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El estudio tiene como objetivo investigar los sesgos asociados a variables socio-cognitivas y psicosociales relacionadas con la desinformación. Especialmente interesa examinar la reflexividad cognitiva, la conciencia metacognitiva, la identificación con el grupo, las ideologías políticas y la religiosidad como variables predictoras asociadas tanto a la identificación de mensajes de desinformación como a la intención de compartirlos. Se llevó a cabo un experimento en línea con 328 estudiantes universitarios (49% mujeres) para estimar la presencia del sesgo de la verdad ilusoria y el efecto de las condiciones de inoculación. Los participantes fueron asignados a una de tres condiciones de inoculación cognitiva (evaluación individual, metacognición o condición de control) y leyeron varios mensajes falsos y verdaderos sobre política y asuntos públicos. Nuestros resultados han revelado la presencia del efecto de la verdad ilusoria en la muestra costarricense. Las personas con mayor conciencia metacognitiva se vieron menos afectadas por el sesgo de la verdad ilusoria al evaluar tanto mensajes falsos como verdaderos. Los participantes que tienden a identificarse con su endogrupo presentan mayor probabilidad de percibir los mensajes falsos como verdaderos. Los hallazgos no mostraron una reducción sustancial del sesgo con las condiciones de inoculación.

It is widely known that test anxiety (TA) is associated with a decrease in test scores. The objective of this study is to provide evidence of the existence of two paths through which TA affects test scores: an indirect path that is associated with the mediation of the updating efficiency and a direct path moderated by the putting into perspective coping strategy. The study was conducted with a sample of 184 high school students who took an entrance exam for a university in Costa Rica. The structural equation modelling related to the theoretical approach was well adjusted, which provided evidence in favour of the hypotheses about the previously mentioned paths. Based on the results, the use of the putting into perspective coping strategy can help people with high TA levels reduce the effect of this emotion in test scores.

Objective To develop a multivariable model for predicting the progression of systemic sclerosis-associated interstitial lung disease (SSc-ILD) over 52 weeks.Methods We used logistic regression models to analyse associations between candidate predictors assessed at baseline and progression of SSc-ILD (absolute decline in forced vital capacity (FVC) % predicted >5% or death) over 52 weeks in the placebo group of the SENSCIS trial. Analyses were performed in the overall placebo group and in a subgroup with early and/or inflammatory SSc and/or severe skin fibrosis (18) at baseline. Model performance was assessed using the area under the receiver operating characteristic curve (AUC).Results In the overall placebo group (n=288), the performance of the final multivariable model for predicting SSc-ILD progression was moderate (apparent AUC: 0.63). A stronger model, with an apparent AUC of 0.75, was developed in the subgroup with early and/or inflammatory SSc and/or severe skin fibrosis at baseline (n=155). This model included diffusing capacity of the lung for carbon monoxide (DLco) % predicted, time since first non-Raynaud symptom, mRSS, anti-topoisomerase I antibody status and mycophenolate use.Conclusion Prediction of the progression of SSc-ILD may require different approaches in distinct subgroups of patients. Among patients with SSc-ILD and early and/or inflammatory SSc and/or severe skin fibrosis, a nomogram based on a multivariable model may be of value for identifying patients at risk of short-term progression.

Abstract Background Systemic sclerosis (SSc) is an autoimmune connective tissue disease characterized by vasculopathy and progressive fibrosis of skin and several internal organs, including lungs. Macrophages are the main cells involved in the immune-inflammatory damage of skin and lungs, and alternatively activated (M2) macrophages seem to have a profibrotic role through the release of profibrotic cytokines (IL10) and growth factors (TGFβ1). Nintedanib is a tyrosine kinase inhibitor targeting several fibrotic mediators and it is approved for the treatment of SSc-related interstitial lung disease (ILD). The study aimed to evaluate the effect of nintedanib in downregulating the profibrotic M2 phenotype in cultured monocyte-derived macrophages (MDMs) obtained from SSc-ILD patients. Methods Fourteen SSc patients, fulfilling the 2013 ACR/EULAR criteria for SSc, 10 SSc patients affected by ILD (SSc-ILD pts), 4 SSc patients non affected by ILD (SSc pts no-ILD), and 5 voluntary healthy subjects (HSs), were recruited at the Division of Clinical Rheumatology-University of Genova, after obtaining Ethical Committee approval and patients’ informed consent. Monocytes were isolated from peripheral blood, differentiated into MDMs, and then maintained in growth medium without any treatment (untreated cells), or treated with nintedanib (0.1 and 1µM) for 3, 16, and 24 h. Gene expression of macrophage scavenger receptors (CD204, CD163), mannose receptor-1 (CD206), Mer tyrosine kinase (MerTK), identifying M2 macrophages, together with TGFβ1 and IL10, were evaluated by quantitative real-time polymerase chain reaction. Protein synthesis was investigated by Western blotting and the level of active TGFβ1 was evaluated by ELISA. Statistical analysis was carried out using non-parametric Wilcoxon test. Results Cultured untreated SSc-ILD MDMs showed a significant increased protein synthesis of CD206 (p

Abstract Objectives The present study aimed to evaluate the measurement invariance of a general measure of the perception of governmental responses to COVID-|19 (COVID-SCORE-10) in the general population of 13 Latin American countries. Methods A total of 5780 individuals from 13 Latin American and Caribbean countries selected by non-probabilistic snowball sampling participated. A confirmatory factor analysis was performed and the alignment method was used to evaluate invariance. Additionally, a graded response model was used for the assessment of item characteristics. Results The results indicate that there is approximate measurement invariance of the COVID-SCORE-10 among the participating countries. Furthermore, IRT results suggest that the COVID-SCORE-10 measures with good psychometric ability a broad spectrum of the construct assessed, especially around average levels. Comparison of COVID-SCORE-10 scores indicated that participants from Cuba, Uruguay and El Salvador had the most positive perceptions of government actions to address the pandemic. Thus, the underlying construct of perception of government actions was equivalent in all countries. Conclusion The results show the importance of initially establishing the fundamental measurement properties and MI before inferring the cross-cultural universality of the construct to be measured.

