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Introduction Idiopathic pulmonary fibrosis (IPF) is progressive and irreversible. Some discrepancies about IPF staging exists, especially in mild phases. Forced vital capacity (FVC) higher than 80% has been considered early or mild IPF even for the design of clinical trials. Methods Spanish multicentre, observational, retrospective study of IPF patients diagnosed between 2012 and 2016, based on the ATS/ERS criteria, which presented FVC greater or equal 80% at diagnosis. Clinical and demographic characteristics, lung function, radiological pattern, treatment, and follow-up were analyzed. Results 225 IPF patients were included, 72.9% were men. The mean age was 69.5 years. The predominant high-resolution computed tomography (HRCT) pattern was consistent usual interstitial pneumonia (UIP) (51.6%). 84.7% of patients presented respiratory symptoms (exertional dyspnea and/or cough) and 33.33% showed oxygen desaturation below 90% in the 6 min walking test (6MWT). Anti-fibrotic treatment was initiated at diagnosis in 55.11% of patients. Median FVC was 89.6% (IQR 17) and 58.7% of patients had a decrease of diffusion lung capacity for carbon monoxide (DLCO) below 60% of theoretical value; most of them presented functional progression (61.4%) and higher mortality at 3 years (20.45%). A statistically significant correlation with the 3-years mortality was observed between DLCO Conclusions Patients with preserved FVC but presenting UIP radiological pattern and moderate–severe DLCO decrease at diagnosis associate an increased risk of progression, death or lung transplantation. Therefore, in these cases, preserved FVC would not be representative of early or mild IPF.

Supported by the National Health and Medical Research Council Centre of Research Excellence in Pulmonary Fibrosis (GNT1116371), and by foundation partner, Boehringer Ingelheim, and program partners, Roche and Galapagos. Rationale : The interstitial lung disease (ILD) multidisciplinary meetings (MDM), composed of pulmonologists, radiologists, and pathologists, is integral to the rendering of an accurate ILD diagnosis. However, there is significant heterogeneity in the conduct of ILD MDMs, and questions regarding their best practices remain unanswered. Objectives : To achieve consensus among ILD experts on essential components of an ILD MDM. Methods : Using a Delphi methodology, semi-structured interviews with ILD experts were used to identify key themes and features of ILD MDMs. These items informed two subsequent rounds of online questionnaires that were used to achieve consensus among a broader, international panel of ILD experts. Experts were asked to rate their level of agreement on a five-point Likert scale. An a priori threshold for consensus was set at a median score 4 or 5 with an interquartile range of 0. Results : We interviewed 15 ILD experts, and 102 ILD experts participated in the online questionnaires. Five items and two exploratory statements achieved consensus on being essential for an ILD MDM following two questionnaire rounds. There was consensus that the presence of at least one radiologist, a quiet setting with a visual projection system, a high-quality chest high-resolution computed tomography, and a standardized template summarizing collated patient data are essential components of an ILD MDM. Experts also agreed that it would be useful for ILD MDMs to undergo an annual benchmarking process and a validation process by fulfilling a minimum number of cases annually. Twenty-seven additional features were considered to be either highly desirable or desirable features based on the degree of consensus. Although our findings on desirable features are similar to the current literature, several of these remain controversial and warrant further research. The study also showed an agreement among participants on several future concepts to improve the ILD MDM, such as performing regular self-assessments and conducting research into shared practices to develop an international expert guideline statement on ILD MDMs. Conclusions : This Delphi study showed consensus among international ILD experts on essential and desirable features of an ILD MDM. Our data represent an important step toward potential collaborative research into future standardization of ILD MDMs. Postprint

[EN] In this paper, the maximum achievable throughput is analyzed in the intra-wagon channel when multiple-input single-output (MISO) and orthogonal frequency division multiplexing (OFDM), MISO-OFDM, techniques are used. This analysis is performed from real wideband propagation channel measurements at 28 and 37 GHz, two potential frequency bands to deploy the future fifth-generation (5G) wireless communications networks. Four different scenarios in terms of the access point (AP) and user equipment (UE) positions inside the wagon have been considered, using 4 and 8 antennas at the AP. The performance of both quasi-orthogonal space-time block code (QSTBC), combined with Hadamard matrices, and transmit beamforming techniques is studied and evaluated from simulation results. The simulation results take into account the signal-to-noise ratio (SNR) and the antenna correlation for each antenna array configuration at the AP. These results provide useful insight to better understand the intra-wagon channel properties and deploy the future 5G wireless networks in this particular scenario at mmWave frequencies, where high-data-rates are expected to support different types of digital applications., This work was supported in part by the Ministerio de Economia y Competitividad MINECO, Spain, under Grant TEC2016-78028C3-2-P and Grant TEC2017-86779-C2-2-R and in part by the European FEDER Funds.

Objective To test whether the use of rituximab (RTX) is effective and safe as a rescue therapy add-on treatment to mycophenolate (MMF) in patients with progressive systemic sclerosis-associated interstitial lung disease (SSc-ILD) in whom conventional immunosuppressants (IS) have failed. Methods Longitudinal retrospective observational study of a cohort of patients with SSc-ILD that started treatment with RTX due to ongoing lung function impairment despite treatment with glucocorticoids and IS (cyclophosphamide and/or MMF). All patients were treated with 2 or more cycles of RTX and evaluated for at least 12 months. Results Twenty-four patients were included. Before initiation of RTX the mean decline in%pFVC and %pDLCO during the previous 2 years (delta) was −12.9% and −12.5%, respectively. After 1 year of treatment with RTX, a significant improvement in %pFVC (∆+8.8% compared to baseline, 95% CI: −13.7 to −3.9; p = 0.001) and%pDLCO (∆+4.6%, 95% CI: −8.2 to −0.8; p = 0.018) was observed. In addition, there was a significant reduction in the median dose of prednisone and it could be suspended in 25% of patients. At 2 years of treatment, RTX had been discontinued in 9 patients (due to adverse events in 3 cases and inefficacy in 6). In the 15 patients (62.5%) that completed 24 months of therapy, the statistically significant amelioration in pulmonary function test parameters was maintained: ∆%pFVC: +11.1% (95% CI: −17.6 to −4.5; p = 0.003) and ∆%pDLCO: +8.7% (95% CI: −13.9 to −8.3; p = 0.003). Conclusion Based on our results, RTX's use as an add-on treatment to MMF appears to be effective as a rescue therapy in patients with a more aggressive SSc-ILD phenotype.

