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To develop evidence based points to consider the use of imaging in the diagnosis and management of juvenile idiopathic arthritis (JIA) in clinical practice. The task force comprised a group of paediatric rheumatologists, rheumatologists experienced in imaging, radiologists, methodologists and patients from nine countries. Eleven questions on imaging in JIA were generated using a process of discussion and consensus. Research evidence was searched systematically for each question using MEDLINE, EMBASE and Cochrane CENTRAL. Imaging modalities included were conventional radiography, ultrasound, MRI, CT, scintigraphy and positron emission tomography. The experts used the evidence obtained from the relevant studies to develop a set of points to consider. The level of agreement with each point to consider was assessed using a numerical rating scale. A total of 13 277 references were identified from the search process, from which 204 studies were included in the systematic review. Nine points to consider were produced, taking into account the heterogeneity of JIA, the lack of normative data and consequent difficulty identifying pathology. These encompassed the role of imaging in making a diagnosis of JIA, detecting and monitoring inflammation and damage, predicting outcome and response to treatment, use of guided therapies, progression and remission. Level of agreement for each proposition varied according to the research evidence and expert opinion. Nine points to consider and a related research agenda for the role of imaging in the management of JIA were developed using published evidence and expert opinion.

Background Skeletally immature osteochondral lesions of the talus (OLTs) have a significant impact on the health status and quality of life of pediatric patients and the involved family. the current literature showed success in 4 out of 10 patients but it is currently unknown which type of non-operative management showed better clinical- and radiological outcomes. The aim of this study is to compare immobilization and supervised rehabilitation with a ‘skillful’’ neglect in the treatment for skeletally immature patients with an OLT. The hypothesis is that a period of immobilization and supervised rehabilitation will lead to better clinical and radiological outcomes compared to ‘’skillful’’ neglect. Methods Multicenter, prospective, comparative study. Skeletally immature children with an OLT will be assigned to the intervention or control group after a shared decision-making process. Patients in the intervention group will undergo a 4-week period of immobilization with normal casting and non-weightbearing, which is followed by 4 weeks of immobilization with a removable cast and weight bearing boot. Afterwards, they will receive a protocolled period of rehabilitation under supervision of a physical therapist. The control group will have a ‘skillful’’ neglect treatment. The main study outcome is the difference between the two groups on the Oxford Ankle and Foot Questionnaire for Children (OxAFQ-C). Secondary study outcomes are radiologic changes in terms of morphology and lesion size. Numeric Rating Scale (NRS) during weight bearing and quality of life measured with a Pediatrics Quality of Life (Peds-QL) and EuroQol-5 Dimension youth (EQ-5D-y). Discussion This protocol reports on the study design of the CARE Study and it aims to setup a study for evaluating different types of non-operative management in pediatric patients suffering an OLT. This study will compare clinical and radiological outcomes between two different non-operative strategies for treating OLTs in the skeletally immature population. Based on the results of this study, an evidence-based treatment protocol for non-operative management for pediatric OLTs can be provided. Trial registration This study is registered in the International Clinical Trial Registry Platform (ICTRP) with trial number NLOMON54282, date of registration 05192023.