Objectives Glucocorticosteroids (GCs) are the most used anti-inflammatory and immunosuppressive drugs due to their effectiveness in managing pain and disease modification in many immune-inflammatory rheumatic diseases (IRDs). However, their use is limited because of adverse effects (AEs). Material and methods The authors analyzed recent studies, including randomized controlled trials (RCTs), observational, translational studies and systematic reviews, providing an in-depth viewpoint on the benefits and drawbacks of GC use in rheumatology. Results Glucocorticosteroids are essential in managing life-threatening autoimmune diseases and a cornerstone in many IRDs given their swift onset of action, necessary in flares. Several RCTs and meta-analyses have demonstrated that when administered over a long time and on a low-dose basis, GC can slow the radiographic progression in early rheumatoid arthritis (RA) patients by at least 50%, satisfying the conventional definition of a disease-modifying anti-rheumatic drug (DMARD). In the context of RA treatment, the use of modified-release prednisone formulations at night may offer the option of respecting circadian rhythms of both inflammatory response and HPA activation, thereby enabling low-dose GC administration to mitigate nocturnal inflammation and prolonged morning fatigue and joint stiffness. Long-term GC use should be individualized based on patient characteristics and minimized due to their potential AEs. Their chronic use, especially at medium/high dosages, might cause irreversible organ damage due to the burden of metabolic systemic effects and increased risk of infections. Many international guidelines recommend tapering/withdrawal of GCs in sustained remission. Treat-to-target (T2T) strategies are critical in setting targets for disease activity and reducing/discontinuing GCs once control is achieved. Conclusions Glucocorticosteroids’ use in treating IRDs should be judicious, focused on minimizing use, tapering and discontinuing treatment, when possible, to improve long-term safety. Glucocorticosteroids remain part of many therapeutic regimens, particularly at low doses, and elderly RA patients, especially with associated chronic comorbidities, may benefit from long-term low-dose GC treatment. A personalized GC therapy is essential for optimal long-term outcomes.

Background and Objectives: Vitamin D is a secosteroid hormone essential for calcium homeostasis and skeletal health, but established evidence highlights its significant roles also in muscle health and in the modulation of immune response. This review aims to explore the impact of impaired vitamin D status on outcomes of muscle function and involvement in inflammatory and autoimmune rheumatic diseases damaging the skeletal muscle efficiency both with direct immune-mediated mechanisms and indirect processes such as sarcopenia. Methods: A comprehensive literature search was conducted on PubMed and Medline using Medical Subject Headings (MeSH) terms: “vitamin D, muscle, rheumatic diseases.” Additionally, conference abstracts from The European Alliance of Associations for Rheumatology (EULAR) and the American College of Rheumatology (ACR) (2020–2023) were reviewed, and reference lists of included papers were scanned. The review emphasizes the evidence published in the last five years, while also incorporating significant studies from earlier years, structured by the extent of evidence linking vitamin D to muscle health in the most commonly inflammatory and autoimmune rheumatic diseases encountered in clinical practice. Results: Observational studies indicate a high prevalence of vitamin D serum deficiency (mean serum concentrations < 10 ng/mL) or insufficiency (Conclusions: Altered serum vitamin D status is commonly observed in inflammatory and autoimmune rheumatic diseases and seems to be associated with adverse muscle health outcomes. While maintaining adequate serum vitamin D concentrations may confer muscle-protective effects, further research is needed to confirm these findings and establish optimal supplementation strategies to obtain a safe and efficient serum threshold.

Magnetoencephalography (MEG) non-invasively provides important information about human brain electrophysiology. The growing use of optically pumped magnetometers (OPM) for MEG, as opposed to fixed arrays of cryogenic sensors, has opened the door for innovation in system design and use cases. For example, cryogenic MEG systems are housed in large, shielded rooms to provide sufficient space for the system dewar. Here, we investigate the performance of OPM recordings inside of a cylindrical shield with a 1 × 2 m2 footprint. The efficacy of shielding was measured in terms of field attenuation and isotropy, and the value of post hoc noise reduction algorithms was also investigated. Localization accuracy was quantified for 104 OPM sensors mounted on a fixed helmet array based on simulations and recordings from a bespoke current dipole phantom. Passive shielding attenuated the vector field magnitude to 50.0 nT at direct current (DC), to 16.7 pT/√Hz at power line, and to 71 fT/√Hz (median) in the 10–200 Hz range. Post hoc noise reduction provided an additional 5–15 dB attenuation. Substantial field isotropy remained in the volume encompassing the sensor array. The consistency of the isotropy over months suggests that a field nulling solution could be readily applied. A current dipole phantom generating source activity at an appropriate magnitude for the human brain generated field fluctuations on the order of 0.5–1 pT. Phantom signals were localized with 3 mm localization accuracy, and no significant bias in localization was observed, which is in line with performance for cryogenic and OPM MEG systems. This validation of the performance of a small footprint MEG system opens the door for lower-cost MEG installations in terms of raw materials and facility space, as well as mobile imaging systems (e.g., truck-based). Such implementations are relevant for global adoption of MEG outside of highly resourced research and clinical institutions.

Objective: In the context of the pandemic due to SARS-CoV-2, concerns were raised about how to adequately assess its impact on the wellbeing of the population. The main reason of this paper is to present the preliminary results of psychometric properties of measures created to evaluate such impact. Method: We created and adapted several assessment instruments, and validated them with a volunteer sample of 699 adults (+18), inhabitants of Costa Rica, who filled out an online questionnaire on mental health consequences of the pandemic during September 2020. Results: The results provided evidence of validity and reliability for the new scales developed. Also, they show a negative correlation between resilient coping and acute stress, which could be considered evidence of discriminant validity. Conclusion: In this order, the measures showed acceptable psychometric properties with the evaluated sample. Future research is needed to gather more evidence of reliability and validity in probabilistic samples.