Resumen: La infección por SARS-CoV-2 puede favorecer el desarrollo de diversas secuelas respiratorias, sobre todo en los pacientes que han sufrido una neumonía grave por COVID-19. Dado el elevado número de pacientes que sufrieron esta infección en un corto periodo de tiempo, se están llevando a cabo numerosas visitas de control post-COVID-19 sin que se haya establecido un protocolo de seguimiento clínico que aconseje sobre las pruebas complementarias a realizar y la frecuencia de las mismas. Este documento de consenso realizado por profesionales de distintas áreas de la Sociedad Española de Neumología y Cirugía Torácica (SEPAR), pretende ayudar al profesional clínico a la identificación de las posibles complicaciones respiratorias que pueden aparecen durante los meses posteriores al cuadro agudo de la enfermedad y a protocolizar su seguimiento y las pruebas complementarias a realizar. Se sugieren las exploraciones e intervenciones a realizar en diversos momentos de la evolución post-COVID-19, con unos objetivos concretos. Por un lado, garantizar que los pacientes reciban un seguimiento clínico oportuno, con un cronograma preestablecido teniendo en cuenta la gravedad de la enfermedad y la probabilidad de secuelas a largo plazo; por otro lado, evitar sobrecargas del sistema sanitario llevando a cabo exploraciones y/o consultas en muchos casos innecesarias; por último, definir criterios para derivar a aquellos pacientes con determinadas secuelas específicas establecidas (enfermedad pulmonar intersticial, enfermedad vascular pulmonar o bronquiectasias) a las unidades monográficas correspondientes. Abstract: SARS-CoV-2 infection can cause a range of respiratory sequelae, especially in patients who have had severe Covid-19 pneumonia. Given the high number of patients who have developed this infection over a short period of time, numerous post-Covid-19 follow-up visits are being carried out, but no clinical follow-up protocol has been established to advise on the complementary tests to be performed and the frequency of these procedures. This consensus document was drawn up by professionals from different areas of the Spanish Society of Pulmonology and Thoracic Surgery (SEPAR) in order to assist the clinician in identifying possible respiratory complications that may occur during the months following the acute disease, and to protocolize their follow-up and additional tests to be performed. It recommends examinations and interventions to be carried out at various stages in the post-Covid-19 period, and details the specific objectives of these procedures. Primarily, we aim to ensure that patients receive timely clinical follow-up, following a pre-established schedule that takes into account the severity of the disease and the likelihood of long-term sequelae. Another objective is to avoid overloading the health system by eschewing examinations and/or consultations that are, in many cases, unnecessary. Finally, we define criteria for referring patients with specific established sequelae (interstitial lung disease, pulmonary vascular disease, bronchiectasis) to the corresponding specialized units.

Lung disease is common in patients with rheumatoid arthritis (RA). The onset of lung involvement in RA is not well known. The objective is to describe the features and evolution of lung involvement in early RA, its relationship with disease activity parameters, smoking and treatments. Consecutive patients with early RA without respiratory symptoms were included and tracked for 5 years. Lung assessment included clinical, radiological and pulmonary function tests at diagnosis and during follow-up. Peripheral blood parameters (erythrocyte sedimentation rate, C reactive protein, rheumatoid factor and anti-citrullinated peptide autoantibodies) and scales of articular involvement, such as DAS28-CRP, were evaluated. 40 patients were included and 32 completed the 5-year follow up. 13 patients presented lung involvement in the initial 5 years after RA diagnosis, 3 of them interstitial lung disease. Significant decrease of diffusion lung transfer capacity of carbon monoxide over time was observed in six patients, 2 of them developed interstitial lung disease. DLCO decrease was correlated with higher values of CRP and ESR at diagnosis. Methotrexate was not associated with DLCO deterioration or lung disease development. Subclinical progressive lung disease correlates with RA activity parameters. Smoking status and methotrexate were not associated with development or progression of lung disease.

Progressive connective tissue disease (CTD)-related lung disease is a challenging condition that requires lung transplantation in some patients. Treatment with rituximab may improve lung function. To evaluate the effect of rituximab in patients with progressive CTD-related lung disease who met criteria for inclusion in waiting list for a lung transplant. Retrospective study of patients with progressive CTD-related lung disease with criteria for lung transplant (FVC < 60% and/or DLCO < 40%) that started treatment with rituximab because of disease progression. Clinical variables, pulmonary function tests and chest computed tomography were used to monitor the effect of rituximab. The cohort included 18 patients; systemic sclerosis (7), rheumatoid arthritis (5), systemic lupus erythematosus (4), Sjogren syndrome (1) and antisynthetase syndrome (1). The radiologic patterns observed were: usual interstitial pneumonia (1), non-specific interstitial pneumonia (9), lymphoid interstitial pneumonia (1), emphysema-usual interstitial pneumonia (1), shrinking lung syndrome (3) and undetermined pattern (3). Over the previous year to rituximab initiation a decline in FVC (- 3.8%, p = 0.095) and DLCO (- 8.4%, p = 0.004) was observed. After 2 years of treatment, DLCO significantly improved (+ 12.4%, p < 0.001 at 1 year and + 15.3%, p = 0.001 at 2 years). Six patients (33.3%) presented adverse events related to rituximab. No patient required lung transplant or died during the study period. Rituximab is an effective treatment for patients with severe and progressive CTD-related lung disease, which allows to delay lung transplantation in some cases.

BackgroundAdvances in life expectancy have led to an increase in the number of elderly people with end-stage renal disease (ESRD). Scarce information is available on the outcomes of kidney transplantation (KT) in extremely elderly patients based on an allocation policy prioritizing donor–recipient age matching.MethodsWe included recipients ≥75 years that underwent KT from similarly aged deceased donors at our institution between 2002 and 2015. Determinants of death-censored graft and patient survival were assessed by Cox regression.ResultsWe included 138 recipients with a median follow-up of 38.8 months. Median (interquartile range) age of recipients and donors was 77.5 (76.3–79.7) and 77.0 years (74.7–79.0), with 22.5% of donors ≥80 years. Primary graft non-function occurred in 8.0% (11/138) of patients. Cumulative incidence rates for post-transplant infection and biopsy-proven acute rejection (BPAR) were 70.3% (97/138) and 15.2% (21/138), respectively. One- and 5-year patient survival were 82.1 and 60.1%, respectively, whereas the corresponding rates for death-censored graft survival were 95.6 and 93.1%. Infection was the leading cause of death (46.0% of fatal cases). The occurrence of BPAR was associated with lower 1-year patient survival [hazard ratio (HR) = 4.21, 95% confidence interval (CI) 1.64–10.82; P = 0.003]. Diabetic nephropathy was the only factor predicting 5-year death-censored graft survival (HR = 4.82, 95% CI 1.08–21.56; P = 0.040).ConclusionsESRD patients ≥75 years can access KT and remain dialysis free for their remaining lifespan by using grafts from extremely aged deceased donors, yielding encouraging results in terms of recipient and graft survival.

The diagnosis of idiopathic pulmonary fibrosis (IPF) is a complex process that requires the multidisciplinary integration of clinical, radiological, and histological variables. Due to its diagnostic yield, surgical lung biopsy has been the recommended procedure for obtaining samples of lung parenchyma, when required. However, given the morbidity and mortality of this technique, alternative techniques which carry a lower risk have been explored. The most important of these is transbronchial cryobiopsy -transbronchial biopsy with a cryoprobe- which is useful for obtaining lung tissue with less comorbidity. Yield may be lower than surgical biopsy, but it is higher than with transbronchial biopsy with standard forceps. This option has been discussed in the recent clinical guidelines for the diagnosis of IPF, but the authors do not go so far as recommend it. The aim of this article, the result of a multidisciplinary discussion forum, is to review current evidence and make proposals for the use of transbronchial cryobiopsy in the diagnosis of IPF.

