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The second part of the Guidelines and Recommendations for Musculoskeletal Ultrasound (MSUS), produced under the auspices of EFSUMB, following the same methodology as for Part 1, provides information and recommendations on the use of this imaging modality for joint pathology, pediatric applications, and musculoskeletal ultrasound-guided procedures. Clinical application, practical points, limitations, and artifacts are described and discussed for every joint or procedure. The document is intended to guide clinical users in their daily practice., info:eu-repo/semantics/published

CD40, BLK and BANK1 genes involved in the development and signaling of B-cells are identified as susceptibility loci for numerous inflammatory diseases. Accordingly, we assessed the potential influence of CD40, BLK and BANK1 on the pathogenesis of immunoglobulin-A vasculitis (IgAV), predominantly a B-lymphocyte inflammatory condition. Three genetic variants within CD40 (rs1883832, rs1535045, rs4813003) and BLK (rs2254546, rs2736340, rs2618476) as well as two BANK1 polymorphisms (rs10516487, rs3733197), previously associated with inflammatory diseases, were genotyped in 382 Caucasian patients with IgAV and 955 sex- and ethnically matched healthy controls. No statistically significant differences were observed in the genotype and allele frequencies of CD40, BLK and BANK1 when IgAV patients and healthy controls were compared. Similar results were found when CD40, BLK and BANK1 genotypes or alleles frequencies were compared between patients with IgAV stratified according to the age at disease onset or to the presence/absence of gastrointestinal or renal manifestations. Moreover, no CD40, BLK and BANK1 haplotype differences were disclosed between patients with IgAV and healthy controls and between patients with IgAV stratified according to the clinical characteristics mentioned above. Our findings indicate that CD40, BLK and BANK1 do not contribute to the genetic background of IgAV., European Commission, Instituto de Salud Carlos III" (ISCIII, Health Ministry, Spain) PI18/00042 PI21/00042 Instituto de Salud Carlos III, European Social Fund (ESF) CM20/00006, RICORS Program from ISCIII, European Commission RD21/0002/0025, RETICS Program (ISCIII) RD16/0012/0009, RETICS Program (European Regional Development Fund (ERDF) RD16/0012/0009, European Union FEDER fund RD16/0012/0014 PI17/00409, Research Executive Agency (REA) of the European Union 734899, Miguel Servet type II program fellowship from the ISCIII, ESF ("Investing in your future") CPII21/00004

The first part of the guidelines and recommendations for musculoskeletal ultrasound, produced under the auspices of the European Federation of Societies for Ultrasound in Medicine and Biology (EFSUMB), provides information about the use of musculoskeletal ultrasound for assessing extraarticular structures (muscles, tendons, entheses, ligaments, bones, bursae, fasciae, nerves, skin, subcutaneous tissues, and nails) and their pathologies. Clinical applications, practical points, limitations, and artifacts are described and discussed for every structure. After an extensive literature review, the recommendations have been developed according to the Oxford Centre for Evidence-based Medicine and GRADE criteria and the consensus level was established through a Delphi process. The document is intended to guide clinical users in their daily practice.Der erste Teil der Leitlinien und Empfehlungen für den muskuloskelettalen Ultraschall, die unter der Schirmherrschaft der European Federation of Societies for Ultrasound in Medicine and Biology (EFSUMB) erstellt wurden, enthält Informationen über den Einsatz des muskuloskelettalen Ultraschalls zur Beurteilung von extraartikulären Strukturen (Muskeln, Sehnen, Gelenke, Bänder, Knochen, Schleimbeutel, Faszien, Nerven, Haut, subkutanes Gewebe und Nägel) und deren Pathologien. Für jede Struktur werden die klinische Anwendung, praktische Punkte, Einschränkungen und Artefakte beschrieben und diskutiert. Nach einer ausführlichen Literaturrecherche wurden die Empfehlungen gemäß den Kriterien des Oxford Centre for Evidence-based Medicine und den GRADE-Kriterien entwickelt, und der Konsensgrad wurde durch die Delphi-Methode ermittelt. Das Dokument ist als Leitfaden für klinische Anwender in der täglichen Praxis gedacht.

Objectives: To evaluate electrocardiogram markers to predict coronary involvement in patients with Kawasaki disease by assessing measures of ventricular repolarization parameters on the 12-lead electrocardiogram. Study design: This cross-sectional study included 180 Spanish and Japanese patients ≤14 years of age with Kawasaki disease, with or without coronary involvement, from 2011 to 2016. We manually measured the Tp-Te/QT ratio and QTc interval (with Bazett's formula) in 12-lead electrocardiogram in the acute and recovery period and explored their potential association with coronary involvement. Results: No association was found between Tp-Te/QT ratio obtained manually in V5 and V6 leads and coronary involvement in the acute (V5:0.25 [IQR, 0.21-0.27] vs 0.25 [IQR, 0.20-0.27], P = .80; V6:0.24 [IQR, 0.21-0.27] vs 0.25 [IQR, 0.20-0.27], P = .86) or the recovery (V5: 0.23 [IQR, 0.20-0.25] vs 0.23 [IQR, 0.19-0.25], P = .68; V6: 0.23 [IQR, 0.20-0.25] vs 0.23 [IQR, 0.17-0.25], P = .50) period. By contrast, QTc in V5 and V6 was significantly lower in patients with Kawasaki disease and coronary involvement in the acute period (V5: 378 ms [IQR, 364-395 ms] vs 390 ms [IQR, 371-411 ms], P = .04; V6: 377 ms [IQR, 364-392 ms] vs 390 ms [IQR, 371-410 ms], P = .01). A QTc interval of

