Implantació i perfecionament, mitjançant l’entorn virtual ATENEA, d’eines telemàtiques i activitats útils per a la formació i avaluació dels estudiants de l’assignatura de Geotècnia dins la titulació d’Enginyeria Tècnica d’Obres Públiques

La implantació dels nous plans d’estudi implica una major participació en les activitats docents per part dels estudiants; Geotècnia es prepara per a aquest sistema docent mitjançant l’ATENEA. S’ha apostat per metodologies docents amb suport TIC efectiu a través de l’Intranet de l’ETSECCPB i l’ús de Moodle (sistema de gestió amb especialització en l’aprenentatge on line), de manera que es recondueixi l’assignatura cap al nou Espai Europeu d’Educació Superior. A part del material habitual (apunts de classe, exercicis d’examen, etc.), s’està emfatitzant l’ús d’un dipòsit permanent de tot el material, amb l’ajut de l’ATENEA, mitjançant proves telemàtiques d’autoavaluació per a la formació dins del sistema metodològic de l’Avaluació Continuada.Peer Reviewe

Management of progressive pulmonary fibrosis associated with connective tissue disease

Introduction: Fibrotic interstitial lung disease (ILD) is a frequent and severe complication of connective tissue disease (CTD). Areas covered: In this narrative review, we update the most relevant differential characteristics of fibrotic ILD associated with CTD (CTD-ILD) and propose a diagnostic and therapeutic approach based on a review of the articles published between 2002 and 2022 through PubMed. Expert opinion: The subset of ILD, mainly the radiological/histological pattern and the degree of fibrotic component, usually determines the prognosis and therapeutic strategy for these patients. Some patients with CTD-ILD can develop progressive pulmonary fibrosis (PPF) with severe deterioration of lung function, rapid progression to chronic respiratory failure, and high mortality. PPF has been described in many CTDs, mainly in systemic sclerosis and rheumatoid arthritis, and requires a multidisciplinary diagnostic and therapeutic approach to improve patient outcomes

Shrinking lung syndrome in systemic lupus erythematosus: A case series and review of the literature

Shrinking lung syndrome (SLS) is a rare and less known complication mainly associated with systemic lupus erythematosus (SLE). In this study, we analyze the clinical features, investigation findings, approaches to management, and outcome in a case series of 9 adult patients with SLE and SLS diagnosed during a 35-year period in 3 referral tertiary care hospitals in Spain. Additionally, we reviewed 80 additional cases previously reported (PubMed 1965-2015). These 80 cases, together with our 9 patients, form the basis of the present analysis. The overall SLS prevalence in our SLE population was 1.1% (9/829). SLS may complicate SLE at any time over its course, and it usually occurs in patients without previous or concomitant major organ involvement. More than half of the patients had inactive lupus according to SELENA-systemic lupus erythematosus disease activity index (SLEDAI) scores. Typically, it presents with progressive exertional dyspnea of variable severity, accompanied by pleuritic chest pain in 76% of the cases. An important diagnostic delay is common. The diagnostic tools that showed better yield for SLS detection are the imaging techniques (chest x-ray and high-resolution computed tomography) along with pulmonary and diaphragmatic function tests. Evaluation of diaphragm dome motion by M-mode ultrasonography and phrenic nerve conduction studies are less useful. There are no standardized guidelines for the treatment of SLS in SLE. The majority of patients were treated with medium or high doses of glucocorticoids. Several immunosuppressive agents have been used in conjunction with steroids either if the patient fails to improve or since the beginning of the treatment. Theophylline and beta-agonists, alone or in combination with glucocorticoids, have been suggested with the intent to increase diaphragmatic strength. The overall long-term prognosis was good. The great majority of patients had significant clinical improvement and stabilization, or mild to moderate improvement on pulmonary function tests. The mortality rate was very low

Documento de expertos sobre el uso de terapia combinada de metotrexato con terapias biológicas o terapias dirigidas a pacientes con artritis reumatoide