Background: Vaccination is one of the most important measures to contain the COVID-19 pandemic, especially for frail patients. VACCINATE is a multicentre prospective observational study promoted by the European Reference Network on Rare and Complex Connective Tissue and Musculoskeletal Diseases (ERN ReCONNET) aimed at assessing the long-term outcomes of COVID-19 vaccination in patients with rare and complex connective tissue diseases (rcCTDs) in terms of efficacy and safety. Methods: Adult rcCTDs patients were eligible for recruitment. Demographic, clinical and vaccination data were collected at enrolment. Follow-up visits were scheduled 4, 12, 24, 36 and 48 weeks after completion of the first vaccination cycle; data on adverse events, disease exacerbations and the occurrence of new SARS-CoV-2 infections were collected at these time-points. Findings: 365 rcCTDs patients (87 % female, mean age 51.8 ± 14.6 years) were recruited. Overall, 200 patients (54.8 %) experienced at least one adverse event, generally mild and in most cases occurring early after the vaccination. During follow-up, 55 disease exacerbations were recorded in 39 patients (10.7 %), distributed over the entire observation period, although most frequently within 4 weeks after completion of the vaccination cycle. The incidence of new SARS-CoV-2 infections was 8.9 per 1000 person-months, with no cases within 12 weeks from vaccine administration and an increasing trend of infections moving away from the primary vaccination cycle. Only one case of severe COVID-19 was reported during the study period. Interpretation: COVID-19 vaccination seems effective and safe in rcCTDs patients. The rate of new infections was rather low and serious infections were uncommon in our cohort. No increased risk of disease flares was observed compared to previous disease history; however, such exacerbations may be potentially severe, emphasising the need for close monitoring of our patients.

[Objetivo] El propósito del estudio fue examinar la relación entre autoeficacia matemática y el rendimiento en la prueba nacional de bachillerato en matemáticas, que considera otros predictores psicosociales relevantes y los efectos moderadores del sexo y del centro educativo. [Metodología] El diseño del estudio fue de corte observacional y transversal, con la participación de 487 estudiantes de undécimo año, provenientes de colegios públicos de la Gran Área Metropolitana de Costa Rica. Se aplicaron varios instrumentos para medir: sexismo hostil y benevolente, percepciones de igualdad de género en las habilidades matemáticas, autoeficacia matemática y habilidades de razonamiento. [Resultados] Los resultados del análisis multinivel bayesiano de efectos aleatorios confirmaron las diferencias a favor de los hombres en la medida de autoeficacia, donde el sexo del estudiantado fue un moderador entre la relación de la autoeficacia matemática y el puntaje de la prueba. Además, se encontró que, según el sexo, la asociación entre autoeficacia y rendimiento también se encontró moderada por el centro educativo. [Conclusiones] Entre las conclusiones se destaca que, si el efecto moderador del colegio entre autoeficacia y rendimiento difiere por sexo, esto implica que las acciones dirigidas a promover la autoeficacia no tendrán el mismo resultado en los hombres y en las mujeres, lo que evidencia la necesidad de desarrollar intervenciones diferenciadas.

Objective Nailfold videocapillaroscopy (NVC) allows the detection of microvascular damage in autoimmune connective tissue diseases (CTDs). The prevalence of the morphological capillary findings was retrospectively evaluated in a wide cohort of patients with Raynaud’s phenomenon secondary to a CTD at the time of the first single NVC, independently from their current treatment, autoantibody profile and comorbidities.Methods One-thousand-one-hundred-eighty-one patients affected by CTDs were included from 2001 to 2021. The considered CTDs were systemic sclerosis (SSc), undifferentiated connective tissue disease (UCTD), mixed connective tissue disease (MCTD), dermatomyositis (DM), systemic lupus erythematosus, Sjögren’s syndrome and primary antiphospholipid syndrome (aPS). The capillaroscopic parameters were distinguished between scleroderma patterns and non-scleroderma patterns.Results Giant capillaries were significantly more frequent in SSc, DM and MCTD than in other CTDs (respectively, in 73%, 73% and 61% of patients, p

Both glioblastoma (GBM) and dementia are devastating diseases with limited treatments that are usually not curative. Having clinically diagnosed dementia with an associated biopsy-proven etiology and a coexisting GBM diagnosis is a rare occurrence. The relationship between the development of neurodegenerative dementia and GBM is unclear, as there are conflicting reports in the literature. We present two cases of simultaneous biopsy-proven dementia, one with Alzheimer’s disease (AD) and GBM, and one with cerebral amyloid angiopathy (CAA) and GBM. We discuss how these diseases may be associated. Whether one pathologic process begins first or develops concurrently is unknown, but certain molecular pathways of dementia and GBM appear directly related while others inversely related. Further investigations of these close molecular relationships between dementia and GBM could lead to development of improved diagnostic tools and therapeutic interventions for both diseases.

Interstitial lung disease (ILD) is a fibrotic disease of the lung parenchyma. It can occur in different connective tissue diseases, including rheumatoid arthritis (RA). Smoking, male gender and longstanding RA are possible risk factors for developing ILD1. Being a common extra-articular manifestation of RA, it can contribute to decreased quality of life, chronic disability, high utilization of healthcare resources, and may also lead to substantial morbidity and mortality for affected patients1. Hence, early identification and management is of paramount importance to improve patient outcomes. Clinical presentation, chest X-ray, pulmonary function testing and high-resolution computed tomography are common tools for investigation and they also allows assessment of subtype and disease extent2. The histopathologic and radiographic features of RA-ILD are heterogeneous. The most frequent patterns of RA-ILD are usual interstitial pneumonia (UIP) and nonspecific interstitial pneumonia (NSIP). Distinguishing the patterns is useful in predicting prognosis and would also affect subsequent management approach2. Traditionally treatment initiated for RA-ILD was generally empirical. Corticosteroids were often used as first-line agents and immunosuppressants maybe added2. However, these treatments are not specific and mainly target inflammation instead of fibrosis. With the advance in medicine, antifibrotic is now indicated for treating fibrosing ILD with progressive phenotype. FVC decline can be slowed in patients with connective tissue disease associated progressive ILD3. In this lecture, Prof. Vanessa Smith will share the current knowledge and evidence in RA-ILD. The approach on identifying and screening ILD in RA patients would be presented. The management strategy and options would also be reviewed.