This research was supported by Asociacion Espanola de LAM; The LAM Foundation Seed Grant 2019; Carlos III Institute of Health grants PI18/01029, PI21/01306 and ICI19/00047 (co-funded by European Regional Development Fund (ERDF), "A way to build Europe"); Ministry of Economy and Competitivity grant SAF2017-88457-R; the Generalitat de Catalunya SGR 2017-449 and 2017-529; PERIS PFI-Salut SLT017-20-000076; and the CERCA Program to IDIBELL and Institut Germans Trias i Pujol. X. Farre is supported by the VEIS project (001-P-001647, ERDF Operational Programme of Catalonia 2014-2020; co-funded by ERDF, "A way to build Europe"). Funding information for this article has been deposited with the Crossref Funder Registry., Introduction Lymphangioleiomyomatosis (LAM) is a rare low-grade metastasising disease characterised by cystic lung destruction. The genetic basis of LAM remains incompletely determined, and the disease cell-of-origin is uncertain. We analysed the possibility of a shared genetic basis between LAM and cancer, and LAM and pulmonary function. Methods The results of genome-wide association studies of LAM, 17 cancer types and spirometry measures (forced expiratory volume in 1 s (FEV1), forced vital capacity (FVC), FEV1/FVC ratio and peak expiratory flow (PEF)) were analysed for genetic correlations, shared genetic variants and causality. Genomic and transcriptomic data were examined, and immunodetection assays were performed to evaluate pleiotropic genes. Results There were no significant overall genetic correlations between LAM and cancer, but LAM correlated negatively with FVC and PEF, and a trend in the same direction was observed for FEV1. 22 shared genetic variants were uncovered between LAM and pulmonary function, while seven shared variants were identified between LAM and cancer. The LAM-pulmonary function shared genetics identified four pleiotropic genes previously recognised in LAM single-cell transcriptomes: ADAM12, BNC2, NR2F2 and SP5. We had previously associated NR2F2 variants with LAM, and we identified its functional partner NR3C1 as another pleotropic factor. NR3C1 expression was confirmed in LAM lung lesions. Another candidate pleiotropic factor, CNTN2, was found more abundant in plasma of LAM patients than that of healthy women. Conclusions This study suggests the existence of a common genetic aetiology between LAM and pulmonary function., Asociacion Espanola de LAM, LAM Foundation Seed Grant 2019, Instituto de Salud Carlos III PI18/01029 PI21/01306 ICI19/00047, European Regional Development Fund (ERDF), "A way to build Europe", Ministry of Economy and Competitivity SAF2017-88457-R, Generalitat de Catalunya, General Electric SGR 2017-449 2017-529, PERIS PFI-Salut SLT017-20-000076, CERCA Program, VEIS project 001-P-001647, ERDF, "A way to build Europe" ERDF Operational Programme of Catalonia 2014-2020

Background: Acute exacerbation of idiopathic pulmonary fibrosis (AE-IPF) is a severe complication associated with a high mortality. However, evidence and guidance on management is sparse. The aim of this international survey was to assess differences in prevention, diagnostic and treatment strategies for AE-IPF in specialised and non-specialised ILD centres worldwide.Material and Methods: Pulmonologists working in specialised and non-specialised ILD centres were invited to participate in a survey designed by an international expert panel. Responses were evaluated in respect to the physicians' institutions.Results: Three hundred and two (65%) of the respondents worked in a specialised ILD centre, 134 (29%) in a non-specialised pulmonology centre. Similarities were frequent with regards to diagnostic methods including radiology and screening for infection, treatment with corticosteroids, use of high-flow oxygen and non-invasive ventilation in critical ill patients and palliative strategies. However, differences were significant in terms of the use of KL-6 and pathogen testing in urine, treatments with cyclosporine and recombinant thrombomodulin, extracorporeal membrane oxygenation in critical ill patients as well as antacid medication and anaesthesia measures as preventive methods.Conclusion: Despite the absence of recommendations, approaches to the prevention, diagnosis and treatment of AE-IPF are comparable in specialised and non-specialised ILD centres, yet certain differences in the managements of AE-IPF exist. Clinical trials and guidelines are needed to improve patient care and prognosis in AE-IPF.

Introduction: Idiopathic pulmonary fibrosis (IPF) prognosis is heterogeneous despite antifibrotic treatment. Cluster analysis has proven to be a useful tool in identifying interstitial lung disease phenotypes, which has yet to be performed in IPF. The aim of this study is to identify phenotypes of IPF with different prognoses and requirements. Methods: Observational retrospective study including 136 IPF patients receiving antifibrotic treatment between 2012 - 2018. Six patients were excluded due to follow-up in other centers. Cluster analysis of 30 variables was performed using approximate singular value-based tensor decomposition method and comparative statistical analysis. Results: The cluster analysis identified 3 different groups of patients according to disease behavior and clinical features, including mortality, lung transplant and progression-free survival time after 3-year follow-up. Cluster 1 (n=60) was significantly associated (p=0.02) with higher mortality. Diagnostic delay was the most relevant characteristic of this cluster, as 48% of patients had ≥ 2 years from first respiratory symptoms to antifibrotic treatment initiation. Cluster 2 (n=22) had the longest progression-free survival time and was correlated to subclinical patients evaluated in the context of incidental findings or familial screening. Cluster 3 (n=48) showed the highest percentage of disease progression without cluster 1 mortality, with metabolic syndrome and cardiovascular comorbidities as the main characteristics. Conclusion: This cluster analysis of IPF patients suggests that diagnostic and treatment delay are the most significant factors associated with mortality, while IPF progression was more related to metabolic syndrome and cardiovascular comorbidities.

Introduction: Telemedicine is proving to be a useful tool in the telemonitoring and telerehabilitation of respiratory patients. The use of telemedicine has been proposed by the main medical societies because of the limited resources and the healthcare workers9 infection risk in the COVID-19 pandemic. Aim and methods: This pilot program aims to evaluate the feasibility of the COVID-19 telerehabilitation program from the hospital to the home with clinical, functional, and patient satisfaction outcomes. Rehabilitation was initiated in the hospital by a physiotherapist and complemented by the "Estoi" app, which was continued at home with telemonitoring and messaging with the medical team. Patients9 habitual use of smartphones was not queried for inclusion. Ethics committee (ref.PR168/20). Results: 16 patients were consecutively enrolled, 47% women with a mean age of 63 years old. 81% of patients used the app for rehabilitation, 88% referred that the telemedicine program incentivized them to do more physical therapy, and 63% would choose telerehabilitation instead of center-based rehabilitation for new rehabilitation programs. 8,4 and 8,9/10 were the satisfaction with the mobile application and with the telerehabilitation program respectively. Conclusion: Beginning telerehabilitation in the hospital could increase the efficacy and efficiency of physical therapy, which is safe for patients and healthcare workers. Following at home, this telerehabilitation program seems to encourage and empower patients who have reported high satisfaction. Further randomized studies with larger numbers of patients and multicenter studies are required to evaluate these results.

BACKGROUND: Patients with coronavirus disease 2019 (COVID-19) can develop severe bilateral pneumonia leading to respiratory failure. We aimed to study the potential role of lung ultrasound score (LUS) in subjects with COVID-19. METHODS: We conducted an observational, prospective pilot study, including consecutive subjects admitted to an intermediate care unit due to COVID-19 pneumonia. LUS is a 12-zone examination method for lung parenchyma assessment. LUS was performed with a portable convex transducer, scores from 0 to 36 points. Clinical and demographic data were collected at LUS evaluation. Survival analysis was performed using a composite outcome including ICU admission or death. Subjects were followed for 30 d from LUS assessment. RESULTS: Of 36 subjects included, 69.4% were male, and mean age was 60.19 ± 12.75 y. A cutoff LUS ≥ 24 points showed 100% sensitivity, 69.2% specificity, and an area under the receiver operating characteristic curve of 0.85 for predicting worse prognosis. The composite outcome was present in 10 subjects (55.6%) with LUS ≥ 24 points, but not in the group with lower LUS scores (P < .001). Subjects with LUS ≥ 24 points had a higher risk of ICU admission or death (hazard ratio 9.97 [95% CI 2.75–36.14], P < .001). Significant correlations were observed between LUS and [Formula: see text] , serum D-dimer, C-reactive protein, lactate dehydrogenase, and lymphocyte count. CONCLUSIONS: LUS ≥ 24 points can help identify patients with COVID-19 who are likely to require ICU admission or to die during follow-up. LUS also correlates significantly with clinical and laboratory markers of COVID-19 severity.