New bone formation in psoriatic arthritis (PsA) can be observed in all musculoskeletal disease domains and can lead to disability due to ankylosis and bridging syndesmophytes [1, 2]. The positive effect of anti-tumor necrosis (anti-TNF) therapy on radiographic damage (e.g. erosion and joint space narrowing) is well known, but there are contradictory findings on its effects on new bone formation [3, 4]. Imaging of new bone formation in PsA is pivotal in order to ascertain the extent and potential therapeutic effects on structural changes at an early stage of treatment. Our group recently demonstrated that molecular new bone formation can be observed in all PsA disease domains, using 18F-Fluoride PET-CT scans. Moreover, the ability of this imaging technique to detect early therapeutic effects in ankylosing spondylitis patients was also demonstrated by our group [5]. To investigate whether 18F-Fluoride PET-CT scans can detect a change in 18F-Fluoride uptake, reflecting a change in new bone formation, after 12 weeks of anti-TNF therapy in clinically active PsA patients. Nine patients (male 4/9, median age 47 (IQR 19)) with PsA fulfilling CASPAR criteria or a clinical diagnosis according to the treating rheumatologist and clinically active disease including ≥1 clinically active enthesitis site were included. In each patient, a whole body 18F-Fluoride PET-CT scan was performed prior to and 12 weeks after starting anti-TNF therapy. Scans were independently assessed for PET-positive lesions (dichotomous) by two readers (blinded for clinical data). Fixed sized volumes of interest were drawn on top of visual PET positive lesions as well as on locations where visual PET positive lesions appeared or disappeared before or after treatment. Standardized uptake values corrected for individual integrated whole blood activity concentration (SUVAUC) were used for quantitative analysis. CT was used for anatomical reference. Combining all PET-positive lesions of all patients, a total of 153 lesions were observed at baseline (89, 30 and 34 in peripheral joints, entheses and spine, respectively). At week 12, a total of 119 PET-positive lesions were observed (69, 13 and 37 in peripheral joints, entheses and the spine, respectively). Grouping all lesions of all patients, there was a decrease in the mean SUVAUC between baseline (1.578, SD 0.99) and week 12 of anti-TNF treatment (1.230, SD 0.85) (Figure 1A). Similar results were observed when grouping all lesions of all patients for separate disease domains. Mean SUVAUC changes between baseline and 12 weeks were -0.294 (SD 0.88), -0.357 (SD 0.69) and -0.300 (SD 0.67) for respectively peripheral joints (Figure 1B), entheses (Figure 1C) and the spine (Figure 1D). 18F-Fluoride PET-CT detected a change in new bone formation in PsA domains during anti-TNF therapy as early as 12 weeks post-treatment. The data point at a mean decrease of new bone formation in peripheral joints, entheses and axial skeleton. [1]de Jongh J et al. (18)F-sodium fluoride PET-CT visualizes both axial and peripheral new bone formation in psoriatic arthritis patients. Eur J Nucl Med Mol Imaging. 2022. [2]Lories RJ et al. Modulation of bone morphogenetic protein signaling inhibits the onset and progression of ankylosing enthesitis. J Clin Invest. 2005;115(6):1571-9. [3]Goulabchand R et al. Effect of tumour necrosis factor blockers on radiographic progression of psoriatic arthritis: a systematic review and meta-analysis of randomised controlled trials. Ann Rheum Dis. 2014;73(2):414-9. [4]Sieper J et al. Critical appraisal of assessment of structural damage in ankylosing spondylitis: implications for treatment outcomes. Arthritis Rheum. 2008;58(3):649-56. [5]Bruijnen STG et al. Bone formation in ankylosing spondylitis during anti-tumour necrosis factor therapy imaged by 18F-fluoride positron emission tomography. Rheumatology (Oxford). 2018;57(4):631-8. NIL. Jerney de Jongh: None declared, Gerben C.J. Zwezerijnen: None declared, Robert Hemke: None declared, Maqsood Yaqub: None declared, Marleen G.H. van de Sande Speakers bureau: UCB, Novartis, Janssen, Consultant of: UCB, Novartis, Abbvie, Eli Lily, Grant/research support from: UCB, Novartis, Eli Lily, Arno Van Kuijk Consultant of: UCB, Janssen, Grant/research support from: Janssen, Novartis, Pfizer, Alexandre Voskuyl Consultant of: GSK, Astra Zeneca, Grant/research support from: Boehringer Ingelheim, Conny J. van der Laken Speakers bureau: Janssen, Abbvie, Pfizer, Novartis, UCB Pharma, Galapagos, Paid instructor for: Lilly, Galapagos, Consultant of: UCB Pharma, GSK, Novartis, Grant/research support from: Pfizer, Abbvie, Novartis, GSK, UCB Pharma [Display omitted]