BACKGROUND Interest in ultrasound (US) as a diagnostic tool in spondyloarthritis is growing. Although the literature has provided detailed description of normal lower-limb entheses for adults and children, validated normal US findings of the pediatric upper-limb (UL) entheses are lacking. OBJECTIVES The aim of this study was to describe the sonographic characteristics of the UL entheses in healthy children to provide a basis for assessing pathologic findings in children with rheumatic diseases. METHODS This is an observational, cross-sectional study. The recruited healthy children were grouped according to age: group 1, 3-9 years (n = 22); group 2, 10 to 13 years (n = 12); and group 3, 14 to 18 years (n = 9). The following UL entheses were examined: (1) supraspinatus, (2) common flexor, (3) common extensor, and (4) triceps. Ultrasound examination was performed in B mode for morphology and structural abnormalities, and power Doppler was applied to detect vascularization within the enthesis and cartilage sites. RESULTS Forty-three children with a median age of 9 years (6-13 years) were included; 55% were boys. A total of 344 entheses were evaluated; all of them presented a homogeneous hyperechoic fibrillar pattern. Tendon insertion thickness at the bone attachment site increased with age and was greater in males. No signs of chronic enthesopathy were detected. Doppler signal was observed in children from groups 1 and 2, but not in any of the patients in group 3. In group 1, intraentheses signal was detected in the common extensor tendon insertion as an isolated signal (n = 2 patients), whereas intracartilage signal was detected in the supraspinatus (n = 1 patient) and the triceps (n = 1 patient). In group 2, intracartilage signal was detected in the common extensor (n = 2 patients) and the common flexor (n = 2 patients). Interobserver agreement for the entheses' thickness was as follows: intracorrelation coefficient class (ICC) of 0.86 (95% confidence interval [CI], 0.84-0.92) for the supraspinatus enthesis, ICC of 0.83 (95% CI, 0.83-0.90) for the common extensor enthesis, ICC of 0.96 (95% CI, 0.91-0.97) for the common flexor enthesis, and ICC of 0.94 (95% CI, 0.89-0.95) for the triceps enthesis. The κ index was 0.93 for the detection of the power Doppler signal. Intraobserver agreement was good for all evaluated entheses: ICC of 0.85 (95% CI, 0.83-0.91) for supraspinatus, ICC of 0.86 (95% CI, 0.84-0.91) for common extensor entheses, ICC of 0.89 (95% CI, 0.86-0.93) for common flexor entheses, and ICC of 0.96 (95% CI, 0.90-0.97) for evaluation of triceps entheses. The κ index was 0.91 for the detection of Doppler signal. CONCLUSIONS The current study provides a comprehensive description of the structural appearance and vascularization of UL entheses on healthy children. This information can be useful as reference for interpretation of pathological findings in children with musculoskeletal diseases in clinical practice.

Tras el análisis de la importancia de la educación superior universitaria, su impacto en el desarrollo de los países y los conflictos que generan el balance entre una autorregulación, respaldada en la autonomía universitaria, y la regulación externa, se evidencia que, la falta de calidad en los procesos formativos y su coherencia con el mercado laboral, termina siendo la opción en como los gobiernos incrementan la regulación externa. Esto se ve reflejado de una manera u otra, en el sistema educativo universitario peruano y en sus procesos de acreditación. En este artículo, se pretende realizar un análisis comparativo con la experiencia de otros países con modelos de acreditación más eficaces, para ello se identifica: (i) la evaluación de la investigación; (ii) la implementación de sistemas de gestión que aseguren la calidad de la institución educativa y; (iii) la formación de evaluadores externos como los elementos diferenciadores, sobre los cuales se establecen alternativas de trabajo en los estándares de acreditación y en los programas de capacitación de pares evaluadores. Además, se enmarca la problemática en la época de la pandemia ocasionada por la Covid-19., After analyzing the importance of university higher education, its impact on the development of countries and the conflicts that generate the balance between self-regulation, supported by university autonomy and external regulation, it is evident that, by a reality or an excuse, the lack of quality of the training processes, and the lack of coherence with the labor market, ends up being the way that governments have the option of increasing external regulation. This is evident in the reality of Peru’s university education system and in its accreditation processes. Through a comparative analysis with the experience of other countries with more mature accreditation models, it can be identified three differentiating elements: (i) the evaluation of the research, (ii) the implementation of management systems that ensure the quality of the educational institution, and (iii) the training of external evaluators, on which work strategies are established in the accreditation standards and in the peer evaluators training programs. At the end, the problem is framed at the time of the Covid-19 pandemic.

Objective To determine the intra- and inter-observer reliability of ultrasound (US)-detected age-related joint vascularization and ossification grading in healthy children. Methods Following standardized image acquisition and machine setting protocols, 10 international US experts examined four joints (wrist, second metacarpophalangeal joint, knee, and ankle) in 12 healthy children (divided into four age groups: 2–4, 5–8, 9–12, and 13–16 years). Grey-scale was used to detect the ossification grade, and power Doppler (PD) US was used to detect physiological vascularization. Ossification was graded from grade 0 (no ossification) to grade 3 (complete ossification). A positive PD signal was defined as any PD signal inside the joint. Kappa statistics were applied for intra- and inter-observer reliability. Results According to the specific joint and age, up to four solitary PD signals (mean, 1.5) were detected within each joint area with predominant localization of the physiological vascularization in specific anatomic positions: fat pad, epiphysis, physis, and short bone cartilage. The kappa values for ossification grading were 0.87 (range, 0.85–0.91) and 0.58 for intra- and inter-observer reliability, respectively. The bias-adjusted kappa values for intra- and inter-observer reliability were 0.71 (range, 0.44–1.00) and 0.69, respectively. Conclusion Detection of normal findings (i.e., grading of physiological ossification during skeletal maturation and identification of physiological vessels) can be highly reliable by using clear definitions and a standardized acquisition protocol. These data will permit development of a reliable and standardized US approach for evaluating paediatric joint pathologies. This article is protected by copyright. All rights reserved.