Patients with connective tissues disease (CTD) are often on immunomodulatory agents before lung transplantation (LTx). Till now, there's no consensus on the safety of using these agents perioperative and post-transplant. The International Society for Heart and Lung Transplantation-supported consensus document on LTx in patients with CTD addresses the risk and contraindications of perioperative and post-transplant management of the biologic disease-modifying antirheumatic drugs (bDMARD), kinase inhibitor DMARD, and biologic agents used for LTx candidates with underlying CTD, and the recommendations and management of non-gastrointestinal extrapulmonary manifestations, and esophageal disorders by medical and surgical approaches for CTD transplant recipients.

Introduction: Many severe COVID-19 patients require respiratory support and monitoring. An intermediate respiratory care unit (IMCU) may be a valuable element for optimizing patient care and limited health-care resources management. We aim to assess the clinical outcomes of severe COVID-19 patients admitted to an IMCU.Methods: Observational, retrospective study including patients admitted to the IMCU due to COVID-19 pneumonia during the months of March and April 2020. Patients were stratified based on their requirement of transfer to the intensive care unit (ICU) and on survival status at the end of follow-up. A multivariable Cox proportional hazards method was used to assess risk factors associated with mortality.Results: A total of 253 patients were included. Of them, 68% were male and median age was 65 years (IQR 18 years). Ninety-two patients (36.4%) required ICU transfer. Patients transferred to the ICU had a higher mortality rate (44.6 vs. 24.2%; p < 0.001). Multivariable proportional hazards model showed that age ≥65 years (HR 4.14; 95%CI 2.31–7.42; p < 0.001); chronic respiratory conditions (HR 2.34; 95%CI 1.38–3.99; p = 0.002) and chronic kidney disease (HR 2.96; 95%CI 1.61–5.43; p < 0.001) were independently associated with mortality. High-dose systemic corticosteroids followed by progressive dose tapering showed a lower risk of death (HR 0.15; 95%CI 0.06–0.40; p < 0.001).Conclusions: IMCU may be a useful tool for the multidisciplinary management of severe COVID-19 patients requiring respiratory support and non-invasive monitoring, therefore reducing ICU burden. Older age and chronic respiratory or renal conditions are associated with worse clinical outcomes, while treatment with systemic corticosteroids may have a protective effect on mortality.

Resumen La aproximacion terapeutica en fibrosis pulmonar idiopatica ha cambiado sustancialmente en los ultimos 5 anos. Las guias nacionales e internacionales para el tratamiento farmacologico recomiendan el uso de 2 farmacos antifibroticos, nintedanib y pirfenidona, con un nivel elevado de evidencia cientifica a favor del beneficio de ambos farmacos, no solo porque enlentecen la progresion de la enfermedad sino tambien porque reducen el riesgo de muerte anual. Actualmente, dentro del manejo terapeutico de estos pacientes, se pone en valor tanto el uso de farmacos que actuan sobre mecanismos patogenicos de la enfermedad como el efecto positivo de mejorar la calidad de vida mediante un apoyo integral multidisciplinar que incluye nutricion, actividad fisica, educacion, apoyo emocional y paliacion de sintomas. Todo ello buscando que el paciente este lo mejor posible el mayor tiempo posible desde el diagnostico. Pero el objetivo de las nuevas terapias antifibroticas combinadas que en estos momentos se evaluan en ensayos clinicos persigue aumentar el beneficio terapeutico o parar completamente la progresion de la enfermedad mediante el potencial efecto sinergico antifibrotico, al actuar sobre diferentes vias patogenicas a la vez. Finalmente, se investiga que marcadores podrian ser utiles para identificar pacientes que se puedan beneficiar mas de unos farmacos antifibroticos u otros, lo que permitiria optimizar recursos e iniciar los primeros pasos hacia una medicina de precision en fibrosis pulmonar. A continuacion, se revisan los principales puntos de mejora en el tratamiento farmacologico de la fibrosis pulmonar idiopatica futuribles a corto, medio y largo plazo.

Background Angiotensin peptides have been implicated in idiopathic pulmonary fibrosis (IPF) pathogenesis. Angiotensin modulators are used to treat arterial hypertension, a frequent comorbidity of IPF. This post hoc analysis evaluated associations of antihypertensive treatments with disease-related outcomes in IPF. Methods All patients randomized to placebo (n = 624) in the CAPACITY and ASCEND studies were categorized by antihypertensive treatment at baseline. Outcomes of disease progression (first occurrence of ≥ 10% absolute decline in % predicted FVC, ≥ 50-m decline in 6-min walk distance, or death) and all-cause mortality were assessed over 52 weeks. Results At baseline, 111 and 121 patients were receiving an angiotensin-converting enzyme inhibitor (ACEi) or an angiotensin II receptor blocker (ARB), respectively; 392 were receiving neither. In multivariable analyses adjusted for differences in baseline characteristics compared with the non-ACEi/ARB group, ACEi treatment (hazard ratio [HR], 0.6 [95% CI, 0.4-0.9]; P = .026), but not ARB (HR, 0.9 [95% CI, 0.6-1.2]; P = .413), was associated with slower disease progression. Furthermore, the increase in all-cause mortality associated with cardiovascular disease was not observed in the ACEi group (HR, 1.1 [95% CI, 0.5-2.9]; P = .782), which presented a similar percentage of IPF-related mortality as the non-ACEi/ARB group (3.6% vs 3.6%). In contrast, patients in the ARB group had greater risk of all-cause mortality (HR, 2.5 [95% CI, 1.2-5.2]). These observations were validated in a pooled analysis that included patients from the INSPIRE trial. Conclusions Prospective clinical trials are needed to evaluate whether angiotensin modulators may be beneficial to clinical outcomes in IPF. Trial Registry ClinicalTrials.gov ; Nos.: NCT01366209 , NCT00287716 , NCT00287729 , NCT00075998 ; URL: www.clinicaltrials.gov ).

Hypersensitivity pneumonitis (HP) is a disease triggered by different inducers in susceptible individuals. The inhaled inducing particles can be of both organic and less frequently inorganic origin...

A study of the impact of the diffuse scattering phenomenon when calculating radio wave multiple-plateau diffraction losses at millimeter-wave (mm-wave) frequencies is hereby presented. In this sense, measurements of the total attenuation caused by a series of either rough or polished brick blocks are properly compared with the theoretical multiple-diffraction losses of an array of ideally smooth brick blocks predicted by a hybrid uniform theory of diffraction-physical optics (UTD-PO) formulation. The results show the influence of diffuse scattering phenomena when it comes to calculating radio wave multiple-plateau diffraction attenuation and can be applied to the obtaining of more realistic mm-wave propagation models when multiple diffraction over rectangular obstacles with irregular (rough) surfaces has to be considered.