Objective To investigate the rate of decline in forced vital capacity (FVC), and the effect of nintedanib on the rate of decline in FVC, in subjects with systemic sclerosis-associated interstitial lung disease (SSc-ILD) who had risk factors for rapid decline in FVC.Methods The SENSCIS trial enrolled subjects with SSc and fibrotic ILD of ≥10% extent on high-resolution CT. The rate of decline in FVC over 52 weeks was analysed in all subjects and in those with early SSc (

Abstract Background In rheumatoid arthritis (RA), macrophages play an important role in modulating the immunoinflammatory response through their polarisation into “classically” (M1) or “alternatively activated” (M2) phenotypes. In RA, CTLA4-Ig (abatacept) reduces the inflammatory activity of macrophages by interacting with the costimulatory molecule CD86. The study aimed to investigate the efficacy of CTLA4-Ig treatment to induce an M2 phenotype both in M1-polarised monocyte-derived macrophages (MDMs) obtained from healthy subjects (HS) and in cultured MDMs obtained from active RA patients. Methods Cultured MDMs were obtained from peripheral blood mononuclear cells of 7 active RA patients and from 10 HS after stimulation with phorbol myristate acetate (5 ng/mL) for 24 h. HS-MDMs were then stimulated with lipopolysaccharide (LPS, 1 mg/mL) for 4 h to induce M1-MDMs. M1-MDMs and RA-MDMs were treated with CTLA4-Ig (100 μM and 500 μM) for 3, 12, 24, and 48 h. The gene expression of CD80, CD86, and TLR4 (M1 markers); CD163, CD204, and CD206 (surface M2 markers); and MerTK (functional M2 marker) was evaluated by qRT-PCR. The protein synthesis of surface M2 markers was investigated by Western blotting. The statistical analysis was performed by the Wilcoxon t-test. Results In LPS-induced HS-M1-MDMs, CTLA4-Ig 100 μM and 500 μM significantly downregulated the gene expression of M1 markers (3 h p

Objectives Tocilizumab showed trends for improving skin fibrosis and prevented progression of lung fibrosis in systemic sclerosis (SSc) in randomised controlled clinical trials. We aimed to assess safety and effectiveness of tocilizumab in a real-life setting using the European Scleroderma Trial and Research (EUSTAR) database.Methods Patients with SSc fulfilling the American College of Rheumatology (ACR)/EULAR 2013 classification criteria, with baseline and follow-up visits at 12±3 months, receiving tocilizumab or standard of care as the control group, were selected. Propensity score matching was applied. Primary endpoints were the modified Rodnan skin score (mRSS) and FVC at 12±3 months compared between the groups. Secondary endpoints were the percentage of progressive/regressive patients for skin and lung at 12±3 months.Results Ninety-three patients with SSc treated with tocilizumab and 3180 patients with SSc with standard of care fulfilled the inclusion criteria. Comparison between groups did not show significant differences, but favoured tocilizumab across all predefined primary and secondary endpoints: mRSS was lower in the tocilizumab group (difference −1.0, 95% CI −3.7 to 1.8, p=0.48). Similarly, FVC % predicted was higher in the tocilizumab group (difference 1.5 (−6.1 to 9.1), p=0.70). The percentage of progressive/regressive patients favoured tocilizumab over controls. These results were robust regarding the sensitivity analyses. Safety analysis confirmed previously reported adverse event profiles.Conclusion Although this large, observational, controlled, real-life EUSTAR study did not show significant effectiveness of tocilizumab on skin and lung fibrosis, the consistency of direction of all predefined endpoints generates hypothesis for potential effectiveness in a broader SSc population.

Abstract Background Circulating fibrocytes are an important source of fibroblasts and myofibroblasts, which are involved in fibrotic processes, including systemic sclerosis (SSc). The study aimed to investigate the effect of nintedanib (a tyrosine kinase inhibitor) in inhibiting the in vitro transition of circulating SSc fibrocytes into myofibroblasts and their pro-fibrotic activity. Methods Circulating fibrocytes were obtained from 18 SSc patients and 5 healthy subjects (HSs). Cultured SSc fibrocytes were maintained in growth medium (untreated cells) or treated with nintedanib 0.1 and 1 μM for 3 and 24 h. Fibroblast-specific protein-1 (S100A4) and α-smooth muscle actin (αSMA), as markers of fibroblast/myofibroblast phenotype, together with type I collagen (COL1) and fibronectin (FN), were investigated by qRT-PCR and Western blotting. Non-parametric tests were used for statistical analysis. Results Significantly elevated gene and protein expressions of αSMA, S100A4, COL1, and FN were observed in SSc fibrocytes compared to HS fibrocytes (gene: αSMA p < 0.001; others p < 0.0001; protein: all p < 0.05). Interestingly, an increased gene and protein expression of αSMA and S100A4 was found in fibrocytes from SSc patients positive for anti-Scl70 and with interstitial lung disease (ILD) (Scl70+ILD+) compared to Scl70−ILD− patients (S100A4: gene: p < 0.01; protein: p < 0.05), whereas no differences were observed for COL1 and FN. Nintedanib reduced gene and protein expression of αSMA, S100A4, COL1, and FN in SSc fibrocytes compared to untreated ones with different statistical significance. Noteworthy, nintedanib significantly downregulated gene and protein expression of αSMA, S100A4, COL1, and FN in Scl70+ILD+ fibrocytes (all p < 0.05), whereas only that of S100A4 and FN was significantly downregulated (p < 0.05) in Scl70−ILD− fibrocytes compared to the related untreated cells. Conclusions Nintedanib seems to downregulate in vitro the transition of fibrocytes into myofibroblasts and their pro-fibrotic activity, particularly in cells isolated from Scl70+ILD+ SSc patients.

Multidisciplinary team (MDT) meetings, involving the integrated collaboration of healthcare professionals, are increasingly used in clinical practice to inform the diagnosis and treatment of interstitial lung diseases (ILDs). Over time, the assessment of patients with ILD has transitioned from discussions among clinicians, radiologists and pathologists to the inclusion of a broader range of clinical data and specialist expertise. Studies have shown that a multidisciplinary approach can have many benefits for the clinical care of patients with ILD by improving the diagnostic confidence for different ILDs and guiding treatment decisions. The utility of MDT discussions for diagnosis, monitoring disease progression and management decisions, will need to be considered based on how it is best positioned in the diagnostic and therapeutic process, as well as the practicality and challenges of its use. There are also uncertainties and heterogeneity concerning the optimal practices of MDT meetings in ILD care. In this review, we describe recent developments refining the approach to MDTs in clinical practice, including who should be involved in the MDTs, when it is most needed, their use in patient management, challenges in their implementation, and ongoing controversies in the field that need further research.