Objective To develop evidence-based recommendations on the use of ultrasound (US) and magnetic resonance imaging in patients with spondyloarthritis, including psoriatic arthritis, and juvenile idiopathic arthritis. Methods Recommendations were generated following a nominal group technique. A panel of experts (15 rheumatologists and 3 radiologists) was established in the first panel meeting to define the scope and purpose of the consensus document, as well as chapters, potential recommendations and systematic literature reviews (we used and updated those from previous EULAR documents). A first draft of recommendations and text was generated. Then, an electronic Delphi process (2 rounds) was carried out. Recommendations were voted from 1 (total disagreement) to 10 (total agreement). We defined agreement if at least 70% of participants voted ≥7. The level of evidence and grade or recommendation was assessed using the Oxford Centre for Evidence Based Medicine levels of evidence. The full text was circulated and reviewed by the panel. The consensus was coordinated by an expert methodologist. Results A total of 12 recommendations were proposed for each disease. They include, along with explanations of the validity of US and magnetic resonance imaging regarding inflammation and damage detection, diagnosis, prediction (structural damage progression, flare, treatment response, etc.), monitoring and the use of US guided injections/biopsies. Conclusions These recommendations will help clinicians use US and magnetic resonance imaging in patients with spondyloarthritis and juvenile idiopathic arthritis.

Resumen Objetivo Establecer recomendaciones, basadas en la evidencia, sobre el uso de la ecografia (US) y la resonancia magnetica en pacientes con espondiloartritis, incluyendo la artritis psoriasica, y en la artritis idiopatica juvenil. Metodos Las recomendaciones se consensuaron mediante metodologia basada en grupos nominales. Un grupo de expertos (15 reumatologos y 3 radiologos) definio el alcance, los usuarios, los apartados, las posibles recomendaciones y las revisiones sistematicas a realizar (se utilizaron y actualizaron las revisiones de documentos de consenso de EULAR), y se asignaron tareas. Los expertos delimitaron los apartados y redactaron las recomendaciones. El nivel de evidencia y el grado de recomendacion se establecieron utilizando el sistema del Centre for Evidence Based Medicine de Oxford, y el grado de acuerdo mediante Delphi a 2 rondas. Las recomendaciones se votaron segun una escala de 1 (total desacuerdo) a 10 (total acuerdo), definiendose el acuerdo como una puntuacion ≥ 7 por al menos el 70% de los participantes. El documento fue revisado por los expertos y el proyecto estuvo coordinado por un metodologo experto. Resultados Se emitieron 12 recomendaciones sobre la validez de la US y la resonancia magnetica para la deteccion de actividad y dano estructural, capacidad diagnostica, predictora (de progresion de dano estructural, brote de la enfermedad, respuesta al tratamiento, etc.), utilidad en la evaluacion y monitorizacion del tratamiento, y uso de la US como guia (para infiltraciones, biopsias, etc.) en pacientes con espondiloartritis y artritis idiopatica juvenil. Conclusiones Se presentan unas recomendaciones utiles para el manejo de la US y la resonancia magnetica por los clinicos en pacientes con espondiloartritis y artritis idiopatica juvenil.

Resumen Objetivo La enfermedad de Kawasaki (EK) es una vasculitis sistemica aguda de arterias de tamano medio, especialmente las coronarias. Su diagnostico clasico se basa en la presencia de fiebre prolongada y diversas manifestaciones clinicas. En la era preinmunoglobulinas, la artritis aguda constituia un hallazgo comun. Nuestro objetivo es definir la prevalencia de la artritis al diagnostico, su respuesta a inmunoglobulinas intravenosas y su relacion con eventos cardiacos. Material y metodos Estudio retrospectivo de 42 pacientes con EK desde enero de 1988 a noviembre de 2013. Las variables demograficas, clinicas, laboratorio y tratamiento fueron revisadas. Resultados Se encontro predominio de varones (57,1%), fiebre (100%), exantema (92,9%), conjuntivitis (78,6%), afectacion oral (76,2%), adenopatia cervical (71,4%), edema (52,4%) y descamacion (46,3%). Ocho pacientes presentaron clinica cardiologica y ecocardiograma con alteraciones coronarias. Siete pacientes (16%) presentaron afectacion articular aguda. El 57% fue oligoarticular con predominio de tobillos, 29% monoarticular de cadera y 14% poliarticular. El 100% aumento los reactantes de fase aguda, con neutrofilia (57%) e hipoalbuminemia (71,5%). Todos respondieron a inmunoglobulinas intravenosas, sin afectacion cardiaca ni secuelas posteriores. Hubo dieciseis EK incompletos: nueve varones, con fiebre (100%), exantema (75%), conjuntivitis (56%), adenopatia cervical (50%), alteracion de mucosa oral (44%) y edemas (25%). Cuatro tuvieron afectacion cardiaca (un aneurisma; tres ectasias). Conclusiones La artritis aguda fue poco frecuente (16%) y sin secuelas posteriores. El tratamiento con inmunoglobulinas intravenosas y acido acetilsalicilico posiblemente previene su desarrollo, disminuyendo el seguimiento por Reumatologia. La afectacion cardiaca, principalmente en EK incompleta, sigue marcando su pronostico, sin hallar empeoramiento cardiaco en pacientes con artritis.

The aim of this study was to explore the usefulness of the determination of free light chains (FLC) in serum as a biomarker of flare in patients with systemic lupus erythematosus (SLE) and to analyze the differences in their discriminatory capacity with complement C3 and C4.This was a prospective cohort study. The definition of flare was based on the Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) Flare Index. The discriminatory capacity of FLC and C3 and C4 levels was compared using receiver operating characteristic (ROC) curves and the area under the curve (AUC).Forty-six patients were enrolled. Patients with SLE flare showed significantly lower C3 (p = 0.025) and C4 levels (p = 0.028), as well as a higher concentration of lambda light chains (λ-LC) (p = 0.028) compared with the non-flare group. λ-LC, as opposed to kappa light chains and total light chains, demonstrated a discriminatory capacity for detecting the presence of SLE flare (AUC 0.781), with 100% sensitivity, 65% specificity, and 69.6% of patients correctly classified for a cutoff point of ≥ 19.5 mg/L. Complement C3 and C4 also showed a high discriminatory capacity for SLE flare (AUC 0.804 and 0.837, respectively). Comparing λ-LC, C3, and C4, the last one demonstrates better discriminatory capacity for SLE flare with the highest AUC (0.837; 95% CI 0.663-1.000).λ-LC have good discriminatory capacity for SLE flare and could be useful as a biomarker of SLE exacerbation.Key Points• The usefulness of free light chains as a biomarker could be compared with complement.• Lambda free light chains have good discriminatory capacity for SLE flare.• Free light chains are a promising marker of SLE activity.