Introduction Duodenal Mucosal Resurfacing (DMR) is an endoscopic ablation technique aimed at improving glycaemia and metabolic health in patients with type 2 diabetes mellitus (T2DM). DMR has an insulin sensitizing effect in patients with T2DM. Reducing hyperinsulinemia can improve cardiovascular health. In the INSPIRE trial, we combined a single DMR with a glucagon-like-peptide-1 receptor agonist (GLP-1RA) and demonstrated elimination of insulin treatment in 69% of patients at 6 months and 53% of patients at 18 months while improving glycaemic control and metabolic health. We hypothesized that this treatment approach is associated with improved cardiovascular health, by reducing hyperinsulinemia. Methods Before and 6 months after starting the combination treatment to replace insulin, the following assessments were performed to evaluate cardiovascular health: magnetic resonance imaging (MRI) to measure abdominal visceral adipose tissue volume, ambulatory 24 h blood pressure (ABPM) analysis, postprandial insulin and triglycerides, fasting lipid panel and urine microalbumin. The Atherosclerotic Cardiovascular Disease (ASCVD) score was calculated to estimate 10-year risk of cardiovascular disease or stroke and the diabetes lifetime-perspective prediction (DIAL) score was calculated to estimate years free of cardiovascular disease. Results Six months after replacing exogenous insulin by DMR and GLP-1RA, visceral adipose tissue decreased significantly by 24%. Postprandial triglyceride and insulin concentrations decreased significantly ( p  < 0.001), as did total cholesterol (from median 3.64 (IQR 3.34–4.89) to 3.48 (3.18–3.97) mmol/l, p  = 0.008), LDL (from median 1.92 (IQR 1.49–2.30) to 1.79 (1.49–2.08 mmol/l, p  = 0.044), and urine microalbumin (from median 7 (IQR 3–27) to 4 (3–8) mg/l, p  = 0.018). All daytime blood pressure values decreased significantly. The ASCVD 10-year risk score decreased (from median 13.6 (IQR 5.7–26.0) to 11.5 (4.2–22.5) %, p  = 0.030)) and the DIAL score increased (from median 82 (IQR 81–83) to 83 (81–84) years, ( p  = 0.039)). Discussion The combination of DMR and GLP-1RA to replace insulin therapy in patients with T2DM is associated with a positive effect on multiple parameters of cardiovascular health. Taken together, they show a pattern of overall improvement in cardiovascular health, as evidenced by decreased risk scores for cardiovascular complications. However, it is not yet clear whether these improvements will translate into a true reduction in cardiovascular events.

Objective To explore how intramuscular injection of botulinum toxin A (BTA) affects the lateral abdominal wall (LAW) musculature, abdominal- and hernia dimensions, and muscle structure on computed tomography (CT) in patients scheduled for complex abdominal wall reconstruction (CAWR). Methods Retrospective analysis of prospectively registered patients who received bilateral intramuscular BTA injections into all three muscles of the LAW. Only patients for which a CT was available before and 3–6 weeks after BTA treatment prior to surgery were analyzed. Results Fifty-two patients were analyzed. Median hernia width in all patients decreased with 0.4 cm (IQR − 2.1;0.6) ( p  = 0.023). Median intra-abdominal transverse diameter increased with 0.9 cm (IQR − 0.2;3.3) ( p  = 0.001) and the intra-abdominal anterior–posterior diameter decreased with 0.5 cm (IQR − 1.3;0.5) ( p  = 0.017), making the abdomen more oval. Median LAW muscle length increased with 0.9 cm (IQR 0.0;2.4) per side ( p  < 0.001), muscle thickness decreased with 0.5 cm (IQR − 0.8;− 0.2) (− 25.0%) per side ( p  < 0.001), and muscle mass decreased with 3.9 cm 2 (IQR − 6.4;-1.5) (− 15.8%) per side ( p  < 0.001). Median HU of the psoas muscles (density) increased with 4.8 HU (IQR 0.4;9.7) (10.3%) per side ( p  < 0.001). Effects of BTA were more pronounced in patients with a loss of domain (LoD) ≥ 20%. Conclusions The main effect of BTA injections is elongation and thinning of the LAW muscles, more than a decrease in hernia width. Concomitantly, the abdomen becomes more oval. An increase of psoas muscles density is seen, associated with offloading of the LAW muscles. Patients with large LoD have a proportionally higher effect of BTA.

