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Interstitial lung disease is a well-recognised manifestation and a major cause of morbidity and mortality in patients with connective tissue diseases. Interstitial lung disease may arise in the context of an established connective tissue disease or be the initial manifestation of an otherwise occult autoimmune disorder. Early detection and characterisation are paramount for adequate patient management and require a multidisciplinary approach, in which imaging plays a vital role. Computed tomography is currently the imaging method of choice; however, other imaging techniques have recently been investigated, namely ultrasound, magnetic resonance imaging, and positron-emission tomography, with promising results. The aim of this review is to describe the imaging findings of connective tissue disease-related interstitial lung disease and explain the role of each imaging technique in diagnosis and disease characterisation. info:eu-repo/semantics/publishedVersion

We present a case of a 33-year-old male who worked as a plumber and a locksmith. The patient presented with diffuse myalgia and asthenia, skin sclerosis and puffy fingers, Raynaud's phenomenon, exertional dyspnea and erectile dysfunction. The presence of specific autoantibodies enabled the diagnosis of systemic sclerosis. Chest-computed tomography revealed upper lobe consolidation. After extensive evaluation, the multidisciplinary interstitial lung disease team concluded that the patient also had advanced silicosis. After a year, there was significant clinical, radiologic, and functional deterioration of the lung disease. The patient was referred for lung transplant. Silica inhalation is the cause of silicosis but is also implicated in the development of systemic sclerosis (Erasmus syndrome). Although they share a common risk factor, it is rare to find both diseases co-existing. We present this case of a young patient where both diseases presented aggressively in order to raise awareness to this association. Apresentamos o caso de um homem de 33 anos que trabalhava como canalizador e serralheiro. Apresentava mialgias e astenia, esclerose cutânea e puffy fingers, fenómeno de Raynaud, dispneia de esforço e disfunção erétil. A presença de autoanticorpos específicos permitiu o diagnóstico de esclerose sistémica. A tomografia computadorizada de tórax revelou consolidações dos lobos superiores. Após extensa avaliação, o grupo multidisciplinar de doenças do interstício concluiu que o doente tinha também silicose avançada. Após um ano, houve agravamento clínico, radiológico e funcional significativo da doença pulmonar. O doente foi encaminhado para transplante pulmonar. A inalação de sílica é a causa da silicose, mas também está implicada no desenvolvimento da esclerose sistémica (síndrome de Erasmus). Embora tenham um fator de risco comum, é raro encontrar as duas doenças simultaneamente. Apresentamos o caso de um doente jovem em que ambas as doenças se apresentaram de forma agressiva para alertar sobre esta associação. info:eu-repo/semantics/publishedVersion

Background Although evidence has emerged indicating that patients with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) pneumonia present a high risk of venous thromboembolism (VTE), its real incidence and best diagnosis course remain unclear. In this study, we aimed to determine the incidence of pulmonary embolism in these patients and the role of D-dimer serum level as a predictive factor of a new VTE event. Methodology This was a single-center retrospective observational cohort study conducted in a tertiary hospital. All patients admitted to the infectious diseases ward with SARS-CoV-2 pneumonia with clinical or laboratory criteria for suspected VTE events were eligible for inclusion in the study. The t-test or Mann-Whitney U test was used to analyze the differences between the with-VTE group and the without-VTE group. Results Overall, VTE incidence was registered to be 30%. Chest computed tomography angiography data revealed thrombus mainly in segmental (five patients, 71%) and subsegmental pulmonary artery branches (four patients, 57%). No thrombus on major branches was documented. D-dimer serum levels (collected at hospital admission, 48 hours before the suspected VTE event date and at suspected VTE event date) were analyzed, and, despite a consistent tendency of higher values in the with-VTE group, no statistical difference was observed. Moreover, no statistical difference was observed between the two groups in mortality rates. Conclusions A clear higher risk of VTE events in SARS-CoV-2 pneumonia patients was not documented, and a link between the impact of VTE occurrence and a worse prognosis was not demonstrated. Therefore, we suggest that the use of D-dimer serum level should not be used as a predictor of VTE in SARS-CoV-2 pneumonia patients. info:eu-repo/semantics/publishedVersion