ObjectiveTo develop ultrasound (US) definitions and a US novel scoring system for major salivary gland (SG) lesions in patients with primary Sjögren’s syndrome (pSS) and to test their intrareader and inter-reader reliability using US video clips.MethodsTwenty-five rheumatologists were subjected to a three-round, web-based Delphi process in order to agree on (1) definitions and scanning procedure of salivary gland ultrasonography (SGUS): parotid, submandibular and sublingual glands (PG, SMG and SLG); (2) definitions for the elementary SGUS lesions in patients with Sjögren’s syndrome; (3) scoring system for grading changes. The experts rated the statements on a 1–5 Likert scale. In the second step, SGUS video clips of patients with pSS and non-pSS sicca cases were collected containing various spectrums of disease severity followed by an intrareader and inter-reader reliability exercise. Each video clip was evaluated according to the agreed definitions.ResultsConsensual definitions were developed after three Delphi rounds. Among the three selected SGs, US assessment of PGs and SMGs was agreed on. Agreement was reached to score only greyscale lesions and to focus on anechoic/hypoechoic foci in a semiquantitative matter or, if not possible on a qualitatively (present/absent) evaluation of fatty or fibrous lesions. Intrareader reliability for detecting and scoring these lesions was excellent (Cohen’s kappa 0.81) and inter-reader reliability was good (Light’s kappa 0.66).ConclusionNew definitions for developing a novel semiquantitative US score in patients with pSS were developed and tested on video clips. Inter-reader and intrareader reliabilities were good and excellent, respectively.

Background Tendon involvement is common in patients with juvenile idiopathic arthritis (JIA). Differentiating tenosynovitis and underlying arthritis may be challenging on the basis of clinical examination (CE) alone. In daily clinical practice, ultrasound (US) imaging with Doppler information can be more versatile as it allows a dynamic assessment of joint inflammation and can be done quickly at the bedside. US can reliably evaluate the location of inflammation and provide direct visualization when local steroid injection is considered as treatment for children with JIA. Objectives To present the published data concerning the US assessment of tenosynovitis and the US metric properties investigated in JIA. Methods A systematic literature review (SLR) was conducted comprising PubMed, Embase and the Cochrane Library (before June 2018). Search terms: “arthritis, juvenile” AND, “tenosynovitis OR tendinopathy” AND “ultrasonography” OR “Doppler” AND “diagnosis OR therapy management”. Selection criteria: Original articles published in English reporting US, tenosynovitis and tendinopathy in the paediatric population (up to18 years old) with JIA. Data were extracted from manuscripts meeting the selection criteria, with particular focus on the definition used, scoring system applied and the US metric properties investigated. The quality of the studies was assessed using the same methodology developed for the SLR on paediatric synovitis [1]. Results After selection, 11 articles were analysed. The tibialis posterior tendon group and peroneal tendons were the most commonly evaluated. Most authors applied the OMERACT definition proposed to rheumatoid arthritis (RA) [2] for evaluating paediatric tenosynovitis. Binary scoring system on both grey scale and Doppler was applied in most of studies. Doppler function was commonly included, with only five specifying the Doppler parameters that were used. Construct validity was reported in roughly 50% of articles, including the CE as the main comparator. However, some of those articles have shown that clinical evaluation underestimates tendon involvement. US reliability and responsiveness for tenosynovitis in JIA was rarely included. Conclusion Although US seems a promising image modality to assess tenosynovitis in JIA, this SLR emphasizes the definitions of US elementary lesions of RA are frequently used in children. Moreover, there is insufficient evidence of studies exploring the construct validity of the US-determined tenosynovitis in JIA. Further validation studies are needed for implementation in clinical practice and trials. References [1] Collado P. Arthritis Care Res2012; 64:1011 [2] Wakefield R. J Rheumatol. 2005;32:2485 Disclosure of Interests PAZ COLLADO: None declared, Silvia Magni-Manzoni Consultant for: Abbvie, Speakers bureau: Abbvie, MARTINA STEINER: None declared, Tracy Ting : None declared, Patricia Vega Fernandez: None declared, Clara Malattia: None declared, Ana Rodriguez: None declared, George Bruyn : None declared, Helen Keen: None declared, Lene Terslev Speakers bureau: Speakers fee from : Roche, Novartis, Pfizer, MSD, BMS, Celgene

Objectives To develop and test the reliability of a new semiquantitative scoring system for the assessment of cartilage changes by ultrasound in a web-based exercise as well as a patient exercise of patients with RA. Methods A taskforce of the Outcome Measures in Rheumatology Ultrasound Working Group performed a systematic literature review on the US assessment of cartilage in RA, followed by a Delphi survey on cartilage changes and a new semiquantitative US scoring system, and finally a web-based exercise as well as a patient exercise. For the web-based exercise, taskforce members scored a dataset of anonymized static images of MCP joints in RA patients and healthy controls, which also contained duplicate images. Subsequently, 12 taskforce members used the same US to score cartilage in MCP and proximal interphalangeal joints of six patients with RA in in a patient reliability exercise. Percentage agreement and prevalence of lesions were calculated, as intrareader reliability was assessed by weighted kappa and interreader reliability by Light’s kappa. Results The three-grade semiquantitative scoring system demonstrated excellent intrareader reliability (kappa: 0.87 and 0.83) in the web-based exercise and the patient exercise, respectively. Interreader reliability was good in the web-based exercise (kappa: 0.64) and moderate (kappa: 0.48) in the patient exercise. Conclusion Our study demonstrates that ultrasound is a reliable tool for evaluating cartilage changes in the MCP joints of patients with RA and supports further development of a new reliable semiquantitative ultrasound scoring system for evaluating cartilage involvement in RA.