Introduction: Treatment of patients with adult congenital heart disease (ACHD) with advanced therapies including heart transplant (HT) is often delayed due to paucity of objective prognostic markers for the severity of heart failure (HF). While the utility of Cardiopulmonary Exercise Testing (CPET) in non-ACHD patients has been well-defined as it relates to prognosis, CPET for this purpose in ACHD is still under investigation. Methods: We performed a retrospective cohort study of 20 consecutive patients with ACHD who underwent HT between March 2010 and February 2016. Only 12 of 20 patients underwent CPET prior to transplantation. Demographics, standard measures of CPET interpretation, and 30-day and 1-year post transplantation outcomes were collected. Results: Patient Characteristics. Twenty patients with ACHD were transplanted at a median of 40 years of age (range: 23-57 years). Of the 12 patients who underwent CPET, 4 had undergone Fontan procedures, 4 had tetralogy of Fallot, 3 had d-transposition of the great arteries, and 1 had Ebstein anomaly. Thirty-day and one-year survival was 100%. All tests included in the analysis had a peak respiratory quotient _1.0. The median peak oxygen consumption per unit time (_VO2) for all diagnoses was 18.2 mL/kg/min (46% predicted), ranging from 12.2 to 22.6. Conclusion: There is a paucity of data to support best practices for patients with ACHD requiring transplantation. While it cannot be proven based on available data, it could be inferred that outcomes would have been worse or perhaps life sustaining options unavailable if providers delayed referral because of the lack of attainment of CPET-specific thresholds.

PURPOSE The incidence and management of sternal wound complications in patients undergoing orthotopic heart transplantation (OHT) is not well studied. We report outcomes in heart transplant patients who developed sternal infections requiring reoperations. METHODS From 2004 to 2013, 437 patients underwent OHT at a single institution. In a retrospective review, patients who developed sternal infections (Infection group, n = 27) were compared with those without (Control group, n = 410). RESULTS Sternal infection rate was 6.2% (n = 27). Demographics were similar (Table 1). Infection group had higher rates of COPD 25% vs 13%, P = 0.03, and previous cardiac surgery via median sternotomy 28% vs 15%, P = 0.03. Infection group had a greater incidence of prolonged ventilation, 44% vs 31%, P = 0.2, renal failure 56% vs 24%, P = 0.001, dialysis requirement 30% vs 10%, P = 0.006, permanent stroke 11% vs 2%, P = 0.02, perioperative myocardial infarction 4% vs 0.2%, P = 0.09. The infection group had a longer ICU stay (524 + 410 vs 187 + 355 hours, P = 0.001) and hospitalization (59 + 28 vs 0.29 + 43 days, P = 0.001). In-hospital/30-day mortality was 30% vs 19%, P = 0.2. The mean time for sternal reoperation at 44 + 50 days. Deep wound infection (41%) and sternal dehiscence (22%) were common presentations. Causative organisms were Enterobacter (22%), Klebsiella (15%), and Pseudomonas (15%). Vancomycin (44%), 4th generation cephalosporin (37%), and fluoroquinolones (30%) were the most commonly used antibiotics. Surgical treatment included sternal debridement with pectoralis muscle flap (52%), primary closure (18%), and omental flap (11%). CONCLUSION Sternal wound infections impart a significant burden on patients with OHT. Causative organisms are predominantly virulent gram-negative bacteria. Therefore, a high index of suspicion must be maintained for early detection and treatment.

Introduction: Several studies have shown that diabetes mellitus (DM) is a risk factor for in hospital mortality due to COVID-19. The aim of our study was to analyze if prior clinical characteristics among DM patients affected the risk of in hospital mortality compared with nondiabetic patients hospitalized with COVID-19. Material and Methods: The study was based on a retrospective, observational design and was conducted at two hospitals located in Albacete (Spain). All patients hospitalized with COVID-19 since the start of the pandemic until 7 July 2020 were included. Results: During the study period, 1468 patients were hospitalized due to COVID-19; 402 of them had DM prior to hospitalization. DM was associated with higher in hospital mortality (28.6% versus 17.5%) in the univariate analysis. But, in a multivariate logistic regression analysis, after adjusting for age, presence of hypertension, smoking, presence of COPD, ACEI, ARB, antiplatelet, or anticoagulant therapy, DM was not a significant risk factor for death. Patients with DM2 receiving insulin treatment had a higher risk of in hospital death than nondiabetic patients (OR 1.78 [1.13-2.81], p=0.0126) after age was considered. The age-associated relative risk was 1.06 (1.05-1.07, p Conclusion: This study confirms that pre-admission need for insulin therapy before hospitalization in patients with DM2 are associated with higher in hospital mortality due to COVID-19 in a large sample of DM inpatients with COVID-19. Disclosure P. J. Pinés-corrales: None. M. Molina: None. L. García-blasco: None. R. Quilez toboso: None. M. Alcaraz: None. L. Vicente gutiérrez: None. G. Paterna mellinas: None. J. Calbo mayo: None. J. Alfaro-martínez: None.

Introduction Idiopathic pulmonary fibrosis (IPF) prognosis is heterogeneous despite antifibrotic treatment. Cluster analysis has proven to be a useful tool in identifying interstitial lung disease phenotypes, which has yet to be performed in IPF. The aim of this study is to identify phenotypes of IPF with different prognoses and requirements. Methods Observational retrospective study including 136 IPF patients receiving antifibrotic treatment between 2012 and 2018. Six patients were excluded due to follow-up in other centres. Cluster analysis of 30 variables was performed using approximate singular value-based tensor decomposition method and comparative statistical analysis. Results The cluster analysis identified three different groups of patients according to disease behaviour and clinical features, including mortality, lung transplant and progression-free survival time after 3-year follow-up. Cluster 1 (n=60) was significantly associated (p=0.02) with higher mortality. Diagnostic delay was the most relevant characteristic of this cluster, as 48% of patients had ≥2 years from first respiratory symptoms to antifibrotic treatment initiation. Cluster 2 (n=22) had the longest progression-free survival time and was correlated to subclinical patients evaluated in the context of incidental findings or familial screening. Cluster 3 (n=48) showed the highest percentage of disease progression without cluster 1 mortality, with metabolic syndrome and cardiovascular comorbidities as the main characteristics. Conclusion This cluster analysis of IPF patients suggests that diagnostic and treatment delay are the most significant factors associated with mortality, while IPF progression was more related to metabolic syndrome and cardiovascular comorbidities., Diagnostic delay and cardiovascular comorbidities impact IPF outcomes https://bit.ly/3lk2Z5y

Background COVID-19 continues to inflict significant morbidity and mortality, particularly on patients with preexisting health conditions. The clinical course, outcomes, and significance of immunosuppression regimen in heart transplant recipients with COVID-19 remains unclear. Methods We included the first 99 heart transplant recipients at participating centers with COVID-19 and followed patients until resolution. We collected baseline information, symptoms, laboratory studies, vital signs, and outcomes for included patients. The association of immunosuppression regimens at baseline with severe disease were compared using logistic regression , adjusting for age and time since transplant. Results The median age was 60 years, 25% were female, and 44% were white. The median time post-transplant to infection was 5.6 years. Overall, 15% died, 64% required hospital admission, and 7% remained asymptomatic. During the course of illness, only 57% of patients had a fever, and gastrointestinal symptoms were common. Tachypnea , oxygen requirement, elevated creatinine and inflammatory markers were predictive of severe course. Age ≥ 60 was associated with higher risk of death and the use of the combination of calcineurin inhibitor , antimetabolite , and prednisone was associated with more severe disease compared to the combination of calcineurin inhibitor and antimetabolite alone (adjusted OR = 7.3, 95% CI 1.8-36.2). Among hospitalized patients, 30% were treated for secondary infection, acute kidney injury was common and 17% required new renal replacement therapy . Conclusions We present the largest study to date of heart transplant patients with COVID-19 showing common atypical presentations and a high case fatality rate of 24% among hospitalized patients and 16% among symptomatic patients.