Ricardo LM Duarte,1,2 Flavio J Magalhães-da-Silveira,1 Tiago S Oliveira-e-Sá,3,4 Joana A Silva,5 Fernanda CQ Mello,2 David Gozal6 1Sleep - Laboratório de Estudo dos Distúrbios do Sono, Centro Médico BarraShopping, Rio de Janeiro, Brazil; 2Instituto de Doenças do Tórax, Universidade Federal do Rio de Janeiro, Rio de Janeiro, Brazil; 3Hospital de Santa Marta, Centro Hospitalar Lisboa Central, Lisbon, Portugal; 4NOVA Medical School, Faculdade de Ciências Médicas, Universidade Nova de Lisboa, Lisbon, Portugal; 5Clínica São Vicente, Rede D’Or, Rio de Janeiro, Brazil; 6Department of Child Health, University of Missouri School of Medicine, Columbia, MO, USACorrespondence: David GozalDepartment of Child Health, University of Missouri School of Medicine, Columbia, MO, USAEmail drdavidgozal@gmail.comBackground: Obstructive sleep apnea (OSA) is a very prevalent disorder. Here, we aimed to develop and validate a practical questionnaire with yes-or-no answers, and to compare its performance with other well-validated instruments: No-Apnea, STOP-Bang, and NoSAS.Methods: A cross-sectional study containing consecutively selected sleep-lab subjects underwent full polysomnography. A 4-item model, named GOAL questionnaire (gender, obesity, age, and loud snoring), was developed and subsequently validated, with item-scoring of 0– 4 points (≥ 2 points indicating high risk for OSA). Discrimination was assessed by area under the curve (AUC), while predictive parameters were calculated using contingency tables. OSA severity was classified based on conventionally accepted apnea/hypopnea index thresholds: ≥ 5.0/h (OSA≥ 5), ≥ 15.0/h (OSA≥ 15), and ≥ 30.0/h (OSA≥ 30).Results: Overall, 7377 adults were grouped into two large and independent cohorts: derivation (n = 3771) and validation (n = 3606). In the derivation cohort, screening of OSA≥ 5, OSA≥ 15, and OSA≥ 30 revealed that GOAL questionnaire achieved sensitivity ranging from 83.3% to 94.0% and specificity ranging from 62.4% to 38.5%. In the validation cohort, screening of OSA≥ 5, OSA≥ 15, and OSA≥ 30, corroborated validation steps with sensitivity ranging from 83.7% to 94.2% and specificity from 63.4% to 37.7%. In both cohorts, discriminatory ability of GOAL questionnaire for screening of OSA≥ 5, OSA≥ 15, and OSA≥ 30 was similar to No-Apnea, STOP-Bang or NoSAS.Conclusion: All four instruments had similar performance, leading to a possible greater practical implementation of the GOAL questionnaire, a simple instrument with only four parameters easily obtained during clinical evaluation.Keywords: obstructive sleep apnea, polysomnography, screening, questionnaire, diagnosis

A significant dysfunction of another organ is usually considered an absolute contraindication for lung transplantation, unless multiorgan transplantation is indicated and practical, as is the case of combined lung-kidney transplantation. Few cases of combined lung-kidney transplantation have been described in the literature; however, it is known that, in certain cases, it is the only way to offer an opportunity to selected patients with renal and lung dysfunction. The authors are not aware of any previously published case of a patient receiving both extracorporeal membrane oxygenation and continuous venovenous hemodiafiltration as a bridge for combined kidney-lung transplantation. The authors present the first case of combined lung-kidney transplantation performed in Portugal. info:eu-repo/semantics/publishedVersion