Objective. Musculoskeletal (MS) ultrasound (US) is increasingly being used as an extension of paediatric clinical practice, particularly in the clinical management of children with juvenile idiopathic arthritis (JIA). MSUS is most commonly used in the assessment of effusion and synovial hypertrophy and detection of soft tissue disorders. It can also be used to visualise other structures, such as hyaline cartilage and bone surfaces. Given the unique anatomy of the growing skeleton, many of the difficulties encountered in its use result from misinterpretation of MSUS images. Thus, a detailed knowledge of anatomy in healthy children, both in B-mode and Doppler mode, is mandatory. Additionally, as US is the most operator-dependent imaging modality, the experience and expertise of the examiner will determine the value of the diagnostic information obtained from this technique. Incorrect acquisition and interpretation of images may result in inappropriate disease management. Thus, a systematic scanning method and a standardised evaluation procedure are essential in children.

A maturity model is a widely used tool in software engineering and has mostly been extended to domains such as education, health, energy, finance, government, and general use. It is valuable for evaluations and continuous improvement of business processes or certain aspects of organizations, as it represents a more organized and systematic way of doing business. In this paper, we only focus on college higher education. For this reason, we present a novel approach that allows detecting some gaps in the existing maturity models for universities, as they are not models that address the dimensions in their entirety. To identify these models and their validities, as well as a classification of models that were identified in universities, we carried out a systematic literature review on 27,289 articles retrieved with respect to maturity models and published in peer-reviewed journals between 2007 and 2020. We found 23 articles that find maturity models applied in universities, through exclusion and inclusion criteria. We then grouped these items into nine categories with specific purposes. We concluded that maturity models used in Universities move towards agility, which is supported by the semantic web.

Subclinical synovitis is often detected by musculoskeletal ultrasound (MSUS) in juvenile idiopathic arthritis (JIA) patients in clinical remission. The main objective of this prospective, observational, longitudinal, multicentre study was to evaluate the predictive value of MSUS-detected subclinical synovitis in relation to flares at 12 months following TNFi tapering in a JIA population in stable clinical remission.We included 56 JIA patients in stable remission undergoing TNFi therapy tapered at baseline and in some cases at 6 months. We performed baseline and 6-month MSUS assessment on B-mode (BM) and power Doppler (PD) mode of 22 joints and 8 tendons.Eighteen patients (32.1%) experienced a flare during the 12-month study period. BM synovitis was frequent (83.9%) but PD synovitis was scarcely found (8.9%). There were no significant differences in MSUS findings between patients who experienced a flare and those who remained in remission. Only 5 patients had positive for PD synovitis, in joints with BM synovitis grades 2 or 3, and none experienced a flare. Concomitant methotrexate (MTX) was more frequent in patients who were successfully tapered (71.1% vs. 27.8%; p=0.002) and patients older than 12 experienced a greater number of flares and earlier onset.Subclinical synovitis, as detected by MSUS, proved not to be a predictor of flares. Those patients on a TNFi-tapered concomitant methotrexate regimen experienced the fewest flares although flare risk increased with age.

Objective Musculoskeletal ultrasonography (US) has potential in the assessment of disease activity and structural damage in childhood arthritides. In order to assess pathology, the US characteristics of joints in healthy children need to be defined first. The aim of this study was to develop definitions for the various components of the normal pediatric joint. Methods The definitions were developed by an expert group and applicability was assessed on a collection of standardized scans of the knee and ankle joints by scoring the scans on a Likert scale. The definitions were then modified and applicability was reassessed before sending the definitions for approval to a larger panel of experts. A final scoring on stored images of all relevant joints at different ages followed. Results Five definitions were developed addressing the articular bone, cartilage, joint capsule, epiphyseal ossification center, and synovial membrane. In total, 224 US images of knees and ankles were acquired, of which 172 were selected for scoring. An agreement of ≥80% was not met for any of the definitions, but after modifications, 81–97% agreement was reached. This version of the definitions was approved by 15 US experts. In the final validation exercise, all definitions reached an agreement of ≥80% for the shoulder, elbow, wrist, metacarpophalangeal hip, knee, ankle and metatarsophalangeal joint. Conclusion US definitions for the normal pediatric joint were successfully developed through a Delphi process and validated in a practical exercise. These results provide the basis to develop definitions for pathology and to support the standardized use of US in pediatric rheumatology.

Objective.To provide an update from the Outcome Measures in Rheumatology (OMERACT) Ultrasound Working Group on the progress for defining ultrasound (US) minimal disease activity threshold at joint level in rheumatoid arthritis (RA) and for standardization of US application in juvenile idiopathic arthritis (JIA).Methods.For minimal disease activity, healthy controls (HC) and patients with early arthritis (EA) who were naive to disease-modifying antirheumatic drugs were recruited from 2 centers. US was performed of the hands and feet, and scored semiquantitatively (0–3) for synovial hypertrophy (SH) and power Doppler (PD). Synovial effusion (SE) was scored a binary variable. For JIA, a Delphi approach and subsequent validation in static images and patient-based exercises were used to developed preliminary definitions for synovitis and a scoring system.Results.For minimal disease activity, 7% HC had at least 1 joint abnormality versus 30% in the EA group. In HC, the findings of SH and PD were predominantly grade 1 whereas all grades were seen in the EA cohort, but SE was rare. In JIA, synovitis can be diagnosed based on B-mode findings alone because of the presence of physiological vascularization. A semiquantitative scoring system (0–3) for synovitis for both B-mode and Doppler were developed in which the cutoff between Doppler grade 2 and grade 3 was 30%.Conclusion.The first step has been taken to define the threshold for minimal disease activity in RA by US and to define and develop a scoring system for synovitis in JIA. Further steps are planned for the continuous validation of US in these areas.