We aimed to develop recommendations for the management of methotrexate (MTX) when considering the combination with biological (b) or targeted synthetic (ts) disease modifying drugs (DMARDs) in rheumatoid arthritis (RA). Methods: Eleven experts on RA were selected. Two coordinators formulated 13 questions about the combination therapy of MTX with bDMARDs or tsDMARDs. A systematic review was conducted to answer the questions. Inclusion and exclusion criteria were established as well as the search strategies (Medline, Embase and the Cochrane Library were searched up to January 2019). Two reviewers selected the articles and collected data. Simultaneously, EULAR and ACR meeting abstracts were evaluated. Based on this evidence, the coordinators proposed preliminary recommendations that the experts discussed and voted in a nominal group meeting. The level of evidence and grade of recommendation was established using the Oxford Center for Evidence Based Medicine and the level of agreement with a Delphi. Agreement was established if at least 80% of the experts voted ‘yes’ (yes/no). Results: The systematic review retrieved 513 citations of which 61 were finally included. A total of 10 recommendations were generated, voted and accepted. The level of agreement was very high in all of them and it was achieved in the first Delphi round. Final recommendations cover aspects such as the optimal MTX dosage, tapering strategy or patients’ risk management. Conclusions: This document is intended to help clinicians solve usual clinical questions and facilitate decision making when treating RA patients with MTX in combination with bDMARDs or tsDMARDsDesarrollar recomendaciones sobre el uso de metotrexato (MTX) en combinación con medicamentos modificadores de la enfermedad (DMARD) biológicos (b) o sintéticos específicos (ts) en la artritis reumatoide (AR). Se seleccionaron 11 expertos en AR. Dos coordinadores formularon 13 preguntas sobre la terapia combinada de MTX con bDMARD o tsDMARD. Se realizó una revisión sistemática para responder las preguntas. Se establecieron criterios de inclusión y exclusión, así como las estrategias de búsqueda (se realizaron búsquedas en Medline, Embase y la Biblioteca Cochrane hasta enero de 2019). Dos revisores seleccionaron los artículos y recopilaron datos. Simultáneamente, se evaluaron los resúmenes de las reuniones EULAR y ACR. Con base en esta evidencia, los coordinadores propusieron recomendaciones preliminares que los expertos discutieron y votaron en una reunión de grupo nominal. El nivel de evidencia y el grado de recomendación se establecieron utilizando el Centro de Oxford para Medicina Basada en Evidencia y el nivel de acuerdo con un Delphi. El acuerdo se estableció si al menos el 80% de los expertos votaron «sí» (sí/no). La revisión sistemática recuperó 513 citas, de las cuales finalmente se incluyeron 61. Se generaron, votaron y aceptaron un total de 10 recomendaciones. El nivel de acuerdo fue muy alto en todas ellas y se logró en la primera ronda de Delphi. Las recomendaciones finales cubren aspectos como la dosis óptima de MTX, la estrategia de reducción o la gestión del riesgo de los pacientes. Este documento está destinado a ayudar a los médicos a resolver preguntas clínicas habituales y facilitar la toma de decisiones al tratar a pacientes con AR con MTX, en combinación con bDMARD o tsDMAR

RIG-I expression in perifascicular myofibers is a reliable biomarker of dermatomyositis