Funding Information: Medical writing support for this article was provided by Paula Pinto, PhD, of PMA – Pharmaceutical Medicine Academy and was funded by Bial. All authors contributed equally to this work and should be considered co-first authors for this study. Funding Information: This article was funded by Bial. Bial provided all necessary scientific bibliography and funded medical writing support and publishing charges. The authors did not receive direct funding for the writing of this article. Publisher Copyright: © 2021 Cardoso et al. The well-recognized individual heterogeneity within COPD patients has led to a growing interest in greater personalization in the approach of these patients. Thus, the treatable traits strategy has been proposed as a further step towards precision medicine in the management of chronic airway disease, both in stable phase and acute exacerbations. The aim of this paper is to perform a critical review on the treatable traits strategy and propose a guide to approach COPD patients in the light of this new concept. An innovative stepwise approach is proposed – a multidisciplinary model based on two distinct phases, with the potential to be implemented in both primary care and hospital settings. The first phase is the initial and focused assessment of a selected subset of treatable traits, which should be addressed in all COPD patients in both settings (primary care and hospital). As some patients may present with advanced disease at diagnosis or may progress despite this initial treatment requiring a more specialized assessment, they should progress to a second phase, in which a broader approach is recommended. Beyond stable COPD, we explore how the treatable traits strategy may be applied to reduce the risk of future exacerbations and improve the management of COPD exacerbations. Since many treatable traits have already been related to exacerbation risk, the strategy proposed here represents an opportunity to be proactive. Although it still lacks prospective validation, we believe this is the way forward for the future of the COPD approach. publishersversion published

Alpha-1 antitrypsin deficiency is an autosomal co-dominant inherited disorder that results in decreased circulating levels of alpha-1 antitrypsin (also known as alpha-1 proteinase inhibitor) and predisposes affected individuals to early onset lung and liver disease. There is currently no cure for alpha-1 antitrypsin deficiency. However, appropriate treatment and a high standard of clinical care can prevent patients from being seriously affected and having to undergo major medical interventions, such as organ transplantation. Beyond managing the symptoms associated with alpha-1 antitrypsin deficiency, alpha-1 proteinase inhibitor therapy is the only treatment for the condition's underlying cause. Early diagnosis is important to ensure efficient therapeutic strategies and to minimize further deterioration of lung function. alpha-1 antitrypsin deficiency is under diagnosed globally, partly because the disease has no unique presenting symptoms. This document was prepared by a Portuguese multidisciplinary group and it aims to set out comprehensive principles of care for Alpha-1 antitrypsin deficiency. These include the importance of registries, the need for clinical research, the need for consistent recommendations (regarding diagnosis, treatment and monitoring), the role of reference centres, the requirement for sustained access to treatment, diagnostic and support services, and the role of patient organizations.A deficiência de alfa-1 antitripsina é uma doença hereditária autossómica co-dominante que resulta numa diminuição dos níveis plasmáticos de alfa-1 antitripsina (também conhecida por inibidor da alfa-1 proteinase) e predispõe os indivíduos afetados ao desenvolvimento de doença pulmonar e hepática precoce. Atualmente não existe cura para a deficiência de alfa-1 antitripsina. No entanto, o tratamento adequado e um elevado padrão de cuidados clínicos podem prevenir que os doentes sejam gravemente afetados e terem que se submeter a intervenções médicas major, como o transplante de órgão. Para além de atuar nos sintomas associados à deficiência de alfa-1 antitripsina, a terapêutica com o inibidor da alfa-1 proteinase é o único tratamento disponível que atua na causa subjacente desta patologia. O diagnostico precoce é importante para assegurar a implementação de estratégias terapêuticas eficientes e para minimizar a destruição adicional da função pulmonar. A deficiência de alfa-1 antitripsina está globalmente sub diagnosticada, em parte devido ao fato desta doença não apresentar sintomas únicos. Este documento foi preparado por um grupo multidisciplinar e visa estabelecer princípios de cuidados abrangentes para a deficiência de alfa-1 antitripsina. Estes incluem a importância dos registros, a necessidade de investigação clinica, a necessidade de recomendações consistentes (no que diz respeito ao diagnostico, tratamento e monitorização), o papel dos centros de referência, a necessidade de acesso sustentado ao tratamento, diagnostico e serviços de suporte, e o papel das associações de doentes.

Chronic obstructive pulmonary disease (COPD) is a complex and heterogeneous disease, and there is a clinical need for validated markers and biomarkers that can contribute to the assessment of patients, risk prediction, treatment guidance, and assessment of response. Although according to the 2018 GOLD guidelines clinically useful biomarkers for COPD patients in stable condition have yet to be identified, several clinical markers and biomarkers have been proposed for COPD. These include isolated clinical markers, such as symptoms and Health Status assessment, exercise tests, function tests and imaging, and also composite scores and molecular markers.However, and despite strong efforts to identify useful markers in an attempt to improve prognostic and therapeutic approaches, results have not been consistent and expectations of relying on these markers in near future are faint.Current approaches to COPD have shifted from treating the disease to treating the individual patient. There is a clear need to identify treatable traits, focusing more on the patient and not on the disease, in order to implement an increasingly personalized treatment of COPD in the clinic, leading to true precision medicine. There is a need to identify combinations of clinical markers and biomarkers, genetic markers, and phenotypes that can guide the personalized therapy of COPD patients.This critical review will therefore focus not only on currently established markers and biomarkers in COPD but also on possible future approaches toward precision medicine. Keywords: COPD, Markers, Biomarkers, Treatable traits, Precision medicine