Background Galactosialidosis (GS) is a rare inherited lysosomal storage disorder (LSD) which is characterized by a defect in the lysosomal glycoprotein catabolism. We report, for the first time, the case of a child affected by GS presenting with recurrent episodes of extensive joint inflammation in both knee joints. The aim of this case-report is to describe the clinical presentation as well as the laboratory, radiologic and microscopic features of this unique presentation of GS. Furthermore, we explore inflammatory mechanisms potentially responsible for the origination of the arthritic joint pathology observed in our patient. Case presentation We describe the rare case of a 12-year-old boy diagnosed with GS (late infantile form) who presented with multiple episodes of inflammatory arthritis involving both knees; no other joints were suspected for joint inflammation. Laboratory results did not indicate an autoimmune disorder. Synovial fluid tested negative for any bacterial infection and ruled out a malignancy and crystal-induced arthritis. Microscopic examination of the synovial tissue revealed numerous foamy macrophages with extensive vacuolization, consistent with the previous diagnosis of GS. Treatment consisted of aspiration of excessive joint fluid and subsequent intra-articular injection of triamcinolonhexacetonide with excellent but transient result. Given the evidence of storage products within macrophages of the inflamed synovial tissue and the absence of other etiological clues, GS itself was considered as the primary cause for the relapsing inflammatory joint pathology. According to the restricted data on articular manifestations in GS, to date, GS cannot be linked directly to joint inflammation. Nevertheless, in several other LSDs, the accumulation of storage material has been associated with numerous osteoimmunological changes that might play a role in the pathophysiology of arthritic processes. Conclusions We hypothesize that the articular build-up of GS storage products triggered systemic as well as local inflammatory processes, resulting in the extensive inflammatory joint pathology as observed in our patient. Future identification of other patients with GS is required to corroborate the existence of an arthritic clinical phenotype of GS and to assess the underlying pathophysiology.

BackgroundEarly detection of microstructural damage to cartilage of patients with juvenile idiopathic arthritis (JIA) might prevent irreversible cartilage damage by timely treatment and follow-up of cartilage microstructure. New, quantitative MRI sequences such as T1ρ are possibly able to detect pre-erosive cartilage damage by quantifying proteoglycan (PG) loss of cartilage[.1–4 ObjectivesTo study feasibility of T1ρ mapping for assessment of articular cartilage integrity in children with JIA and study correlation between T1ρ relaxation time and the Juvenile Arthritis MRI score (JAMRIS)[5 for disease activity.MethodsAfter IRB approval and informed consent, the knee of patients with JIA or suspected JIA was imaged at 3T MRI using T2, pre and post contrast T1 and a sagittal T1ρ sequence with 400 Hz and spin lock time of 5, 10, 20, 40 and 50 ms. A region of interest (ROI) was drawn in articular cartilage of the knee on the T1ρ images, resulting in a mean T1ρ value of articular cartilage per patient. Using regular T2W and T1W pre and post contrast scans, JAMRIS was assigned to discriminate inflamed knees (JAMRIS ≥1) from non-inflamed knees (JAMRIS 0). In SPSS, Mann-Whitney U test and Spearman correlation coefficient were used to compare the mean T1ρ value between patients with and without arthritis on MRI and to correlate T1ρ values with JAMRIS score. ROI drawing was performed twice in 5 subjects. Intraclass correlation coefficient (ICC) was used to study intra-reader reliability.ResultsOf all 13 patients (median age 13.7 years), 7 patients had inflammation in the knee. No cartilage lesions were observed on standard MRI sequences. Acquisition of the T1ρ was successful and without artefacts in 100% of the children. Patients with inflammation in the knee showed a significantly longer T1ρ value than patients without inflammation in the knee: 36.3 ms (IQR 29.0–40.4) versus 27.7 ms (IQR 25.8–30.2), p=0.02. Correlation between T1ρ value and JAMRIS score was 0.76 (p=0.003). Repeatability of ROI drawing was characterised by ICCs>0.99, p<0.05.ConclusionsThis pilot study indicates that, even in the absence of cartilage erosions, significant differences are seen in cartilage integrity with higher T1ρ relaxation times in patients with knee arthritis. This might indicate loss of glycosaminoglycan content in active synovitis of the knee. Thus, our pilot data suggest that T1ρ could serve as an imaging biomarker for cartilage integrity, ultimately aiming to prevent irreversible cartilage damage and long-term disability in this young JIA population.References[1] Tsushima, et al. Rheumatol Int2012;32:2867–2875.[2] Takayama, et al. Eur J Radiol2013;82:e192–198.[3] Duvvuri, et al. Osteoarthritis Cartilage2002;10:838–844.[4] Regatte, et al. Acad Radiol2002;9:1388–1394.[5] Hemke, et al. Eur Radiol2013;23:1075–1083.Disclosure of InterestNone declared