BackgroundPatient registries play a crucial role in supporting clinical practice, healthcare planning and medical research, offering a real-world picture on rare and complex connective tissue diseases (rCTDs). ERN ReCONNET launched the first European Registry Infrastructure with the aim to plan, upgrade and link registries for rCTDs, with the final goal to promote a harmonized data collection approach all over Europe for rCTDs.MethodsAn online survey addressed to healthcare professionals and patients' representatives active in the field of rCTDs was integrated by an extensive database search in order to build a mapping of existing registries for rCTDs.FindingsA total of 140 registries were found, 38 of which include multiple diseases. No disease-specific registry was identified for relapsing polychondritis, mixed connective tissue disease and undifferentiated connective tissue disease.DiscussionThis overview on the existing registries for rCTDs provides a useful starting point to identify the gaps and the strengths of registries on the coverage of rCTDs, and to develop a common data set and data collection approach for the establishment of the TogethERN ReCONNET Infrastructure.

Background Nailfold videocapillaroscopy (NVC) non-specific abnormalities may be present in subjects with isolated Raynaud’s phenomenon (RP) before the potential transition to systemic sclerosis (SSc) specific microvascular alterations (‘scleroderma pattern’). This study aims to investigate NVC non-specific abnormalities, notably capillary dilations, in RP patients, as possible forerunners of the ‘scleroderma pattern’.Methods A 10-year retrospective NVC-based investigation evaluated 55 RP patients sorted into 3 sex-matched and age-matched groups according to clinical evolution: 18 later developing SSc (cases), 19 later developing other connective tissue disease and 18 maintaining primary RP at long-term follow-up (controls). All patients had a basal NVC showing non-specific abnormalities, namely non-specific >30 µm dilated capillaries (30–50 μm diameter). Sequential NVCs were longitudinally evaluated using current standardised approach. Statistical analysis assessed the risk for developing a ‘scleroderma pattern’.Results Significantly larger capillary diameters were observed in cases versus controls both at basal NVC and during follow-up NVC (p=30 µm dilated capillaries/mm at basal NVC was the strongest single predictor of ‘scleroderma pattern’ evolution with 24% increased risk per each dilated capillary (OR 1.24, 95% CI 1.17,1.32). Additionally, a tree-based analysis suggested the efferent (venous) diameter of the most dilated capillary on basal NVCas a variable of interest to identify patients maintaining primary RP.Conclusion This is the first study to describe an NVC ‘prescleroderma signature’ to potentially identify RP patients later developing a ‘scleroderma pattern’.

Abstract Background Antiphospholipid syndrome (APS) is a systemic autoimmune disease characterized by specific vascular and obstetric manifestations and by antiphospholipid antibodies (aPL) positivity. Microvascular damage in the course of APS and “aPL carrier” patients without symptoms is poorly investigated. Objectives This study aims to compare nailfold videocapillaroscopy (NVC) microvascular parameters in APS patients and non-symptomatic "aPL carriers" and to investigate their possible correlations with different aPL subtypes. Methods NVC was performed during standard evaluations in 18 APS patients (mean age 50 ± 13.8 years), 24 "aPL carriers" without symptoms (mean age 46.4 ± 16.4 years), and 18 control patients (CTR) (mean age 74 ± 12.5 years) taking oral anticoagulants for non-immunological indications (i.e., cardiovascular accidents). All patients were investigated for the presence of dilated capillaries, giant capillaries, microhemorrhages, capillary loss, and further non-specific/specific abnormalities (i.e., branched “bushy” capillaries, sign of neoangiogenesis) by NVC. Every alteration was also classified according to a semi-quantitative score. Lupus anticoagulant, anticardiolipin antibodies, and antibeta2 glycoprotein I antibodies were tested in each patient. Results APS patients showed at NVC increased frequency of microhemorrhages (p = 0.039)—particularly a “comb-like” pattern (parallel hemorrhages) (p = 0.002)—than "aPL carriers". Of note, there were no significant differences concerning the isolated number of microhemorrhages between APS and the CTR group (p = 0.314), but “comb-like” hemorrhages were significantly more frequent in the APS group (p = 0.034). Not any significant correlation was found between the aPL subtypes and NVC parameters. Conclusions APS patients showed significantly a greater number of non-specific NVC abnormalities than "aPL carriers", particularly the “comb-like” NVC pattern. Oral anticoagulants may represent a confounding factor for isolated microhemorrhages. Not any correlation was found between aPL subtypes and NVC parameters. Further investigations are needed to better characterize the microvascular endothelium damage induced by aPL.

Objective: To investigate in an unselected, systemic sclerosis (SSc) cohort if baseline laser speckle contrast analysis (LASCA) peripheral blood perfusion (PBP) measurements differ between ‘early’ SSc (without skin involvement, or ‘limited’ SSc—LSSc) and ‘clinically overt’ SSc (with skin involvement, limited cutaneous SSc—LcSSc and diffuse cutaneous SSc—DcSSc) in routine setting. Methods: A group of twenty consecutive ‘early’ SSc patients and forty consecutive ‘clinically overt’ SSc patients (twenty LcSSc and twenty DcSSc) underwent clinical and LASCA examinations (to assess the peripheral blood perfusion [PBP] of both hands volar). Results: No statistically significant difference in adjusted PBP was found in the ‘early’ versus the ‘clinically overt’ group (p = 0.77) when adjusted for possible confounding factors (e.g., vasoactive medication, active smoking, history of DTL and disease duration). A wide variability was noted when observing the individual datapoints of each subset. Conclusion: This study with an unselected SSc population in daily routine, non-research setting, showed there was no difference in adjusted PBP at baseline between ‘early’ SSc and ‘clinically overt’ SSc when corrected for possible confounding factors. Interestingly a wide variation of individual datapoints were observed in each subset, which emphasizes the heterogeneity of SSc.