Background Obstructive sleep apnea (OSA) is associated with increased cardiovascular risk. This study aim to assess differences in changes in arterial stiffness of two groups of patients, defined as having daytime sleepiness or not, after continuous positive airway pressure (CPAP) treatment. Methods A selected cohort of consecutive male patients, under 65 years old, with moderate to severe OSA and without great number of comorbidities was studied. The diagnosis was confirmed by home respiratory poligraphy. Sleepiness was considered with an Epworth Sleepiness Scale (ESS) > 10. An ambulatory blood pressure (BP) monitoring and carotid-femoral pulse wave velocity (cf-PWV) measurements were performed, before and after four months under CPAP. Compliant patients, sleepy and non-sleepy, were compared using linear mixed effects regression models. A further stratified analysis was performed with non-sleepy patients. Results Thirty-four patients were recruited, with mean age 55.2 (7.9) years, 38.2% were sleepy, 79.4% with hypertension, 61.8% with metabolic syndrome and 82.4% with dyslipidaemia. In univariable analysis, cf-PWV was strongly related to systolic BP parameters and age, but also to antihypertensive drugs (p = 0.030), metabolic syndrome (p = 0.025) and daytime sleepiness (p = 0.004). Sleepy patients had a more severe OSA, with AHI 44.8 (19.0) vs 29.7 (15.7) events/h (p = 0.018), but sleep study parameters were not associated with cf-PWV values. On multivariable regression, a significant interaction between time (CPAP) and sleepiness (p = 0.033) was found. There was a weak evidence of a cf-PWV reduction after CPAP treatment (p = 0.086), but the effects of treatment differed significantly between groups, with no changes in non-sleepy patients, while in sleepy patients a significant decrease was observed (p = 0.012). Evaluating non-sleepy patients group under CPAP therapy, results showed that both higher pulse pressure (p = 0.001) and lower LDL-cholesterol levels (p = 0.015) at baseline were associated to higher cf-PWV changes. Conclusions Patients with daytime sleepiness had a more severe OSA and presented a greater arterial stiffness improvement after CPAP therapy, independently from age and BP. Besides sleepiness, cf-PWV reduction after CPAP therapy was mainly associated to CV risk factors, and less to sleep study parameters. Trial registration Clinicaltrials.gov NCT02273089 23.10.2014 retrospectively registered. Electronic supplementary material The online version of this article (10.1186/s12890-017-0518-z) contains supplementary material, which is available to authorized users.

Idiopathic pulmonary fibrosis (IPF) is a progressive and fatal lung disease that up to now has been associated with a poor prognosis. However, the results of the INPULSIS and ASCEND trials and the approval of nintedanib and pirfenidone have marked the beginning of a new era for IPF patients. Questions remain, however. Should these drugs be used earlier? What effect will they have on more severe disease? Will their effects last beyond the trial period? This manuscript is the outcome of a multidisciplinary meeting between pulmonology, radiology, and pathology clinicians on the use of antifibrotic agents in IPF. In our opinion, the existing data show that pirfenidone and nintedanib slow functional decline in early stages of disease. These drugs also appear to result in therapeutic benefits when administered to patients with advanced disease at diagnosis and maintain effective over time. The data also suggest that continuing antifibrotic therapy after disease progression may confer benefits, but more evidence is needed. Early diagnosis and treatment are crucial for reducing functional decline, slowing disease progression, and improving quality of life. Keywords: Idiopathic pulmonary fibrosis, Interstitial lung disease, Nintedanib, Pirfenidone