Background: Dermatomyositis (DM) is inflammatory myopathy or myositis characterized by muscle weakness and skin manifestations. In the differential diagnosis of DM the evaluation of the muscle biopsy is of importance among other parameters. Perifascicular atrophy in the muscle biopsy is considered a hallmark of DM. However, perifascicular atrophy is not observed in all patients with DM and, conversely, perifascicular atrophy can be observed in other myositis such as antisynthetase syndrome (ASS), complicating DM diagnosis. Retinoic acid inducible-gene I (RIG-I), a receptor of innate immunity that promotes type I interferon, was observed in perifascicular areas in DM. We compared the value of RIG-I expression with perifascicular atrophy as a biomarker of DM. Methods: We studied by immunohistochemical analysis the expression of RIG-I and the presence of perifascicular atrophy in 115 coded muscle biopsies: 44 patients with DM, 18 with myositis with overlap, 8 with ASS, 27 with non-DM inflammatory myopathy (16 with polymyositis, 6 with inclusion body myositis, 5 with immune-mediated necrotizing myopathy), 8 with muscular dystrophy (4 with dysferlinopathy, 4 with fascioscapulohumeral muscle dystrophy) and 10 healthy controls. Results: We found RIG-I-positive fibers in 50% of DM samples vs 11% in non-DM samples (p < 0.001). Interestingly, RIG-I staining identified 32% of DM patients without perifascicular atrophy (p = 0.007). RIG-I sensitivity was higher than perifascicular atrophy (p < 0.001). No differences in specificity between perifascicular atrophy and RIG-I staining were found (92% vs 88%). RIG-I staining was more reproducible than perifascicular atrophy (κ coefficient 0.52 vs 0.37). Conclusions: The perifascicular pattern of RIG-I expression supports the diagnosis of DM. Of importance for clinical and therapeutic studies, the inclusion of RIG-I in the routine pathological staining of samples in inflammatory myopathy will allow us to gather more homogeneous subgroups of patients in terms of immunopathogenesis

Health Assessment Questionnaire-Disability Index (HAQ-DI) use in modelling disease progression in diffuse cutaneous systemic sclerosis: an analysis from the EUSTAR database

BACKGROUND: Patients with diffuse cutaneous systemic sclerosis (dcSSc) have a poor prognosis. The importance of monitoring subjective measures of functioning and disability, such as the Health Assessment Questionnaire-Disability Index (HAQ-DI), is important as dcSSc is rated by patients as worse than diabetes or hemodialysis for quality of life impairment. This European Scleroderma Trials and Research (EUSTAR) database analysis was undertaken to examine the importance of impaired functionality in dcSSc prognosis. The primary objectives were to identify predictors of death and HAQ-DI score progression over 1 year. HAQ-DI score, major advanced organ involvement, and death rate were also used to develop a comprehensive model to predict lifetime dcSSc progression. METHODS: This was an observational, longitudinal study in patients with dcSSc registered in EUSTAR. Death and HAQ-DI scores were, respectively, analyzed by Cox regression and linear regression analyses in relation to baseline covariates. A microsimulation Markov model was developed to estimate/predict natural progression of dcSSc over a patient's lifetime. RESULTS: The analysis included dcSSc patients with (N = 690) and without (N = 4132) HAQ-DI score assessments from the EUSTAR database. Baseline HAQ-DI score, corticosteroid treatment, and major advanced organ involvement were predictive of death on multivariable analysis; a 1-point increase in baseline HAQ-DI score multiplied the risk of death by 2.7 (p &lt; &nbsp;0.001) and multiple advanced major organ involvement multiplied the risk of death by 2.8 (p &lt; &nbsp;0.05). Multivariable analysis showed that baseline modified Rodnan Skin Score (mRSS) and baseline HAQ-DI score were associated with HAQ-DI score progression at 1 year (p &lt; &nbsp;0.05), but there was no association between baseline organ involvement and HAQ-DI score progression at 1 year. HAQ-DI score, major advanced organ involvement, and death were successfully used to model long-term disease progression in dcSSc. CONCLUSIONS: HAQ-DI score and major advanced organ involvement were comparable predictors of mortality risk in dcSSc. Baseline mRSS and baseline HAQ-DI score were predictive of HAQ-DI score progression at 1 year, indicating a correlation between these endpoints in monitoring disease progression. It is hoped that this EUSTAR analysis may change physician perception about the importance of the HAQ-DI score in dcSSc

Racial differences in systemic sclerosis disease presentation: a European Scleroderma Trials and Research group study

