (DXA). Mixed connective tissue disease (MCTD) is a rare connective tissue disease, and about 25% of all cases have a pediatric presentation. One study implies that osteoporosis is common in postmenopausal women with MCTD (1), but data regarding BMD and osteopenia are lacking in younger patients with the disease.ObjectivesTo assess the BMD in a cohort of juvenile mixed connective tissue disease (JMCTD) patients compared to a matched control group.MethodsBMD was measured in a cohort of 52 JMCTD patients with mean age 28.2 years (SD 10.3) and mean disease duration 16.2 years (SD 10.3), and 52 controls matched for age and gender. Inclusion criteria were fulfilment of the Kasukawa- or the Alarcon-Segovia criteria and symptom debut before the age of 18 years.ResultsPatients and controls did not differ in age, body mass index, smoking habits, level of physical activity or vitamin D status (Table 1). Forty-four (85%) were female. Patients were shorter and had lower body weight compared to controls. None had had osteoporotic fractures. Patients over 20 years of age at examination had significantly lower BMD in the spine, femoral neck, proximal- and distal radius. Patients also had lower T-score compared to matched controls.Table 1.VariableJMCTD patients (n=52)Controls (n=52)P valueBMI, kg/m222.7 (3.5)23.4 (3.0)0.239Height, cm166.0 (7.5)170.1 (8.5)0.016Weight, kg63.0 (12.1)68.1 (12.1)0.033Never smokers, n (%)35 (67)34 (65)0.83Moderate physical activity (hours/week), median (range)2.0 (0-28)1.2 (0-30.0)0.278Vitamin D*, nmol/l, median (range)50.0 (27-106)51.5 (23-115)0.829BMD, gm/cm2Spine L2-L41.19 (0.13)1.25 (0.10)0.110< 20 years (n = 14, 27%)1.14 (0.21)1.17 (0.11)0.496>20 years (n = 38, 73%)1.19 (0.12)1.26 (0.10)0.018Femoral neck0.97 (0.12)1.04 (0.09)0.006>20 years0.97 (0.12)1.04 (0.09)0.002Proximal radius0.79 (0.12)0.86 (0.09)0.007>20 years0.79 (0.12)0.86 (0.09)0.013Distal radius0.40 (0.07)0.50 (0.10)<0.001>20 years0.41 (0.07)0.51 (0.10)<0.001T-score >20 yearsSpine L2-L4, median (range)-0.40 (-2.3 to 2.0)0.29 (-1.4 to 1.8)0.006Femoral neck, median (range)-0.50 (-2.2 to 2.1)0.40 (-1.7 to 1.6)0.007Proximal radius, median (range)-0.90 (-0.2 to 1.6)-0.30 (-0.2 to 1.3)0.044Distal radius, median (range)-1.0 (-3.0 to 1.3)0.20 (-2.6 to 3.8)<0.001All values are mean (SD) unless otherwise statedConclusionBone mineral density was impaired in JMCTD patients after 16 years disease duration compared to matched controls.References[1]Bodolay E et. Al, Osteoporosis in mixed connective tissue disease, Clin Rheumatol. 2003Disclosure of InterestsNone declared

Background Manual assessment of bone marrow signal is time-consuming and requires meticulous standardisation to secure adequate precision of findings. Objective We examined the feasibility of using deep learning for automated segmentation of bone marrow signal in children and adolescents. Materials and methods We selected knee images from 95 whole-body MRI examinations of healthy individuals and of children with chronic non-bacterial osteomyelitis, ages 6–18 years, in a longitudinal prospective multi-centre study cohort. Bone marrow signal on T2-weighted Dixon water-only images was divided into three color-coded intensity-levels: 1 = slightly increased; 2 = mildly increased; 3 = moderately to highly increased, up to fluid-like signal. We trained a convolutional neural network on 85 examinations to perform bone marrow segmentation. Four readers manually segmented a test set of 10 examinations and calculated ground truth using simultaneous truth and performance level estimation (STAPLE). We evaluated model and rater performance through Dice similarity coefficient and in consensus. Results Consensus score of model performance showed acceptable results for all but one examination. Model performance and reader agreement had highest scores for level-1 signal (median Dice 0.68) and lowest scores for level-3 signal (median Dice 0.40), particularly in examinations where this signal was sparse. Conclusion It is feasible to develop a deep-learning-based model for automated segmentation of bone marrow signal in children and adolescents. Our model performed poorest for the highest signal intensity in examinations where this signal was sparse. Further improvement requires training on larger and more balanced datasets and validation against ground truth, which should be established by radiologists from several institutions in consensus.