BackgroundJuvenile idiopathic arthritis (JIA) is characterised by chronic inflammation of the joints which can lead to structural bone damage.ObjectivesThe objective of this study was to evaluate the response of new onset JIA patients to an early targeted treatment by conventional radiography.MethodsJIA patients participating in the BeSt for Kids study (NTR 1574) were eligible in case of wrist involvement at inclusion and if conventional radiographs were available at baseline or within 6 months before or after study inclusion. Follow-up radiographs of hands and wrists after 12–36 months were available for comparison. Radiographic bone damage as reflected by carpal length was assessed using the Poznanski score1, providing ‘Z’ as indication of the deviation from a healthy population as measured by radiometacarpal length relative to the second metacarpal length (RM/M2). BoneXpert method2 was used to automatically determine bone age and bone mineral density (BMD) of the wrist.Abstract AB1126 – Table 1Baseline Z-score (95% CI)Compared to healthy populationFollow-up Z-score (95% CI)Compared to healthy populationChange in Z-score Poznanski score0.047 (-0.32 to 0.41)p=0.7950.055 (-0.28 to 0.39)p=0.744p=0.937BMD−0.71 (-1.12 to −0.30)p=0.001−0.44 (-0.75 to −0.12)p=0.008p=0.032Bone age−0.08 (-0.44 to 0.28)p=0.651−0.25 (-0.59 to 0.09)p=0.574p=0.092ResultsForty JIA (27 female) patients were evaluated for Poznanski score and BMD (mean age 7.2±3.4 years), 26 patients (15 female) were evaluated for bone age (mean age 9.3±2.2 years). Assessed by the mean Z-score of RM/M2, we did not detect wrist damage at baseline nor at follow-up. Assessed by the mean Z-score of the bone age, we did not detect deviating bone age at baseline nor at follow-up. At baseline BMD was significantly diminished compared to healthy controls (Z-score −0.71, 95% CI=−1.12 to −0.30). BMD at follow-up improved significantly (Z-score −0.44, 95% CI=−0.75 to −0.12, p=0.032). Results are summarised in table 1.ConclusionsIn this cohort of JIA patients treated early and targeted at inactive disease, we have detected no radiographic wrist damage at baseline or follow-up as detected by Poznanski score. BMD was significantly diminished at baseline but improved significantly after follow-up.References[1] Poznanski AK, Hernandez RJ, Guire KE, Bereza UL, Garn SM. Carpal length in children – a useful measurement in the diagnosis of rheumatoid arthritis and some congenital malformation syndromes. Radiology1978;129:661–8.[2] Anink J, Nusman CM, van Suijlekom-Smit LW, van Rijn RR, Maas M, van Rossum MA. Automated determination of bone age and bone mineral density in patients with juvenile idiopathic arthritis: a feasibility study. Arthritis Res Ther 2014;16:424.Disclosure of InterestNone declared

BackgroundDelayed and/or inappropriate treatment of juvenile idiopathic arthritis (JIA) may lead to permanent loss of joint functionality.1 Contrast-enhanced MRI is increasingly being accepted as a sensitive tool for detecting JIA disease activity in an early stage.2 ObjectivesThe aim of this study was to assess the validity of two reliable contrast-enhanced MRI scores for the assessment of synovitis and tenosynovitis in the wrist of clinically active JIA patients by a comparison with children unaffected by clinical arthritis.MethodsAn axial T1-weighted MRI sequence with contrast-enhancement and fat-saturation was performed on the wrist of 25 children who had no signs of joint inflammation at clinical examination and who were already subjected to contrast-enhanced MR enterography. Wrist MRI scans of 25 clinically active JIA patients were matched based on time-interval between contrast injection and start of the MRI sequence. After being blinded for clinical status, two radiologists scored synovitis and tenosynovitis in consensus. Synovitis was scored at 5 locations by degrees of synovial enhancement (0–2 scale) and synovial inflammation (0–3 scale). Tenosynovitis was scored at the extensor tendons (compartments II, IV and VI) and flexor tendons by degree of inflammation based on a 0–3 scale.3, 4 ResultsChildren unaffected by clinical arthritis had