Introduction: Home Mechanical Ventilation (HMV) is increasing worldwide. Objective: Characterization of the Portuguese HMV Units. Methods: The HMV Team Group of the Portuguese Pulmonology Society prepared a questionnaire that was sent by e-mail addressed to Pneumology Department Directors throughout the country, and the responses were then analyzed. The results enabled a provisional classification of the Units, which followed specific criteria. Results: Thirty centers were surveyed, of which 60% (18) sent the answers to the questionnaire. As for the results obtained, only one center was considered as a basic unit. Most centers (14/18) were considered specialized units. 3/18 centers were classified as highly complex multidisciplinary units. Of the 12 centers that did not answer the questionnaire, one refused to do it and another center was in transition period. Conclusions: Analysis of the results reveals the high number of patients treated with HMV in Portugal, supports the importance of creating protocols to standardize HMV countrywide, and audit its practice through the creation of a national register. info:eu-repo/semantics/publishedVersion

Purpose: Discrepancies between subjective and objective measures of total sleep time (TST) are frequent among insomnia patients, but this issue remains scarcely investigated in obstructive sleep apnea (OSA). We aimed to evaluate if sleep perception is affected by the severity of OSA. Methods: We performed a 3-month cross-sectional study of Brazilian adults undergoing overnight polysomnography (PSG). TST was objectively assessed from PSG and by a self-reported questionnaire (subjective measurement). Sleep perception index (SPI) was defined by the ratio of subjective and objective values. Diagnosis of OSA was based on an apnea/hypopnea index (AHI) ≥ 5.0/h, being its severity classified according to AHI thresholds: 5.0-14.9/h (mild OSA), 15.0-29.9/h (moderate OSA), and ≥ 30.0/h (severe OSA). Results: Overall, 727 patients were included (58.0% males). A significant difference was found in SPI between non-OSA and OSA groups (p = 0.014). Mean SPI values significantly decreased as the OSA severity increased: without OSA (100.1 ± 40.9%), mild OSA (95.1 ± 24.6%), moderate OSA (93.5 ± 25.2%), and severe OSA (90.6 ± 28.2%), p = 0.036. Using logistic regression, increasing SPI was associated with a reduction in the likelihood of presenting any OSA (p = 0.018), moderate/severe OSA (p = 0.019), and severe OSA (p = 0.028). However, insomnia was not considered as an independent variable for the presence of any OSA, moderate/severe OSA, and severe OSA (all p-values > 0.05). Conclusion: In a clinical referral cohort, SPI significantly decreases with increasing OSA severity, but is not modified by the presence of insomnia symptoms. info:eu-repo/semantics/publishedVersion

Objective: We aimed to compare patient's and physician's ratings of inhaled medication adherence and to identify predictors of patient-physician discordance. Design: Baseline data from two prospective multicentre observational studies. Setting: 29 allergy, pulmonology and paediatric secondary care outpatient clinics in Portugal. Participants: 395 patients (≥13 years old) with persistent asthma. Measures: Data on demographics, patient-physician relationship, upper airway control, asthma control, asthma treatment, forced expiratory volume in one second (FEV1) and healthcare use were collected. Patients and physicians independently assessed adherence to inhaled controller medication during the previous week using a 100 mm Visual Analogue Scale (VAS). Discordance was defined as classification in distinct VAS categories (low 0-50; medium 51-80; high 81-100) or as an absolute difference in VAS scores ≥10 mm. Correlation between patients' and physicians' VAS scores/categories was explored. A multinomial logistic regression identified the predictors of physician overestimation and underestimation. Results: High inhaler adherence was reported both by patients (median (percentile 25 to percentile 75) 85 (65-95) mm; 53% VAS>80) and by physicians (84 (68-95) mm; 53% VAS>80). Correlation between patient and physician VAS scores was moderate (rs=0.580; p

A 31-year-old black man, previously resident in Angola, was referred to our hospital complaining of 6 months of progressively worsening shortness of breath with exertion and mucous productive cough. During this period, he had a significant weight loss (about 10 kg) and fatigue. His past medical history included suspected pulmonary tuberculosis (TB), for which he had completed 6 months of anti-bacillary treatment in the year before. He is a construction worker and a nonsmoker, with no other environmental exposure or medication. The physical examination was remarkable for slight tachycardia (heart rate 105 beats·min−1), tachypnoea (respiratory rate 20 cycles·min−1), oxygen saturation of 94% and bilateral inspiratory crackles on both lungs. Figure 1 shows the chest radiograph., Can you diagnose this man with progressively worsening shortness of breath, mucous productive cough, weight loss, fatigue and a history of suspected pulmonary tuberculosis? http://bit.ly/2VUdnTr