Objective: To describe the findings of focal high signal on T2 weighted (T2W) images of the bone marrow in the axial skeleton as assessed by whole-body MRI in healthy and asymptomatic children and adolescents. Material and methods: We assessed the bone marrow of the mandible, shoulder girdle, thorax, spine, and pelvis on water-only Dixon T2W sequences as part of a whole-body MRI protocol in 196 healthy and asymptomatic children aged 5-19 years. Intensity (0-2 scale) and extension (1-4 scale) of focal high signal areas in the bone marrow were scored and divided into minor or major findings, based on intensity and extension to identify the potentially conspicuous lesions in a clinical setting. Results: We registered 415 areas of increased signal in the axial skeleton whereof 75 (38.3%) were major findings. Fifty-eight (29.6%) individuals had at least one major finding, mainly located in the pelvis (54, 72%). We found no differences according to gender. The number of minor findings increased with age (p = 0.020), but there were no significant differences in the number of major findings. The most conspicuous findings were in the pelvis, spine and sternum. Conclusion: Non-specific bone marrow T2W hyperintensities in the axial skeleton are frequently detected on whole-body MRI in healthy, asymptomatic children. Awareness of this is important as some findings may resemble clinically silent lesions in children with suspected multifocal skeletal disease.

OBJECTIVE:Juvenile idiopathic arthritis (JIA) is the most common chronic immune-mediated joint disease among children and encompasses a heterogeneous group of immune-mediated joint disorders classified into 7 subtypes according to clinical presentation. However, phenotype overlap and biologic evidence suggest a shared mechanistic basis between subtypes. This study was undertaken to systematically investigate shared genetic underpinnings of JIA subtypes. METHODS:We performed a heterogeneity-sensitive genome-wide association study encompassing a total of 1,245 JIA cases (classified into 7 subtypes) and 9,250 controls, followed by fine-mapping of candidate causal variants at each genome-wide significant locus, functional annotation, and pathway and network analysis. We further identified candidate drug targets and drug repurposing opportunities by in silico analyses. RESULTS:In addition to the major histocompatibility complex locus, we identified 15 genome-wide significant loci shared between at least 2 JIA subtypes, including 10 novel loci. Functional annotation indicated that candidate genes at these loci were expressed in diverse immune cell types. CONCLUSION:This study identified novel genetic loci shared by JIA subtypes. Our findings identified candidate mechanisms underlying JIA subtypes and candidate targets with drug repurposing opportunities for JIA treatment.

Objective To compare body composition parameters in patients with long-standing JDM and controls and to explore associations between body composition and disease activity/inflammation, muscle strength, health-related quality of life (HRQoL) and cardiometabolic measures. Methods We included 59 patients (median disease duration 16.7 y; median age 21.5 y) and 59 age- and sex-matched controls in a cross-sectional study. Active and inactive disease were defined by the PRINTO criteria. Body composition was assessed by total body DXA, inflammation by high-sensitivity CRP (hs-CRP) and cytokines, muscle strength by the eight-muscle manual muscle test, HRQoL by the 36-item Short Form Health Survey physical component score and cardiometabolic function by echocardiography (systolic and diastolic function) and serum lipids. Results DXA analyses revealed lower appendicular lean mass index (ALMI; reflecting limb skeletal muscle mass), higher body fat percentage (BF%) and a higher android fat:gynoid fat (A:G) ratio (reflecting central fat distribution) in patients than controls, despite similar BMI. Patients with active disease had lower ALMI and higher BF% than those with inactive disease; lower ALMI and higher BF% were associated with inflammation (elevated monocyte attractant protein-1 and hs-CRP). Lower ALMI was associated with reduced muscle strength, while higher BF% was associated with impaired HRQoL. Central fat distribution (higher A:G ratio) was associated with impaired cardiac function and unfavourable serum lipids. Conclusion Despite normal BMI, patients with JDM, especially those with active disease, had unfavourable body composition, which was associated with impaired HRQoL, muscle strength and cardiometabolic function. The association between central fat distribution and cardiometabolic alterations is a novel finding in JDM.