Objectives. Racial factors play a significant role in SSc. We evaluated differences in SSc presentations between white patients (WP), Asian patients (AP) and black patients (BP) and analysed the effects of geographical locations.Methods. SSc characteristics of patients from the EUSTAR cohort were cross-sectionally compared across racial groups using survival and multiple logistic regression analyses.Results. The study included 9162 WP, 341 AP and 181 BP. AP developed the first non-RP feature faster than WP but slower than BP. AP were less frequently anti-centromere (ACA; odds ratio (OR) = 0.4, P &lt; 0.001) and more frequently anti-topoisomerase-I autoantibodies (ATA) positive (OR = 1.2, P = 0.068), while BP were less likely to be ACA and ATA positive than were WP [OR(ACA) = 0.3, P &lt; 0.001; OR(ATA) = 0.5, P = 0.020]. AP had less often (OR = 0.7, P = 0.06) and BP more often (OR = 2.7, P &lt; 0.001) diffuse skin involvement than had WP.AP and BP were more likely to have pulmonary hypertension [OR(AP) = 2.6, P &lt; 0.001; OR(BP) = 2.7, P = 0.03 vs WP] and a reduced forced vital capacity [OR(AP) = 2.5, P &lt; 0.001; OR(BP) = 2.4, P &lt; 0.004] than were WP. AP more often had an impaired diffusing capacity of the lung than had BP and WP [OR(AP vs BP) = 1.9, P = 0.038; OR(AP vs WP) = 2.4, P &lt; 0.001]. After RP onset, AP and BP had a higher hazard to die than had WP [hazard ratio (HR) (AP) = 1.6, P = 0.011; HR(BP) = 2.1, P &lt; 0.001].Conclusion. Compared with WP, and mostly independent of geographical location, AP have a faster and earlier disease onset with high prevalences of ATA, pulmonary hypertension and forced vital capacity impairment and higher mortality. BP had the fastest disease onset, a high prevalence of diffuse skin involvement and nominally the highest mortality

Utilidad de los antagonistas de los receptores de la endotelina para la prevención primaria de hipertensión pulmonar en pacientes afectos de esclerosis sistémica