Thrombotic microangiopathy (TMA) is a pathologic condition characterized by microangiopathic hemolytic anemia, thrombocytopenia, and organ injury due to microvascular endothelial lesions and thrombosis. It occurs in a variety of diseases and, unless recognized and treated, leads to severe morbidity and mortality. We present the case of a 48-year-old woman who underwent lung transplantation, initially under tacrolimus, mycophenolate mofetil (MMF), and prednisolone. Several complications emerged in the following months, including abdominal aortic and left renal artery thrombosis and cutaneous infections, although her renal function remained normal. Six months after transplant, her renal function began to deteriorate, which was assumed to be due to elevated tacrolimus levels and doses were adjusted. Due to leukopenia, MMF was changed to everolimus. One year after, she was admitted with fatigue, anemia, and renal dysfunction. Complementary exams revealed only iron deficiency, leukopenia, normal platelets, and elevated lactate dehydrogenase; her renal ultrasound was normal. A renal biopsy was performed and thrombotic microangiopathy was subsequently identified as the main cause of the renal dysfunction. Tacrolimus was therefore discontinued and MMF restarted with slow improvement of renal function. Only when everolimus was stopped did the patient's renal function show incremental improvement. TMA may be a serious complication after lung transplantation and the risk is higher when a combination of tacrolimus and everolimus is used. Renal biopsy findings are essential to confirm the final diagnosis of TMA, allowing for a change in immunosuppression to prevent permanent and severe renal damage. info:eu-repo/semantics/publishedVersion

COPD is one of the major public health problems in people aged 40 years or above. It is currently the 4th leading cause of death in the world and projected to be the 3rd leading cause of death by 2020. COPD and cardiac comorbidities are frequently associated. They share common risk factors, pathophysiological processes, signs and symptoms, and act synergistically as negative prognostic factors. Cardiac disease includes a broad spectrum of entities with distinct pathophysiology, treatment and prognosis. From an epidemiological point of view, patients with COPD are particularly vulnerable to cardiac disease. Indeed, mortality due to cardiac disease in patients with moderate COPD is higher than mortality related to respiratory failure. Guidelines reinforce that the control of comorbidities in COPD has a clear benefit over the potential risk associated with the majority of the drugs utilized. On the other hand, the true survival benefits of aggressive treatment of cardiac disease and COPD in patients with both conditions have still not been clarified. Given their relevance in terms of prevalence and prognosis, we will focus in this paper on the management of COPD patients with ischemic coronary disease, heart failure and dysrhythmia. Keywords: Chronic obstructive pulmonary disease, Cardiovascular Disease, Comorbidity, Diagnostic Techniques and Procedures

Noninvasive ventilation (NIV) is an increasingly used method of respiratory support. The use of NIV is expanding over the time and if properly applied, it can save patients' lives and improve long-term prognosis. However, both knowledge and skills of its proper use as life support are paramount. This systematic review aimed to assess the importance of NIV education and training. Literature search was conducted (MEDLINE: 1990 to June, 2018) to identify randomized controlled studies and systematic reviews with the results analyzed by a team of experts across the world through e-mail based communications. Clinical trials examining the impact of education and training in NIV as the primary objective was not found. A few studies with indirect evidence, a simulation-based training study, and narrative reviews were identified. Currently organized training in NIV is implemented only in a few developed countries. Due to a lack of high-grade experimental evidence, an international consensus on NIV education and training based on opinions from 64 experts across the twenty-one different countries of the world was formulated. Education and training have the potential to increase knowledge and skills of the clinical staff who deliver medical care using NIV. There is a genuine need to develop structured, organized NIV education and training programs, especially for the developing countries. info:eu-repo/semantics/publishedVersion