Reconociendo que el género, como constructo, es expresión de una estructura de poder, se buscó explicar diferencias observadas a favor de los varones en dos pruebas estandarizadas de contexto matemático: componente matemático de la Prueba de Admisión de la Universidad de Costa Rica y la Prueba de Matemática del Bachillerato de la Educación Formal. La muestra fue de 487 estudiantes que en 2015 cursaban el undécimo año en 10 secundarias públicas diurnas, de la gran área metropolitana de Costa Rica, 269 eran mujeres. Se estimaron modelos de regresión multinivel con el puntaje en cada prueba como variable de respuesta. El modelo reducido solo incluyó como predictor el sexo de cada estudiante, y el modelo completo incluyó, además, un indicador de habilidades de razonamiento y los puntajes en las siguientes escalas: sexismo hostil y benevolente, actitud hacia la equidad de género en matemática y autoeficacia matemática. Los resultados apuntan a que las diferencias de género se reducen considerablemente al controlar habilidades de razonamiento. El sexismo benévolo predice el desempeño en las pruebas, aunque en menor medida. También se generó evidencia del efecto moderador del colegio para la relación entre el sexo y el puntaje en la prueba de admisión, y, especialmente, para los promedios de la prueba de bachillerato. La autoeficacia matemática también resultó de relevancia predictiva en esta última. Estos resultados evidencian la necesidad de que el personal docente promueva activamente esta dimensión de autoeficacia en sus estudiantes, especialmente en las mujeres. Las considerables diferencias en los promedios de las pruebas de Bachillerato, según colegio, aun siendo todos públicos y de la gran área metropolitana, sugieren la presencia de inequidades que deberían estudiarse con mayor profundidad, incluidos centros educativos de las diversas regiones del país, los cuales podrían presentar todavía mayores desigualdades.

Abstract Background Acculturation stress is associated with poorer physical and mental health and a lower level of psychological well-being. The causes of acculturation stress are diverse, but most are similar in the migrant population. Despite the importance of evaluating this variable, few studies have reported culturally adapted and validated instruments for specific populations. Based on this, the aim of this study was to evaluate the psychometric properties of a short scale for the evaluation of acculturation stress (EBEA). Methods Two studies were conducted, involving 1725 first-generation Colombian and Peruvian migrants living in Chile, between the ages of 18 and 60 years. In addition to the EBEA and as evidence of validity, the Beck Anxiety Scale and the WHOQOL-BREF psychological health domain were applied. A confirmatory factor analysis was carried out, and the reliability and nomological validity were evaluated. Results The results in both studies indicated that the scale presents a factorial structure of three dimensions: (a) the stress derived from the preparation and departure from the country of origin, (b) the stress produced by socioeconomic concerns in the host country, and (c) the tensions typical of adaptation to sociocultural changes or Chilean society. The reliability coefficients and the analysis of their nomological validity were very good. Conclusions The EBEA is a measure that offers quick, useful screening for researchers who need a short measure for research among migrants. This tool contributes to the work of education, prevention, and intervention in the field of general health and migrants’ mental health.

Esta investigación utiliza la perspectiva de la eficacia escolar considerando tres factores: características sociodemográficas, actitudes hacia la matemática y creencias sociales en torno al género en contextos matemáticos. El objetivo es explorar el valor predictivo de variables que podrían estar asociadas al rezago en asignaturas de contenido matemático en estudiantes de colegios ubicados en territorios indígenas y del Gran Área Metropolitana de Costa Rica (GAM). El estudio de corte transversal y correlacional se realizó con una muestra de 356 estudiantes, quienes respondieron cuatro escalas: autoeficacia matemática, utilidad de la matemática, creencias sexistas y estereotipos de género en contextos matemáticos. Se utilizaron modelos de regresión multinivel con dos variables dependientes: a) un contador de rezago para asignaturas de contenido matemático y b) una variable que identificó a quienes debieron presentar pruebas de aplazados en matemática. Los resultados mostraron la autoeficacia matemática como el principal predictor del rezago en ambas poblaciones y en ambas variables dependientes. Solo en los colegios del GAM fueron relevantes las predictoras edad, sexismo benevolente y equidad. Aunado a esto, el valor explicativo de los predictores en las variables dependientes, es considerablemente mayor para los colegios de la GAM. A pesar de las diferencias contextuales, se concluyeque la autoeficacia matemática es un predictor relevante para el rezago, reforzando la importancia de su estímulo en el aula. Otra conclusión es la necesidad de realizar estudios específicos en colegios de territorios indígenas, con el fin de contribuir a la comprensión de los factores asociados al rezago en esta población particular.

Se ha sugerido que la práctica profesional de un instrumento musical esta relacionada con mejores puntajes en tareas de funciones ejecutivas. Con el fin de examinar sistemáticamente esta hipótesis se realizó un metaanálisis para identificar el efecto de la interpretación de un instrumento en las funciones ejecutivas, así como las variables que podrían moderar dicho efecto. El metaanálisis incluyó estudios empíricos que: (1) compararon los puntajes en medidas de funciones ejecutivas entre músicos y no músicos; (2) reportaron las medias y desviaciones estándar; (3) el grupo de comparación no recibió entrenamiento musical; y (4) los participantes no presentaron discapacidad motora o cognitiva. Se identificó que los músicos, en comparación con los no músicos, muestran mejores puntajes en tareas de funciones ejecutivas (d = .712). Los resultados apoyan la hipótesis de que la práctica de un instrumento musical está relacionada con mejores puntuaciones en tareas de funciones ejecutivas.

Objective. The study aimed to develop and test the validity and reliability of a gender-based food intake stereotype scale (GBFISS) to further the understanding of gender stereotype influences on food intake. Design. Two cross-sectional studies were conducted among adolescents. In the first one (n = 611), exploratory and confirmatory factor analyses were performed on subsamples to identify and cross-validate the scale’s structure. Evidence of concurrent validity (correlation with sexism) was also examined. In the second study (n = 813), confirmatory factor analysis was conducted to confirm the scale’s dimensionality on a different sample. Further evidence of construct validity (correlations with food intake and social desirability) was examined. Invariance was tested for different features as well. Main outcome. The Gender-Based Food Intake Stereotype Scale. Results. Factor analyses on the first and second studies helped identify and confirm the GBFISS as a three-dimensional scale. The studies also provided evidence of construct validity. Support for invariance by gender and age was found, and reliability was acceptable. Conclusion. The evidence suggests that the GBFISS is valid and reliable. Further research is recommended. The contribution of gender stereotypes, as measured by the GBFISS, to well-established health behavior models should be examined.