Objectives Musculoskeletal ultrasonography (US) has the potential to be an important tool in the assessment of disease activity in childhood arthritides. To assess pathology, clear definitions for synovitis need to be developed first. The aim of this study was to develop and validate these definitions through an international consensus process. Methods The decision on which US techniques to use, the components to be included in the definitions as well as the final wording were developed by 31 ultrasound experts in a consensus process. A Likert scale of 1-5 with 1 indicating complete disagreement and 5 complete agreement was used. A minimum of 80% of the experts scoring 4 or 5 was required for final approval. The definitions were then validated on 120 standardized US images of the wrist, MCP and tibiotalar joints displaying various degrees of synovitis at various ages. Results B-Mode and Doppler should be used for assessing synovitis in children. A US definition of the various components (i.e. synovial hypertrophy, effusion and Doppler signal within the synovium) was developed. The definition was validated on still images with a median of 89% (range 80-100) of participants scoring it as 4 or 5 on a Likert scale. Conclusions US definitions of synovitis and its elementary components covering the entire pediatric age range were successfully developed through a Delphi process and validated in a web-based still images exercise. These results provide the basis for the standardized US assessment of synovitis in clinical practice and research. This article is protected by copyright. All rights reserved.

Background Transitional care should be a planned movement of adolescents with chronic diseases from child-centred to adult-oriented health care system. Recently a EULAR/PReS taskforce has developed the first international set of recommendations and standards for transitional care 1 . Objectives To describe the results from a specific transitional care programme. Methods The current transitional care programme includes a multidisciplinary PED-REUMA clinic (MPRC) weekly and a non-defined period of rheumatologic follow-ups by the same rheumatologist of MPRC. The transitional care team is composed of two pediatricians, one adult rheumatologist -as transition coordinator-, a clinical nurse specialist and administrative support, as well as a psychologist and physiotherapists. Clinical information and therapies were collected throughout the disease course, and the HEADSS method of psychosocial interviewing has been included recently. Population of the present study included young patients (YP) from that programme who are going to transfer to adult-oriented health care system. Descriptive study of socio-demographics and clinic features was included, as well as patients9 adherence. YP confidence to be transferred and satisfaction with the current transitional process were measured using on a scale of 0 to 10. In patients suffering from juvenile idiopathic arthritis (JIA), clinical status of disease activity and clinical remission and CHAQ were tested before transfer. Results Twenty-seven YP with female predominance (63%) were included. The average age was 21±3 yo at time of a planned transfer and 16±3.4 yo at inclusion to the MPRC. JIA was the commonest condition whereas dermatomyositis was uncommon. Up to 63% patients required some DMARDs during the MPRC follow-up, but only a 37% needs maintained immunosuppressive therapies and three (11%) patients required changing the therapeutic target before transfer. YP adherence to rheumatologist appointments was high. Regarding HEADSS data: most YP were students and living at family home, around 50% gave up sports or other activities due to homework or exams, 29% of YP occasionally drank some alcohol but none used tobacco, and 47% of YP felt sad or down once in a while. Patient9s confidence to be transferred was 7.7±2.1 (mean±SD; min-max: 2–10). YP showed high satisfaction with the current transitional process, 9.7±0.4 (min-max: 9–10). Before transfer, 17 patients with JIA showed a mean±SD value of JADAS10 of 2±5 (min-max, 0–18), clinical remission on/off medication was 23% and 53% respectively. Mostly functional status reported by patient was low, YP-rated CHAQ (mean 0.06; min-max, 0.0–0.75). Conclusions To the best of our knowledge, this is the first study evaluating a Spanish transitional care programme. The study reports a positive impact across adolescence of our transitional care model in a real life situation. Implementation of recommendations depended on the local available resources. References Foster HE. EULAR/PReS standards and recommendations for the transitional care of young people with juvenile-onset rheumatic diseases. Ann Rheum Dis Published Online First: [1, November 2016] doi:10.1136/ annrheumdis-2016–210112. Acknowledgements Beatriz Jimenez. Auxiliary to the clinical nurse specialist and administrative support of Rheumatology Department. Disclosure of Interest None declared

Background Anti-TNF therapy is effective and safe in JIA. Changes in anti-TNF doses are common when remission is achieved 1 . Subclinical synovitis on Doppler mode (PD) detected by ultrasound can predict flares in adult RA, but it is not yet clear in JIA 2 Objectives The aim of this study is to evaluate the predictive value of subclinical synovitis detected by PD-US in relation to flares in patients with JIA on remission under anti-TNF when therapy is tapered. The preliminary results were presented at the EULAR congress 2015 in Rome (FRI0520). Methods Observational, prospective and multicenter study. We included JIA patients on remission at least 6 months with anti-TNF, ETN and ADA, in whom anti-TNF was tapered due to clinical decision. ETN was tapered by increasing the injection 3 days and ADA by increasing a week. Patients were clinically assessed every 3 months and also with PD-US at baseline. Bilateral US assessment included joints and tendons. Adult synovitis definitions and semiquantitative scoring system were used, no synovitis definitions are available for JIA. We collected demographics (date of birth, JIA subcategory, previous and current treatments. Flare was defined as clinical signs and/or symptoms of arthritis that required increase of systemic therapy Results We included 57 patients, with 19 patients (33.33%) having a flare during the 12 months follow-up. 38 patients (66.67%) were receiving ETN and 19 (33.3%) ADA, of which 11 patients (28.95%) had a flare with ETN and 8 patients (42.11%) with ADA. Table 1 shows demographics. Median time to flare was 5.73 months (IR 2–93–8.9). Concomitant methotrexate was lower in patients with flare (26.32% vs 71.05%). In 18 patients (31.58%), a previous tapering was done and median time of remission before being included was 22 months (IR 15.5–28.5). US does not predict flare in our cohort. Global synovitis score at baseline was 4 (IQR 1.3–10.8) and 0 in BM and PD respectively, and tenosynovitis was 0 both BM and PD Conclusions Anti-TNF tapering was safe in our JIA patients in more than half of patients after 1 year follow-up. US did not predict flares in our patients. Concomitant treatment with methotrexate was more frequent in patients who remained on remission References Cai Y. Rheumatol Int.2013. Magni-Manzoni S.Ann Rheum Dis.2013. Disclosure of Interest None declared