Rheumatoid arthritis (RA) is a systemic autoimmune disease whose main extra-articular organ affected is the lung, sometimes in the form of diffuse interstitial lung disease (ILD) and conditions the prognosis. A multicenter, observational, descriptive and cross-sectional study of consecutive patients diagnosed with RA-ILD. Demographic, analytical, respiratory functional and evolution characteristics were analyzed to evaluate the predictors of progression and mortality. 106 patients were included. The multivariate analysis showed that the diagnostic delay was an independent predictor of mortality (HR 1.11, CI 1.01–1.23, p = 0.035). Also, age (HR 1.33, 95% CI 1.09–1.62, p = 0.0045), DLCO (%) (HR 0.85, 95% CI 0.73–0.98, p = 0.0246), and final SatO2 (%) in the 6MWT (HR 0.62, 95% CI 0.39–0.99, p = 0.0465) were independent predictor variables of mortality, as well as GAP index (HR 4.65, 95% CI 1.59–13.54, p = 0.0051) and CPI index (HR 1.12, 95% CI 1.03–1.22, p = 0.0092). The withdrawal of MTX or LFN after ILD diagnosis was associated with disease progression in the COX analysis (HR 2.18, 95% CI 1.14–4.18, p = 0.019). This is the first study that highlights the diagnostic delay in RA-ILD is associated with an increased mortality just like happens in IPF.

Patient-reported outcome measures (PROMs), tools to assess patient self-report of health status, are now increasingly used in research, care and policymaking. While there are two well-developed disease-specific PROMs for interstitial lung diseases (ILD) and idiopathic pulmonary fibrosis (IPF), many unmet and urgent needs remain. In December 2019, 64 international ILD experts convened in Erice, Italy to deliberate on many topics, including PROMs in ILD. This review summarises the history of PROMs in ILD, shortcomings of the existing tools, challenges of development, validation and implementation of their use in clinical trials, and the discussion held during the meeting. Development of disease-specific PROMs for ILD including IPF with robust methodology and validation in concordance with guidance from regulatory authorities have increased user confidence in PROMs. Minimal clinically important difference for bidirectional changes may need to be developed. Cross-cultural validation and linguistic adaptations are necessary in addition to robust psychometric properties for effective PROM use in multinational clinical trials. PROM burden of use should be reduced through appropriate use of digital technologies and computerised adaptive testing. Active patient engagement in all stages from development, testing, choosing and implementation of PROMs can help improve probability of success and further growth.

Inhibition of mTOR is the standard of care for lymphangioleiomyomatosis (LAM). However, this therapy has variable tolerability and some patients show progressive decline of lung function despite treatment. LAM diagnosis and monitoring can also be challenging due to the heterogeneity of symptoms and insufficiency of non-invasive tests. Here, we propose monoamine-derived biomarkers that provide preclinical evidence for novel therapeutic approaches. The major histamine-derived metabolite methylimidazoleacetic acid (MIAA) is relatively more abundant in LAM plasma, and MIAA values are independent of VEGF-D. Higher levels of histamine are associated with poorer lung function and greater disease burden. Molecular and cellular analyses, and metabolic profiling confirmed active histamine signaling and metabolism. LAM tumorigenesis is reduced using approved drugs targeting monoamine oxidases A/B (clorgyline and rasagiline) or histamine H1 receptor (loratadine), and loratadine synergizes with rapamycin. Depletion of Maoa or Hrh1 expression, and administration of an L-histidine analog, or a low L-histidine diet, also reduce LAM tumorigenesis. These findings extend our knowledge of LAM biology and suggest possible ways of improving disease management., This research was supported by AELAM, The LAM Foundation (Seed Grant 2019), Instituto de Salud Carlos III grants PI15/00854, PI18/01029, and ICI19/00047 (co-funded by European Regional Development Fund (ERDF), a way to build Europe), Generalitat de Catalunya SGR grants 2014-364 and 2017-449, the CERCA Program, and ZonMW-TopZorg grant 842002003. C.L.M. acknowledges the financial support (PRA-2017-51 project) of the University of Pisa. A.U.K. is supported by Nottingham Trent University’s Independent Fellowship Scheme.

Background and objective The relationship between IPF development and environmental factors has not been completely elucidated. Analysing geographic regions of idiopathic pulmonary fibrosis (IPF) cases could help identify those areas with higher aggregation and investigate potential triggers. We hypothesize that cross-analysing location of IPF cases and areas of consistently high air pollution concentration could lead to recognition of environmental risk factors for IPF development. Methods This retrospective study analysed epidemiological and clinical data from 503 patients registered in the Observatory IPF.cat from January 2017 to June 2019. Incident and prevalent IPF cases from the Catalan region of Spain were graphed based on their postal address. We generated maps of the most relevant air pollutant PM2.5 from the last 10 years using data from the CALIOPE air quality forecast system and observational data. Results In 2018, the prevalence of IPF differed across provinces; from 8.1 cases per 100 000 habitants in Barcelona to 2.0 cases per 100 000 in Girona. The ratio of IPF was higher in some areas. Mapping PM2.5 levels illustrated that certain areas with more industry, traffic and shipping maintained markedly higher PM2.5 concentrations. Most of these locations correlated with higher aggregation of IPF cases. Compared with other risk factors, PM2.5 exposure was the most frequent. Conclusion In this retrospective study, prevalence of IPF is higher in areas of elevated PM2.5 concentration. Prospective studies with targeted pollution mapping need to be done in specific geographies to compile a broader profile of environmental factors involved in the development of pulmonary fibrosis.

BackgroundNon-invasive biomarkers for the assessment of disease severity in idiopathic pulmonary fibrosis (IPF) are urgently needed. Calprotectin belongs to the S-100 proteins produced by neutrophils, which likely contribute to IPF pathogenesis. Calprotectin is a well-established biomarker in inflammatory bowel diseases. In this cross-sectional study, we aimed to establish the potential role of calprotectin as a biomarker in IPF. Specifically, we hypothesised that patients with IPF have higher serum calprotectin levels compared with healthy controls, and that calprotectin levels are associated with disease severity.MethodsBlood samples were obtained from healthy volunteers (n=26) and from two independent IPF cohorts (derivation cohort n=26, validation cohort n=66). Serum calprotectin levels were measured with a commercial kit adapted for that purpose and compared between healthy controls and patients with IPF. Clinical parameters, including forced vital capacity, diffusing capacity for carbon monoxide (DLCO) and the Composite Physiologic Index (CPI), were correlated with calprotectin serum levels.ResultsThe IPF derivation cohort showed increased serum calprotectin levels compared with healthy controls (2.47±1.67 vs 0.97±0.53 µg/mL, pConclusionSerum calprotectin levels are significantly increased in patients with IPF compared with healthy controls and correlate with DLCO and CPI. Calprotectin might be a potential new biomarker for disease severity in IPF.