En Chile, el incremento del flujo migratorio desde América Latina ha llamado la atención con respecto a los procesos de ajuste psicológico y sociocultural de los nuevos inmigrantes. Por tanto, con este trabajo se busca (a) establecer relaciones entre las orientaciones de aculturación, el estrés aculturativo y el bienestar psicológico, (b) identificar perfiles según las preferencias de aculturación, y (c) determinar la existencia de diferencias significativas en el estrés y el bienestar, de acuerdo con estas tipologías. Para esto, se realizó un estudio de tipo correlacional con una muestra de (N = 194) inmigrantes latinoamericanos con edades entre los 18 y los 67 años (M = 34.77; DE = 10.181), donde se evaluaron las orientaciones de aculturación, el estrés por aculturación y el bienestar psicológico. Los resultados indican que el predominio de la integración es más salutogénico que la tendencia a la separación cultural como orientación preeminente, lo que facilita un mejor afrontamiento de los estresores del proceso migratorio. Asimismo, se encontraron tres perfiles aculturativos individualista moderado, integracionista y separatista, y la comparación entre perfiles arroja que cuando la integración es más saliente, el bienestar es mayor que en presencia del individualismo como orientación predominante. Este trabajo aporta evidencia con respecto al individualismo como orientación de aculturación escasamente considerada en la literatura, cuya posible influencia negativa puede relacionarse con la pertenencia grupal como vía para acceder a recursos que aportan a la adaptación al nuevo contexto.

The PERSEIDS study aimed to estimate incidence/prevalence of interstitial lung diseases (ILDs), fibrosing interstitial lung diseases (F-ILDs), idiopathic pulmonary fibrosis (IPF), systemic sclerosis-associated ILD (SSc-ILD), other non-IPF F-ILDs and their progressive-fibrosing (PF) forms in six European countries, as current data are scarce. This retrospective, two-phase study used aggregate data (2014–2018). In Phase 1, incident/prevalent cases of ILDs above were identified from clinical databases through an algorithm based on codes/keywords, and incidence/prevalence was estimated. For non-IPF F-ILDs, the relative percentage of subtypes was also determined. In Phase 2, a subset of non-IPF F-ILD cases was manually reviewed to determine the percentage of PF behaviour and usual interstitial pneumonia-like (UIP-like) pattern. A weighted mean percentage of progression was calculated for each country and used to extrapolate incidence/prevalence of progressive-fibrosing ILDs (PF-ILDs). In 2018, incidence/105 person-years ranged between 9.4 and 83.6 (ILDs), 7.7 and 76.2 (F-ILDs), 0.4 and 10.3 (IPF), 6.6 and 71.7 (non-IPF F-ILDs), and 0.3 and 1.5 (SSc-ILD); and prevalence/105 persons ranged between 33.6 and 247.4 (ILDs), 26.7 and 236.8 (F-ILDs), 2.8 and 31.0 (IPF), 22.3 and 205.8 (non-IPF F-ILDs), and 1.4 and 10.1 (SSc-ILD). Among non-IPF F-ILDs, sarcoidosis was the most frequent subtype. PF behaviour and UIP-like pattern were present in a third of non-IPF F-ILD cases each and hypersensitivity pneumonitis showed the highest percentage of progressive behaviour. Incidence of PF-ILDs ranged between 2.1 and 14.5/105 person-years, and prevalence between 6.9 and 78.0/105 persons. To our knowledge, PERSEIDS is the first study assessing incidence, prevalence and rate of progression of ILDs across several European countries. Still below the threshold for orphan diseases, the estimates obtained were higher and more variable than reported in previous studies, but differences in study design/population must be considered.

Background: Specific autoantibodies and nailfold videocapillaroscopy (NVC) findings are serum and morphological diagnostic hallmarks of systemic sclerosis (SSc) as well as useful biomarkers which stratify the microvascular progression and prognosis of patients. Methods: The aim of our narrative review is to provide an update and overview of the link between SSc-related autoantibodies, used in clinical practice, and microvascular damage, evaluated by NVC, by exploring the interaction between these players in published studies. A narrative review was conducted by searching relevant keywords related to this field in Pubmed, Medline and EULAR/ACR conference abstracts with a focus on the findings published in the last 5 years. Results: Our search yielded 13 clinical studies and 10 pre-clinical studies. Most of the clinical studies (8/13, 61.5%) reported a significant association between SSc-related autoantibodies and NVC patterns: more specifically anti-centromere autoantibodies (ACA) were associated more often with an “Early” NVC pattern, whereas anti-topoisomerase autoantibodies (ATA) more frequently showed an “Active” or “Late” NVC pattern. Five studies, instead, did not find a significant association between specific autoantibodies and NVC findings. Among the pre-clinical studies, SSc-related autoantibodies showed different mechanisms of damage towards both endothelial cells, fibroblasts and smooth muscle vascular cells. Conclusions: The clinical and laboratory evidence on SSc-related autoantibodies and microvascular damage shows that these players are interconnected. Further clinical and demographic factors (e.g., age, sex, disease duration, treatment and comorbidities) might play an additional role in the SSc-related microvascular injury whose progression appears to be complex and multifactorial.

The present article describes the development of a Modern Biased Information Test (MBIT) inspired by the work published by Donald Campbell in 1950 on indirect measures of prejudice. A biased information test aims to tap individuals' intergroup attitudes from the selective information they use to describe group members. Two biased information tests were developed to measure ethnocentric and androcentric biases, respectively, and applied in four convenience samples of students from two different cultural settings (Costa Rica and the USA). The internal consistency for the accuracy indicators derived from both tests was acceptable and comparable across cultures. In contrast, the internal consistency for ethnocentric biases was adequate across samples and cultures, but the internal consistency for androcentric biases was unacceptable across both cultures. Results are discussed in the line of the usefulness of alternative measures for tapping implicit attitudes.