Background Ultrasound (US) was found to have face and content validity for detecting synovitis in juvenile idiopathic arthritis (JIA) with higher sensitivity than clinical examination. In order to test validity and improve the applicability of US in JIA, the OMERACT US pediatric subtask force recently published preliminary definitions for the sonographic features of synovitis in children. Objectives Aim of this study was to confirm and improve B-mode and color power/Doppler (PD) US definitions for synovial components and grading in children, by using an image and patient based exercise. Methods The definitions were confirmed and modified in a multi-step process. In the 1st step, definitions were developed in multi-round Delphi web based consensus process were ≥80% of participants would need to reach ≥80% of agreement on a Likert scale from 1–5 (1 strongly disagree, 2 disagree, 3 neutral, 4 agree, 5 strongly agree). In the 2nd step, in a face to face meeting, a subgroup of these experts revised the definitions for final wording and performed intra- and inter-observer reliability exercise study in JIA patients as the final 3rd phase of the process. The definitions were tested in four joints (wrist, 2nd MCP, knee and ankle) of JIA patients divided in four age groups following standardized image acquisition and machine setting protocol. Statistics program R (version 3.3.0) was used for the statistical analyses. For intra-rater agreement Cohen kappa and for inter-rater agreement prevalence and bias adjusted kappa (PABAK) were calculated if needed. Results Reliability exercise included 20 JIA patients (distributed in equal numbers by age groups), 14 observers, 4 joints/observer, 3 observers/joint, 360 intra- and 360 inter-observer tests. A 0–3 semi-quantitative B-mode and color power/Doppler US definitions for synovial components and grading, were agreed (presented in Figure 1). Conclusions The proposed synovitis grading for children showed to be reliable why the next step should be to test sensitivity to change in order to possibly be used as an outcome tool in JIA. Disclosure of Interest None declared

Background Ultrasonography (US) of salivary glands (USSG) may support diagnosis and evaluation of disease activity in primary Sjogren9s syndrome pSS. In order to extent the use of USSG in daily practice, consensus definitions of normal and abnormal USSG findings are needed. Objectives To obtain consensual definitions of normal and abnormal USSG findings using a Delphi process. Methods Twenty-seven experts participated in the Delphi process. A Likert scale from 1–5 was used in which 1 indicated complete disagreement and 5 complete agreement. Three core items were proposed: 1. Consensus of assessment of SG (parotid gland (PG), submandibular glands (SMG), sublingual glands SLG)). 2. Consensus for elementary lesions of PG, SMG, and SLG changes in PSS patients. 3. Consensus on grading of PG, SM, and SLG in pSS. Results The consensus of definitions between experts was reached after three rounds.The results (table 1) showed agreement with more than 75% of agreement in normal and abnormal definitions in SG and concluded to not evaluate SLG in pSS patients as well as to score pSS SG patients with at least two abnormal glands out of 4 (2 PG and 2 SMG) with a scoring system >2 for each abnormal gland. Conclusions We developed a consensual US definition of normal and abnormal USGS findings through a Delphi exercise process. This finding will be used as a basis to further proceed with the validation of USGS for clinical application. Disclosure of Interest None declared

Objective. The aim of this study was to investigate the current use of musculoskeletal US (MSUS) and the most relevant areas of interest for this imaging modality in paediatric rheumatology. Methods. A questionnaire was developed by the paediatric subgroup of the OMERACT US task force and e-mailed to the members of the main international paediatric rheumatology networks and societies. Responses were entered in an electronic database. Results were analysed quantitatively or summarized qualitatively in the case of open questions. Results. The overall response rate was 36% (262/719). The use of MSUS varied among members of the various networks/societies. MSUS was considered of high relevance for improvement of diagnostic skills, for the guidance of joint injections and for the assessment of specific joints, namely the hip, ankle, midfoot and wrist. It was considered useful for early detection of synovitis and in determining disease activity and disease remission. Conclusion. Although at present MSUS is not widely used by paediatric rheumatologists, there is considerable interest in this imaging technology among members of the international networks. The results of this survey suggest that the next objective in the research agenda should be the standardization of the assessment of joints in healthy children. This will then help differentiate pathological (i.e. synovitic) joints from normal joints. The initial target joints should be the hip, ankle, midfoot and wrist. MSUS training focused on the assessment of paediatric patients might be very important in implementing the use of this technique in clinical practice and research.

Sjogren's syndrome (SS) is a systemic autoimmune disease characterized by the infiltration of lymphocytes into exocrine glands, resulting in the typical sicca symptoms. Unlike adults, primary SS is a very rare condition in childhood, and the risk of malignancy in juvenile SS (JSS) has not been defined. We report the detection of extranodal marginal zone B-cell lymphoma (EMZL) occurring in two children with SS. Fine needle aspiration of the salivary glands (SG) showed nonspecific findings that led to delayed diagnosis of SS. The diagnosis of B-cell lymphoma associated with JSS was based on morphologic and immunohistochemical staining done during the biopsy. To highlight awareness of EMZL as a timely and appropriate update of an unusual complication in children with SS.

Juvenile idiopathic arthritis (JIA) is a heterogeneous group of arthritides characterized by chronic synovial inflammation that can lead to structural damage. The main objective of JIA therapies is to induce disease control to avoid disability in childhood. The advances in therapeutic effectiveness have created a need to search for imaging tools that describe more precisely disease activity in children with JIA. Musculoskeletal ultrasound and magnetic resonance imaging have demonstrated to be more sensitive than clinical examination in early detection of synovitis. These modalities can detect both inflammatory and destructive changes. The unique characteristics of the growing skeleton and a scarce validation of imaging in children result in important challenges in evaluating paediatric population. This review describes indications and limitations of these imaging techniques and suggests some advices for a rational use in the management of JIA in clinical practice.