Background To assess differences in platelet inhibition during ticagrelor monotherapy (TIC) or dual therapy with ticagrelor and aspirin (TIC+ASP) in patients after percutaneous coronary intervention using a comprehensive panel of functional tests. Methods and Results In a single‐center parallel group, open label, randomized controlled trial, 110 participants were randomized to receive either TIC (n=55) or TIC+ASP (n=55) for 4 weeks. The primary outcome was the platelet aggregation response with 10 μmol/L thrombin receptor activation peptide‐6 (TRAP‐6). The secondary outcomes were platelet aggregation responses and binding of surface activation markers with a panel of other activators. The mean percentage aggregation for 10 μmol/L TRAP‐6 was similar for the TIC and TIC+ASP groups (mean difference+4.29; 95% CI, −0.87 to +9.46). Aggregation was higher in the TIC group compared with the TIC+ASP group with 1 μg/mL (+6.47; +2.04 to +10.90) and 0.5 μg/mL (+14.00; +7.63 to +20.39) collagen related peptide. Aggregation responses with 5 μmol/L TRAP‐6, 5 μmol/L or 2.5 μmol/L thromboxane A 2 receptor agonist and surface activation marker binding with 5 μmol/L TRAP‐6 or 0.5 μg/mL collagen related peptide were the same between the treatment groups. Conclusions Patients with PCI show similar levels of inhibition of most platelet activation pathways with TIC compared with dual therapy with TIC + ASP. However, the greater aggregation response with collagen related peptide during TIC indicates incomplete inhibition of glycoprotein VI (collagen) receptor‐mediated platelet activation. This difference in pharmacodynamic response to anti‐platelet medication may contribute to the lower bleeding rates observed with TIC compared with dual antiplatelet therapy in recent clinical trials. Registration Information URL: https://www.isrctn.com ; Unique Identifier ISRCTN84335288.

Rationale: The impact of coronavirus disease (COVID-19) on patients with interstitial lung disease (ILD) has not been established. Objectives: To assess outcomes in patients with ILD hospitalized for COVID-19 versus those without ILD in a contemporaneous age-, sex-, and comorbidity-matched population. Methods: An international multicenter audit of patients with a prior diagnosis of ILD admitted to the hospital with COVID-19 between March 1 and May 1, 2020, was undertaken and compared with patients without ILD, obtained from the ISARIC4C (International Severe Acute Respiratory and Emerging Infection Consortium Coronavirus Clinical Characterisation Consortium) cohort, admitted with COVID-19 over the same period. The primary outcome was survival. Secondary analysis distinguished idiopathic pulmonary fibrosis from non–idiopathic pulmonary fibrosis ILD and used lung function to determine the greatest risks of death. Measurements and Main Results: Data from 349 patients with ILD across Europe were included, of whom 161 were admitted to the hospital with laboratory or clinical evidence of COVID-19 and eligible for propensity score matching. Overall mortality was 49% (79/161) in patients with ILD with COVID-19. After matching, patients with ILD with COVID-19 had significantly poorer survival (hazard ratio [HR], 1.60; confidence interval, 1.17–2.18; P = 0.003) than age-, sex-, and comorbidity-matched controls without ILD. Patients with an FVC of Conclusions: Patients with ILD are at increased risk of death from COVID-19, particularly those with poor lung function and obesity. Stringent precautions should be taken to avoid COVID-19 in patients with ILD.

Background: Many severe COVID-19 patients require respiratory support and monitoring. An intermediate respiratory care unit (IMCU) may be a valuable element for optimizing patient care and limited health-care resources management. We aim to assess the impact of an IMCU in the management of severe COVID-19.Methods: Observational, retrospective study including patients admitted to the IMCU due to COVID-19 pneumonia during the months of March and April 2020. Patients were stratified based on their requirement of transfer to the intensive care unit (ICU) and on survival status at the end of follow-up. A multivariable Cox proportional hazards method was used to assess risk factors associated with mortality.Results: A total of 253 patients were included. Of them, 68% were male and median age was 65 years (IQR 18 years). Ninety-two patients (36.4%) required ICU transfer. Patients transferred to the ICU had a higher mortality rate (44.6% Vs 24.2%; pConclusions: IMCU allow to safely and effectively manage severe COVID-19 patients requiring respiratory support and non-invasive monitoring, therefore reducing ICU burden. Older age and chronic respiratory or renal conditions are associated with worse clinical outcomes, while treatment with systemic corticosteroids may have a protective effect on mortality.

BackgroundInterstitial lung abnormalities (ILA) occur in around 10% of subjects over 60 years, and are associated with a higher rate of all-cause mortality. The pathogenic mechanisms are unclear, and the putative contribution of alterations in the immune response has not been explored. Normal ageing is associated with immune deficiencies, including Naïve T-cell decrease and greater expression of the proliferative-limiting, co-inhibitory receptor killer-cell lectin-like receptor G1 (KLRG1).ObjectiveTo evaluate the frequency and activation state of different T-cell subpopulations in ILA subjects.MethodsPeripheral blood mononuclear cells were obtained from 15 individuals with ILA, 21 age-matched controls and 28 healthy young subjects. T-cells phenotype was characterised by flow cytometry, and proliferation and activation by stimulation with anti-CD3/anti-CD28 or phorbol myristate acetate/ionomycin; KLRG1 isoforms were evaluated by western blot and cytokines were quantified by ELISA and Multiplex.ResultsA significant increase of Naïve CD4+T cells together with a decrease of central and effector memory CD4+T cells was observed in ILA compared with age-matched controls. CD4+T cells from ILA subjects exhibited greater basal proliferation, which raised after anti-CD3/anti-CD28 stimulation. Additionally, a significant increase in the levels of interleukin-6 and interferon gamma was observed in isolated CD4+T cells and plasma of ILA subjects. They also displayed fewer KLRG1+/CD4+T cells with an increase of circulating E-cadherin, the ligand of KLRG1+. No changes were observed with CD8+T cell subsets.ConclusionCD4+T cells from ILA subjects are highly proliferative and show an excessive functional activity, likely related to the loss of KLRG1 expression, which may contribute to an inflammatory state and the development of ILA.