To describe the appearances of bone marrow in the appendicular skeleton on fat-suppressed T2-weighted sequences as assessed by whole-body MRI in healthy and asymptomatic children and adolescents.Following ethical approval, we assessed the bone marrow of the extremities on water-only Dixon T2-weighted images as part of a whole-body MRI in 196 healthy and asymptomatic children aged 5-19 years. Based on a newly devised and validated scoring system, we graded intensity (0-2 scale) and extension (1-4 scale) of focal high signal bone marrow areas, and divided them into minor or major findings, based on intensity and extension, reflecting their potential conspicuousness in a clinical setting.In the upper extremity, we registered 366 areas with increased signal whereof 79 were major findings. In the lower extremities there were 675 areas of increased signal of which 340 were major findings. Hundred-and-fifteen (58.79%) individuals had at least one major finding, mainly located in the hand and proximal humerus, and the feet and knees. We found no differences according to gender, reported hours of sports activity, handedness, or age group, except for more minor findings in the upper extremities amongst 15-18-year-olds as compared to those aged 5-8 years.Focal areas of high signal intensity on whole-body MRI, T2-weighted fat suppressed images that, in a clinical setting could cause concern, were seen in more than half of healthy, asymptomatic children and adolescents. Awareness of this is important when interpreting whole-body MRI in this age group, particularly in the assessment of clinically silent lesions.

ObjectiveTo explore quality of life (QOL) using the individualized Patient Generated Index (PGI) in young adults who were diagnosed with juvenile idiopathic arthritis (JIA) in childhood, and to examine associations between PGI ratings and standardized health-related outcome measures.MethodsPatients (N = 79, mean age 25.1 [SD 4.2] yrs, 72% female) completed the PGI and the standardized measures: Health Assessment Questionnaire–Disability Index, 12-item Short Form Health Survey (SF-12; physical and mental health-related QOL [HRQOL]), Brief Pain Inventory (pain severity and interference), 5-item Hopkins Symptom Checklist, and visual analog scale for fatigue. Information on morning stiffness, medications, and demographics was also collected. Patients were compared to 79 matched controls.ResultsThe most frequently nominated areas of importance for patients’ personally generated QOL (assessed by PGI) were physical activity (n = 38, 48%), work/school (n = 31, 39%), fatigue (n = 29, 37%) and self-image (n = 26, 33%). Nomination of physical activity was associated with older age, morning stiffness, and more pain interference. Nomination of fatigue was associated with current use of disease-modifying antirheumatic drugs, whereas nomination of self-image was associated with polyarticular course JIA and pain interference. Nomination of work/school was not associated with other factors. Higher PGI scores (indicating better QOL) correlated positively with all SF-12 subscales except role emotional, and negatively with disability, pain severity, pain interference, and morning stiffness. Compared to controls, patients had more pain, poorer physical HRQOL, and less participation in full-time work or school.ConclusionPhysical activity, work/school, fatigue, and self-image were frequently nominated areas affecting QOL in young adults with JIA. The PGI included aspects of QOL not covered in standardized measures.

This study aimed at exploring the association between detectable cardiac and pulmonary involvement in long-term juvenile dermatomyositis (JDM) and to assess if patients with cardiac and pulmonary involvement differ with regard to clinical characteristics. 57 JDM patients were examined mean 17.3 (10.5) years after disease onset; this included clinical examination, myositis specific/associated autoantibodies (immunoblot), echocardiography, pulmonary function tests and high-resolution computed tomography. Cardiac involvement was defined as diastolic and/or systolic left ventricular dysfunction and pulmonary involvement as low diffusing capacity for carbon monoxide, low total lung capacity and/or high-resolution computed tomography abnormalities. Patients were stratified into the following four groups: (i) no organ involvement, (ii) pulmonary only, (iii) cardiac only, and (iv) co-existing pulmonary and cardiac involvement. Mean age was 25.7 (12.4) years and 37% were males. One patient had coronary artery disease, seven had a history of pericarditis, seven had hypertension and three had known interstitial lung disease prior to follow-up. There was no association between cardiac (10/57;18%) and pulmonary (41/57;72%) involvement ( p = 0.83). After stratifying by organ involvement, 21% of patients had no organ involvement; 61% had pulmonary involvement only; 7% had cardiac involvement only and 11% had co-existing pulmonary or cardiac involvement. Patients with co-existing pulmonary or cardiac involvement had higher disease burden than the remaining patients. Patients with either cardiac or pulmonary involvement only, differed in clinical and autoantibody characteristics. We found no increased risk of developing concomitant cardiac/pulmonary involvement in JDM. Our results shed light upon possible different underlying mechanisms behind pulmonary and cardiac involvement in JDM.