Parenting styles are a risk factor for adolescents overweight/obesity worldwide, but this association is not well understood in the context of Latin America. This study examines the association between the parenting styles of mothers and fathers and the risk of overweight/obesity among Costa Rican adolescents. Data are cross-sectional from a sample of adolescents (13–18 years old) enrolled in ten urban and eight rural schools (n = 18) in the province of San José, Costa Rica, in 2017. Hierarchical logistic regression analyses were performed to assess the likelihood of adolescents being overweight according to the mothers’ and fathers’ parenting styles. A significant association was found between the risk of adolescent overweight/obesity and the paternal authoritarian style only in rural areas (B = 0.622, SE = 0.317, Wald = 3.864, ExpB = 1.863, p = 0.04), and between said risk and the paternal permissive style only in male adolescents (B = 0.901, SE = 0.435, Wald = 4.286, ExpB = 2.461, p = 0.038). For maternal parenting styles, no associations reached significant levels once logistic regression models were adjusted for the fathers’ parenting styles. These findings underscore the importance of further studying the role of fathers’ paternal parenting styles on Latin American adolescent weight outcomes. Expanding our understanding of the parenting styles of fathers has important implications for the design and implementation of culturally- and gender-appropriate family interventions.

Active vitamin D [1,25(OH)2D3—calcitriol] is a secosteroid hormone whose receptor is expressed on all cells of the immune system. Vitamin D has a global anti-inflammatory effect and its role in the management of a SARS-CoV-2 infection has been investigated since the beginning of the COVID-19 pandemic. In this narrative review, the laboratory and clinical results of a vitamin D supplementation have been collected from both open-label and blinded randomized clinical trials. The results are generally in favor of the utility of maintaining the serum concentrations of calcifediol [25(OH)D3] at around 40 ng/mL and of the absolute usefulness of its supplementation in subjects with deficient serum levels. However, two very recent large-scale studies (one open-label, one placebo-controlled) have called into question the contribution of vitamin D to clinical practice in the era of COVID-19 vaccinations. The precise role of a vitamin D supplementation in the anti-COVID-19 armamentarium requires further investigations in light of the breakthrough which has been achieved with mass vaccinations.

OBJECTIVES Characteristics of Swiss patients with systemic sclerosis have not been described so far. The aim of the current study was to identify unmet needs in comparison with other European countries that could inform specific interventions to improve the care of systemic sclerosis patients. METHODS We analysed Swiss and other European systemic sclerosis patients registered in European Scleroderma Trials And Research (EUSTAR) and the Very Early Diagnosis Of Systemic Sclerosis (VEDOSS) cohort. Demographics, clinical profiles, organ involvement and survival of established, early/mild and very early / very mild systemic sclerosis patients were described and compared between the cohorts. RESULTS We included 679 Swiss and 8793 European systemic sclerosis patients in the analysis. Over 95% of patients in both cohorts were Caucasian, disease subsets were similar, and no age difference was found. The Swiss cohort had more male patients (25% vs 16% European, p = 0.005) and higher prevalence of early/mild and very early / very mild patients (26.1 vs 8.5% European and 14.9% vs 6.7% European, respectively, both p

Vitamin D [1,25(OH)2D—calcitriol] is basically a steroid hormone with pleiotropic biologic effects, and its impact on the regulation of immune system may influence several clinical conditions. Calcidiol (25OHD), as precursor of calcitriol, derives, for the most part (80%), from cutaneous cholesterol (7-dehydrocholesterol) under the action of UV-B (sunlight). Consequently, serum concentrations fluctuate during the year following the circannual rhythm of sun exposition. We will update about the available evidence regarding the complex influence of seasonal vitamin D changes on two different chronic connective tissue diseases, namely rheumatoid arthritis (RA) and systemic sclerosis (SSc). Notably, RA is an emblematic model of autoimmune disease with prevalent joint inflammatory features, while SSc is mainly an autoimmune progressive pro-fibrotic disease. However, in both conditions, low serum concentrations of 25OHD are involved in the pathogenesis of the diseases, and emerging data report their impact on clinical manifestations.

Immune checkpoint inhibition has revolutionized the treatment for numerous cancer patients; however, the spectrum of immune-related adverse events (irAEs) remains to be fully uncovered. We report a 48-year-old woman who was treated in the Phase III IMpassion130 study (atezolizumab and nanoparticle albumin-bound [nab]-paclitaxel) for metastatic triple-negative breast cancer. She experienced a partial response after 3 months. Nevertheless, the patient presented with thickening of the skin and muscle fatigue in the distal extremities together with blood eosinophilia after 15 months. Skin biopsy and magnetic resonance imaging were diagnostic of eosinophilic fasciitis (EF). Symptoms clearly improved upon stopping atezolizumab, while tumor response is still ongoing after discontinuing treatment. We identified five other cases of EF during immunotherapy, all occurring after about 1 year and in contrast to our case, mostly accompanied by other irAEs. This highlights that even if EF is a rare irAE, timely recognition and management remains important.

Background. Whereas parental feeding styles (PFS) influence children’s diet, less is known about this relationship in adolescents. Methods. A cross-sectional study in 686 Costa Rican adolescents (13–18 years) evaluated 4 validated PFS scores: healthy eating verbal encouragement; scolding; directly controlling diet; instrumental/emotional. Diet was evaluated through 3-day food records, deriving a Traditional Costa Rica Adolescents Diet Score (TCRAD). Excess weight (EW) measured by BMI was dichotomized following standards. Regression-based mediation analysis estimated the overall and sex-stratified odds ratios of EW for natural direct (NDE), natural indirect (NIE), and total effects (TE) of the pathway PFS→TCRAD→EW. Results. A one-unit increase in the direct control PFS score was associated with higher EW odds overall [(TE: 1.55; 95% CI: 1.04–2.31; p-value = 0.033), (NDE: 1.52; 95% CI: 1.02–2.27; p-value = 0.039)], and in boys [(TE: 2.13; 95% CI: 1.04–4.38; p-value = 0.039), (NDE: 2.10; 95% CI: 1.03–4.31; p-value = 0.042)]. Non-significant mediation by TCRAD was observed for the healthy eating verbal encouragement PFS overall (p-value = 0.06). Associations for the instrumental/emotional and scolding PFS were not significant. Conclusions. Direct diet control from parents may contribute to adolescents’ excess weight, particularly among boys. Parents encouraging healthy eating might support adolescents’ healthy weight through a healthy diet. Longitudinal research should clarify the association between PFS and diet-related outcomes among diverse adolescents.