Objective To develop evidence-based recommendations on the use of ultrasound (US) and magnetic resonance imaging (MRI) in patients with rheumatoid arthritis (RA). Methods Recommendations were generated following a nominal group technique. A panel of experts, consisting of 15 rheumatologists and 3 radiologists, was established in the first panel meeting to define the scope and purpose of the consensus document, as well as chapters, potential recommendations and systematic literature reviews (we used and updated those from previous EULAR documents). A first draft of recommendations and text was generated. Then, an electronic Delphi process (2 rounds) was carried out. Recommendations were voted from 1 (total disagreement) to 10 (total agreement). We defined agreement if at least 70% of experts voted ≥7. The level of evidence and grade or recommendation was assessed using the Oxford Centre for Evidence-based Medicine Levels of Evidence. The full text was circulated and reviewed by the panel. The consensus was coordinated by an expert methodologist. Results A total of 20 recommendations were proposed. They include the validity of US and MRI regarding inflammation and damage detection, diagnosis, prediction (structural damage progression, flare, treatment response, etc.), monitoring and the use of US guided injections/biopsies. Conclusions These recommendations will help clinicians use US and MRI in RA patients.

Objective To develop guidelines for Musculoskeletal Ultrasound (MSKUS) training for rheumatologists in the Americas. Methods A total of 25 Rheumatologists from 19 countries of the American Continent participated in a consensus-based interactive process (Delphi method) using 2 consecutive electronic questionnaires. The first questionnaire included the following: the relevance of organizing courses to teach MSKUS to Rheumatologists, the determination of the most effective educational course models, the trainee levels, the educational objectives, the requirements for passing the course(s), the course venues, the number of course participants per instructor, and the percentage of time spent in hands-on sessions. The second questionnaire consisted of questions that did not achieve consensus (>65%) in the first questionnaire, topics, and pathologies to be covered at each course MSKUS level. Results General consensus was obtained for MSKUS courses to be divided into 3 educational levels: basic, intermediate, and advanced. These courses should be taught using a theoretical-didactic and hands-on model. In addition, the group established the minimum requirements for attending and passing each MSKUS course level, the ideal number of course participants per instructor (4 participants/instructor), and the specific topics and musculoskeletal pathologies to be covered. In the same manner, the group concluded that 60% to 70% of course time should be focused on hands-on sessions. Conclusion A multinational group of MSKUS sonographers using a consensus-based questionnaire (Delphi method) established the first recommendations and guidelines for MSKUS course training in the Americas. Pan-American League of Associations for Rheumatology urges that these guidelines and recommendations be adopted in the future by both national and regional institutions in the American continent involved in the training of Rheumatologists for the performance of MSKUS.

Objective To evaluate the sensitivity to change of power Doppler ultrasound (PDUS) assessment of joint inflammation and the predictive value of PDUS parameters in disease activity and radiologic outcome in patients with early rheumatoid arthritis (RA). Methods Forty-two patients with early RA who started therapy with disease-modifying antirheumatic drugs underwent blinded sequential clinical, laboratory, and ultrasound assessment at baseline, 3 months, 6 months, and 1 year and radiographic assessment at baseline and 1 year. For each patient, 28-joint Disease Activity Score (DAS28) was recorded at each visit. The presence of synovitis was investigated in 28 joints using gray-scale ultrasonography and intraarticular power Doppler signal. Active synovitis was defined as intraarticular synovitis detected with power Doppler signal. The ultrasound joint count for active synovitis and an overall joint index for power Doppler signal were calculated. Sensitivity to change of PDUS variables was assessed by estimating the smallest detectable difference (SDD) from the intraobserver variability. Results The SDD for ultrasound joint count for active synovitis and ultrasound joint index for power Doppler signal was lower than mean changes from baseline to 3 months, 6 months, and 1 year. Time-integrated values of PDUS parameters demonstrated a highly significant correlation with DAS28 after 1 year (r = 0.63, P < 0.001) and a stronger correlation with radiographic progression (r = 0.59–0.66, P < 0.001) than clinical and laboratory parameters (r < 0.5). Conclusion PDUS is a sensitive and reliable method for longitudinal assessment of inflammatory activity in early RA. PDUS findings may have a predictive value in disease activity and radiographic outcome.

Kawasaki disease (KD) is an acute systemic vasculitis affecting medium-sized arteries, particularly the coronary arteries. Classic diagnosis is based in prolonged fever and different clinical features, including acute arthritis. Our objective is to determine the prevalence of arthritis at the moment of the diagnosis, the response to intravenous immunoglobulin infusion and the relation with cardiac findings.Retrospective study through review of medical records of 42 patients with KD from 1988 to 2013. Demographic, clinical, laboratory variables and treatment were reviewed.Male sex was predominant (57%). Fever (100%), exanthema (92,9%), conjunctivitis (78,6%), oropharingeal changes (76,2%), cervical lymphadenopathy (71,4%), edema (52,4%) and peripheral desquamation (46,3%) were reported. Eight patients presented ecocardiography alterations (ectasia and aneurism). Acute articular involvement was reported in 7 (16%) patients, including oligoarticular (57%), monoarticular (29%) and polyarticular (14%) patterns. All patients had elevation of acute phase reactants with neutrophilia (57%) and hypoalbuminemia (71,5%), but showed a good therapeutic response to intravenous immunoglobulin, without sequelaes. Sixteen patients had incomplete KD nine males, with 100% of fever exanthema (75%), conjunctivitis (56%) and 50% of cervical lymphadenopathy. Whereas oropharingeal changes and edemas was described in 44% and 25% of them. Four patients with incomplete KD had coronary artery abnormalities.Acute arthritis was an uncommon finding (16%) and resulted in no sequelae. Maybe the treatment with intravenous immunoglobulin and aspirin prevents the development of articular abnormalities and then leading to a decrease in its follow-up requierement by reumathologist. The cardiovascular sequelae, mainly incomplete KD, remains determining its prognosis. The presence of articular involvement seems not to have influence over cardiac involvement.