While several mm-wave and sub-THz frequency bands are being proposed for next generation wireless systems to meet the increasing traffic demand, the electromagnetic properties of many common use materials at those frequencies still need to be determined. The evaluation of such properties is important for the design and deployment of future wireless networks. Recently, a simple method based on Fabry-Pérot resonance has been proposed to address the need of easy material characterization at mm-wave frequencies. In this study, the method has been improved and applied to the characterization of several materials at mm-wave and sub-THz frequencies, in order to assess its reliability, usability and accuracy in practical cases. The method is shown to achieve a good accuracy level despite the very simple measurement setup and the great flexibility. However, some application limitations are highlighted and discussed in the paper

Background Currently there is major lack of agreement on the diagnostic and therapeutic management of patients with idiopathic pulmonary fibrosis (IPF) and lung cancer. Our aim was to identify variations in diagnostic and management strategies across different institutions and provide rationale for a consensus statement on this issue. Methods This was a joint-survey by European Respiratory Society (ERS) Assemblies 8, 11 and 12. The survey consisted of 25 questions. Results Four hundred and ninety-four (n=494) physicians from 68 different countries and five continents responded to the survey. Ninety-four per cent of participants were pulmonologists, 1.8% thoracic surgeons and 1.9% oncologists; 97.7% were involved in multidisciplinary team approaches on diagnosis and management. Regular low-dose high-resolution computed tomography (HRCT) scan was used by 49.5% of the respondents to screen for lung cancer in IPF. Positron emission tomography (PET) scan and endobronchial ultrasound (EBUS) is performed by 60% and 88% to diagnose nodular lesions with mediastinal lymphadenopathy in patients with advanced and mild IPF, respectively. Eighty-three per cent of respondents continue anti-fibrotics following lung cancer diagnosis; safety precautions during surgical interventions including low tidal volume are applied by 67%. Stereotactic radiotherapy is used to treat patients with advanced IPF (diffusing capacity of the lung for carbon monoxide (DLCO), The diagnosis and management of IPF-LC is heterogeneous with most survey respondents calling for a consensus statement https://bit.ly/2Smie0o

Idiopathic pulmonary fibrotic (IPF) patients present progressive worsening in quality of life (QoL) despite of anti-fibrotic medication. QoL includes different domains that require specific therapeutic approach. With the use of specific QoL questionnaires the impact of any integral patient support program (iPSP) may be measured. Methods: A home iPSP, which includes a trained nurse and physiotherapist that perform disease and treatment education, monitoring, and physiotherapy, is being conducted for 12 months, including the first month 4 visits, and then, 1 visit per month. Recruitment period was 6 months and all patients included from 22 sites received nintedanib (initiated at least one month later and not for more than 2 years). QoL (K-BILD) is measured at time 0,6 and 12 months. Results: In line with sample size calculation, a total of 90 patients have been recruited. Currently, 85 have completed the 6th-month follow-up visit. 83 could be analyzed (mean age 69.8, 75% males, 71% ex-smokers). 74% presented some comorbidity, cardiovascular disease was the most prevalent. Initial (V0) K-BILD mean value was 78. A significant improvement of QoL was observed at visit 6 (p=0.029). The best result was observed in the activity and dyspnea domain (p=0.002), followed by psychological (p=0.068) and symptoms domain (p=0.939). Improvement in these values was greater in those male patients older than 65 y. and in those who started the iPSP between 1 and 6 months after initiating nintedanib. Conclusion: iPSP conducted by trained nurses and physiotherapists, in treated IPF patients, improves their QoL especially in activity and psychological scores. The multidisciplinary managment is beneficial in these patients. Supported by Boehringer Ing

The incidence of gastrointestinal adverse events (AEs) associated with pirfenidone varies in IPF patients. We hypothesized that the type of dietary fatty acids (FA) affects digestibility and gastric motility, with a decrease in saturated-SFA or an increase in monounsaturated-MUFA. Objective: To compare the incidence of gastrointestinal AEs in patients with IPF initiating pirfenidone therapy, according to diet; MUFA vs SFA. Methods: 90 patients agreeing to maintain the same diet for 4 months were included. Allocation to SFA or MUFA-diet was determined by the Food Frequency Questionnaire (FFQ) assessed by an independent Central Committee. 7 centres, 4 in northern and 3 in southern Europe, have participated. Patient diet and AEs were recorded over 16 weeks; FVC and DLCO were measured at V1 and V5. Results: Interim results are based on 64 patients (38 MUFA and 26 SFA arm). Most patients were male (70%), mean age 71 (8.08), mean BMI 29 (without differences between groups). Baseline lung functions: FVC 81.5%, DLCO 55.4% for the MUFA group and FVC 75%, DLCO 44% for the SFA group. Except for the Netherlands, SFA diet was prevalent in the northern centres. Gastrointestinal AEs were reported in 31% of the SFA group and 17% of the MUFA group. Full dose of pirfenidone was taken by 73.1% of SFA and 94.7% of MUFA patients. Skin rash-photosensitivity did not differ between groups (1.2% and 1.5%, respectively). Medication withdraw was reported in 2 cases of SFA group. Conclusions: SFAdiet is associated with more gastrointestinal AEs during pirfenidone treatment in patients with IPF compared to MUFAdiet. Diet may be explored for improving medication adherence. Supported by Roche

Introduction: LAM is a rare and lethal disease characterized by progressive cystic lung destruction. Inhibition of mTOR with rapamycin is the current standard of care, which can slow-down disease. Plasma major histamine metabolite (Methylimidazoleacetic acid [MIAA]) is increased in LAM. Loratadine is a histamine receptor antagonist (HR1), which inhibits LAM cell proliferation. Therefore, a novel phase-II clinical trial for assessing safety and potential benefits of loratadine in LAM has been initiated. Methods:LORALAM clinical trial, phase-II, double-blind, randomized, placebo controlled, parallel-group, multicentre study initiates recruitment in July 2020. Enrollment plan includes 62 subjects with LAM on treatment with rapamycin ≥3 months, randomized 1:1 to add oral loratadine 10mg/day or placebo, once daily, for 52 weeks. Recruitment will end in June 2021. The primary endpoints are 1) to assess the safety profile of loratadine associated with rapamycin, 2) lung function decline after 52 weeks of treatment. The secondary endpoints are a) quality of life and progression free-survival time, b) changes in the established LAM serum biomarker VEGFD, c) the utility of MIAA for monitoring disease progression and biological treatment effect. Ethics and Dissemination: The study will be carried out in accordance with Good Clinical Practice guidelines, Declaration of Helsinki principles, and each ethical committee. This clinical trial contemplates the possibility of increasing the number of centers and including patients from patients support groups (LAM foundation, AELAM). Clinicaltrials.gov registered

Telomere dysfunction has been related to post-transplant morbi-mortality.Study aim´s: post-transplant survival and morbidity evaluation in fibrotic ILD with and without telomeropathy Methods: Prospective observational study of fibrotic ILD cases from Bellvitge Hospital that underwent lung transplant(LT) in V.Hebron Hospital. Telomere length was measured from lymphocytes DNA, through quantitative PCR. Whole-exome sequencing for telomere maintenance genes was performed in case of telomere shortening(TS) Results: 20 patients(IPF 40%, FPF 40%, hypersensitivity pneumonitis 5%, CPFE 5%, descamative interstitial pneumonia 5%, CTD-ILD 5%). Twelve(60%) patients had TS and mutations in RTEL1, TERT, DKC1,PARN. A similar pre-LT functional status was reported regardless of TS. LT morbidity: patients with TS had a greater need for extracorporeal circulation, longer ICU admission period and higher number of hospital admissions(p=0.033) with 2.9 times higher risk for readmission[(CI 1.11-7.59)(p=0.029)]; being the only ones with hematological complications during the first month. At long term, no differences in morbidity were found regardless of TS. Survival: 17/20(85%) patients,[10/12(83.3%) with TS and 7/8(87.5%) without]. Quality of life: overall mean FVC post-LT increase of 0.55 liters(p=0.0003)[CI95%: 0.21;0.89]. Oxygen withdrawal in 16/17(94.1%) cases with independence for daily life activities[15/17(88.2%)] and active life[11/17(64.7%)] regardless of TS(p=1.000) Conclusions: Post-LT morbidity differs accoding to elapsed time from LT and genetic susceptibility.However, a positive impact in post-LT survival and quality of life was shown for fibrotic ILD regardless of telomeropathy