significantly lower total synovial enhancement (median=1 vs 4, p<0.001) and total synovial inflammation (median=1 vs 4, p<0.001) scores compared to clinically active JIA patients (graph). No significant difference in total tenosynovitis score was found between both groups (median=0 vs 0, p=0.220). Fifteen out of 25 (60%) clinically active JIA patients were given a total tenosynovitis score of 0.ConclusionsThe contrast-enhanced MRI scores for the assessment of synovial enhancement and synovial inflammation in the wrist of clinically active JIA patients appear valid. Due to a low incidence of wrist tenosynovitis in this cohort, the validity of the tenosynovitis score could not be assessed. These findings further establish contrast-enhanced MRI as a diagnostic tool with synovitis as the primary target of disease in the wrist of JIA patients.References[1] Hoeksma AF, et al. High prevalence of hand- and wrist-related symptoms, impairments, activity limitations and participation restrictions in children with juvenile idiopathic arthritis. J Rehabil Med2014.[2] Colebatch-Bourn AN, et al. EULAR-PReS points to consider for the use of imaging in the diagnosis and management of juvenile idiopathic arthritis in clinical practice. Ann Rheum Dis2015.[3] Damasio MB, et al. MRI of the wrist in juvenile idiopathic arthritis: proposal of a paediatric synovitis score by a consensus of an international working group. Results of a multicentre reliability study. Pediatr Radiol2012.[4] Lambot K, et al. MRI assessment of tenosynovitis in children with juvenile idiopathic arthritis: inter- and intra-observer variability. Pediatr Radiol2013.Disclosure of InterestNone declared

BackgroundTiming of acquisition of post-contrast MR images for the assessment of the synovial membrane is important: delayed acquisition can result in contrast washout into synovial fluid.1 Several authors demonstrated the importance of this timing using qualitative data (membrane appearance on MRI).1–4 Nonetheless, there is no international consensus on timing of post-contrast images in arthritis, leading to acquisitions at 1 to 10 min after contrast injection.1 5 This could result in incorrect measurement of synovial thickness and thus imprecise assessment of disease activity and over or under treatment of patients.ObjectivesTo quantitatively measure and compare thickness of the synovial membrane on early and late post-contrast knee MRI in patients with juvenile idiopathic arthritis (JIA).MethodsProspectively collected dynamic contrast-enhanced T1 MRIs of children with JIA were used to measure synovial thickness at time point 1 (TP1), 1 min and TP2, 5 min after contrast administration. Written and verbal informed consent for participation in our IRB-approved study was obtained. Two experienced readers, who were blinded for the time point, independently measured synovial thickness on a predefined, marked location in the patellofemoral compartment on randomised images. The Wilcoxon test was used to compare the mean synovial thickness measurements from TP1 and TP2. Moreover, we studied the number of patients judged to have active synovial inflammation (synovium >2 mm) on both time points.ResultsMeasured synovial thickness in 53 patients with JIA (median age 13.5 years, 58.6% female) increased with prolonged time-after-contrast (TP1 1.4 mm and TP2 1.5 mm, p<0.001). Moreover, we found a 25% relative increase of patients with active synovial inflammation (synovial membrane >2 mm) when comparing the measurements at TP2 versus TP1.ConclusionsOur study is the first to add quantitative data to the literature showing that synovial thickness as measured on post-contrast MRI increases with a prolonged interval between contrast administration and acquisition of the post-contrast images. Our data, together with previous studies1 3 4 indicate that it is questionable whether one can reliably measure synovial thickness without standardisation of the interval between contrast administration and acquisition of post-contrast sequences. This could not only influence clinical interpretation and quantitative scoring in JIA, but possibly also impacts other rheumatologic diseases in which synovial thickness is quantified in scoring systems, such as rheumatoid arthritis and osteoarthritis.6 7 References[1] Østergaard, et al. ARD2001;60:1050–1054.[2] Kursunoglu, et al. Radiology1990;176(3):831–835.[3] Yamato, et al. J CAT1993;17(5):781–785.[4] Rieter, et al. Pediatr Radiol2016;46(11):1562–1567.[5] Johnson, et al. Clin Radiol2002;57(6):466–471.[6] Østergaard, et al. J Rheumatol2003;30:1385–1386.1.[7] Guermazi, et al. Ann Rheum Dis2011;70(5):805–811.Disclosure of InterestNone declared