La esclerosis sistémica (ES) es una enfermedad autoinmune en la que el daño de la microcirculación es fundamental para la aparición de la enfermedad. Las complicaciones vasculares en la ES tienen una patogenia similar y son muy relevantes dentro de la enfermedad. Por ejemplo, las úlceras digitales (UDs) son una complicación frecuente en ES y la hipertensión arterial pulmonar (HAP) una de las principales causas de mortalidad. Se ha demostrado que el uso de los fármacos antagonistas receptores de la endotelina (ARE) es útil para el tratamiento de la HAP asociada a ES, así como para prevenir nuevos episodios de UD en pacientes con la enfermedad. Sin embargo, se desconoce si los ARE podrían ser útiles para prevenir la HAP. Objetivo: El objetivo de nuestro estudio es determinar si los ARE pueden ser útiles para la prevención de HAP asociada a ES, así como ver si existen diferencias en los resultados de ecocardiografía o en las pruebas de función respiratoria de los enfermos tratados o no con ARE. Material y Métodos: Estudio multicéntrico retrospectivo con diseño caso-control realizado con 237 pacientes con ES y UD. Se analizaron los datos durante el seguimiento de los pacientes tratados o no tratados con Bosentan (BOS) para la prevención secundaria de UD. Se definió la aparición de hipertensión pulmonar (HP) mediante la determinación ecocardiográfica de una presión arterial sistólica (PAPs) > 40mmHg durante el seguimiento. De todos los pacientes se recogieron variables demográficas, género, subtipo de la ES, manifestaciones clínicas de la enfermedad, datos de autoinmunidad, hallazgos de la capilaroscopia periungueal y los datos ecocardiográficos y de pruebas de función pulmonar realizados durante el seguimiento de los pacientes. Se consideró significancia estadística a aquellos valores de p < 0,05. Resultados: Quince pacientes presentaban cifras de PAPs por ecocardiografía > 40mmHg, y se excluyeron del análisis. De los 222 pacientes restantes la mayor parte eran mujeres (91%) con una edad media de 63,9±19,6 años. La primera manifestación de la enfermedad ocurrió a los 40,7±17,9 años, siendo el fenómeno de Raynaud la manifestación inicial más frecuente (85,6%). La presencia de UD fue constante en todos los enfermos. 59 pacientes (26,6%) fueron tratados con BOS. La dosis más habitual de BOS fue de 250mg diarios (60%) y se administró el tratamiento una mediana de 34 meses. Se registró sospecha de HP por ECO en el 21% de los pacientes. El 13,8% de pacientes en tratamiento con BOS presentaron hipertensión pulmonar, frente el 23,7% de los pacientes sin tratamiento (OR = 0,52; IC95% = 0,22 a 1,19; p>0,05). Al realizar el modelo de regresión estadística ajustado por los diferentes factores de confusión los pacientes no tratados con BOS presentaron 3,9 veces más hipertensión pulmonar que los pacientes tratados (OR 3,913; IC95%:1,322-11,58; p<0,02). Al analizar los resultados de las pruebas complementarias los pacientes con BOS no presentaron caída del porcentaje esperado de la capacidad de difusión de monóxido de carbono (DLCO) respecto a los valores al inicio del seguimiento (61,8±14% vs 57,3±20,1%; p: 0,89). Esto fue estadísticamente diferente (p<0,04) al comparar con los pacientes no tratados con BOS que presentaron una disminución de los niveles del porcentaje esperado de DLCO a final del seguimiento (65,5±20,2 vs 60,5±19,9; p<0,01). Conclusiones: Nuestro trabajo retrospectivo parece demostrar que aquellos enfermos tratados con BOS para la prevención de UD presentan un riesgo menor de desarrollar HP a lo largo de la enfermedad, así como la estabilización de los valores de DLCO. Estos datos apoyan la realización de un ensayo clínico prospectivo para estudiar el efecto de los ARE en la prevención de HAP en pacientes con ES.Systemic sclerosis (SSc) is a systemic autoimmune disease in which the damage of microcirculation is critical to develop the disease. In SSc, vascular complications have very similar pathogenic findings, which are relevant in the disease. For example, digital ulcers (DU) are a frequent complication in SSc patients and pulmonary arterial hypertension (PAH) is one of the leading causes of death. The use of endothelin receptor antagonists (ERAs) has been shown to be useful for the treatment of PAH related to SSc, and to prevent new episodes of DU in patients with the disease. However, it is not known if ERAs are useful for the prevention of PAH. Objective: The aim of our study is to determine if ERAs are useful to prevent PAH in SSc patients. Furthermore, we aim to determine if there are any differences in echocardiographic or pulmonary function tests for patients treated with or without ERAs. Methods: This was a retrospective, multicentre case-control study with 237 SSc patients with DU. Data were analysed during follow-up for patients treated or not treated with Bosentan (BOS) to prevent DU. The occurrence of pulmonary hypertension (PH) was defined by an echocardiogram (ECO) exhibiting systolic pulmonary arterial pressure (sPAP) > 40 mmHg at any stage of the follow up. For all patients, demographic variables, gender, SSc subtype, clinical involvement, autoimmunity data, capillaroscopy findings and different echocardiographic and pulmonary function test data, performed from baseline to follow-up were collected. Statistical significance was denoted by p values less than 0.05 Results: Fifteen patients had ECO values of sPAP> 40mmHg and were excluded from the analyses. Of the remaining 222 patients the majority were women (91%) with a mean age (±SD) of 63.9 (±19.6) years. The first manifestation of the disease occurred at 40.7 (±17.9) years, while Raynaud's phenomenon was the most frequent initial finding (85.6%). Fifty-nine patients (26.6%) were treated with BOS. The most common dose was 250mg daily (60%) and BOS was taken for a median of 34 months. In 21% of patients, PH was suspected due to ECO findings. During the follow up 13.8% of patients treated with BOS presented with PH, in comparison to 23.7% of untreated patients (OR 0.52, 95% CI: 0.22-1.19; p = 0.13). Adjusted regression analyses showed patients not treated with BOS were 3.9 times more likely to develop PH during follow-up (OR 3.913, CI95%:1.32-11.58; p < 0.02). Analysis of the tools of patient evaluation showed that the percentage of diffusing capacity for carbon monoxide (DLCO) in BOS-treated patients did not significantly decrease from baseline to the end of follow-up (61.8±14% vs 57±20.1%, p=0.89). This was statistically significant (p < 0.04) when compared to BOS-untreated patients who showed a significant decrease in the percentage of DLCO at the end of follow-up (65.5±20.2% vs 60.5±19.9%; p < 0.01). Conclusions: Our retrospective study shows that those patients treated with BOS to prevent UD have a lower risk to develop PH during the disease as well as stabilization of DLCO percentages. Our results support the need for a prospective, randomized, clinical trial to study the effect of ERA in prevention of PAH in SSc patients

Systematic Review of Systemic Corticosteroids for Treatment of Organizing Pneumonia

Introduction: Regardless corticosteroids are recommended for the treatment of organizing pneumonia there is limited evidence supporting this practice. Thus, we performed a systematic review of the literature on systemic corticosteroid treatment for organizing pneumonia. Methods: A search was implemented in the PubMed database (Medline) for articles published in the last 20 years. Those studies with incomplete or insufficient data and case reports were excluded. We collected data including: demographics, clinical data, diagnostic procedures, aetiology, treatment regimen (drug, posology, duration, response) and evolution. Results: A total of 135 publications were selected and finally 13 studies with 849 patients were included in the review: 12 retrospective observational studies and a single prospective observational study. Most of the patients were started on treatment with systemic corticosteroids – a total of 627 (30–100% depending on the series), but there was a great heterogeneity regarding drug, doses and duration. On those that started treatment, 226 (36%) presented a relapse of the disease during follow-up. Only one study provided information regarding treatment side-effects. Conclusion: The findings of this systematic review show the low quality data supporting the use of corticosteroids for the treatment of organizing pneumonia. This highlights a need to undertake appropriately designed studies to investigate which is the most appropriate treatment regimen that trades off benefits and risks of prolonged corticosteroid administration. Resumen: Introducción: Aunque los corticosteroides están recomendados para tratar la neumonía organizada, hay pocos datos que respalden esta práctica, por lo cual efectuamos una revisión sistemática de la bibliografía sobre el tratamiento con corticosteroides sistémicos para la neumonía organizada. Métodos: Se hizo una búsqueda en la base de datos PubMed (Medline) de artículos publicados en los últimos 20 años. Se descartaron los estudios con datos y casos clínicos incompletos o insuficientes. Los datos que recabamos abarcaron: datos demográficos, datos clínicos, técnicas diagnósticas, etiología, pauta terapéutica (fármaco, posología, duración, respuesta) y evolución. Resultados: Se eligieron 135 publicaciones en total y se incorporaron finalmente a la revisión 13 estudios con 849 pacientes: 12 estudios observacionales retrospectivos y un solo estudio observacional prospectivo. La mayor parte de los pacientes habían comenzado el tratamiento con corticosteroides sistémicos, un total de 627 (30%-100% en función de la serie), pero la duración, las dosis y el fármaco manifestaron una gran heterogeneidad. Entre los que habían empezado el tratamiento, 226 (36%) presentaron una recidiva de la enfermedad durante el seguimiento. Solo en un estudio se ofreció información sobre los efectos adversos del tratamiento. Conclusión: Los resultados de esta revisión ponen de manifiesto la escasa calidad de los datos sobre el tratamiento de la neumonía organizada con corticosteroides. Este hecho destaca la necesidad de emprender estudios diseñados correctamente para investigar la pauta terapéutica más adecuada que compense los riesgos y beneficios de la administración prolongada de corticosteroides