Purpose: Obstructive sleep apnea (OSA) is very common occurrence among morbidly obese patients. Our main objectives were to validate the No-Apnea, a 2-item screening tool, in morbidly obese patients and compare its performance with three other instruments: STOP-Bang questionnaire, NoSAS score, and Epworth Sleepiness Scale (ESS). Methods: A cross-sectional analysis of morbidly obese patients (body mass index [BMI] ≥ 35.0 kg/m2) grouped into two independent samples: bariatric surgery patients (BS) and non-bariatric surgery patients (NBS). All patients underwent overnight polysomnography. Discriminatory ability was assessed by area under the curve (AUC). OSA severity was defined by apnea/hypopnea index cut-off points: ≥ 5.0/h (OSA≥5), ≥ 15.0/h (OSA≥15), and ≥ 30.0/h (OSA≥30). Results: A total of 1017 subjects (40.4% in BS cohort and 59.6% in NBS cohort) were evaluated. In the BS cohort, No-Apnea had similar discrimination to STOP-Bang and NoSAS for predicting OSA≥5 (p = 0.979 and p = 0.358, respectively), OSA≥15 (p = 0.158 and p = 0.399, respectively), and OSA≥30 (p = 0.388 and p = 0.903, respectively). In the NBS cohort, No-Apnea had similar discrimination to STOP-Bang and NoSAS for predicting OSA≥5 (p = 0.528 and p = 0.428, respectively), OSA≥15 (p = 0.825 and p = 0.108, respectively), and OSA≥30 (p = 0.458 and p = 0.186, respectively). Moreover, No-Apnea performed significantly better than ESS in both BS and NBS cohorts (p < 0.001). Conclusions: No-Apnea is a useful and practical tool for screening of OSA in morbidly obese patients, with non-inferior performance to STOP-Bang questionnaire and NoSAS score. info:eu-repo/semantics/publishedVersion

Introduction: The overlap between asthma and chronic obstructive pulmonary disease (COPD) (ACO) has been discussed for many years but clinical recommendations for this entity have been diverse. This study is intended to reach a consensus on diagnosis, treatment and patient orientation for ACO, within the Portuguese medical community. Methods: This study was conducted by a multidisciplinary panel of experts from three distinct medical specialties (Pulmonology, Family Medicine and Immunoallergology). This panel selected a total of 190 clinicians, based on their expertise in obstructive airway diseases, to participate in a Delphi structured survey with three rounds of questionnaires. These results were ultimately discussed, in a meeting with the panel of experts and some of the study participants, and consensus was reached in terms of classification criteria, treatment and orientation of ACO patients. Results: The majority of clinicians (87.2%) considered relevant the definition of an overlap entity between asthma and COPD. A consensus was achieved on the diagnosis of ACO – presence of simultaneous clinical characteristics of asthma and COPD together with a fixed airflow obstruction (FEV1/FVC < 0.7) associated with 2 major criteria (previous history of asthma; presence of a previous history of smoking exposure and/or exposure to biomass combustion; positive bronchodilation test (increase in FEV1 of at least 200 mL and 12%) on more than 1 occasion) plus 1 minor criteria (history of atopy; age ≥40 years; peripheral eosinophilia (>300 eosinophils/μL or >5% of leukocytes); elevation of specific IgEs or positive skin tests for common allergens). A combination of inhaled corticosteroid (ICS) with long-acting beta2-agonist (LABA) or long-acting muscarinic antagonist (LAMA) was considered as first line pharmacological treatment. Triple therapy with ICS plus LABA and LAMA should be used in more severe or symptomatic cases. Non-pharmacological treatment, similar to what is recommended for asthma and COPD, was also considered highly important. A hospital referral of ACO patients should be made in symptomatic or severe cases or when there is a lack of diagnostic resources. Conclusions: This study highlights the relevance of defining ACO, within the Portuguese medical community, and establishes diagnostic criteria that are important for future interventional studies. Recommendations on treatment and patient's orientation were also achieved., Actelion Pharmaceuticals (undefined), info:eu-repo/semantics/publishedVersion

Clinical practice guidelines are ubiquitous and are developed to provide recommendations for the management of many diseases, including chronic obstructive pulmonary disease. The development of these guidelines is burdensome, demanding a significant investment of time and money. In Europe, the majority of countries develop their own national guidelines, despite the potential for overlap or duplication of effort. A concerted effort and consolidation of resources between countries may alleviate the resource-intensity of maintaining individual national guidelines. Despite significant resource investment into the development and maintenance of clinical practice guidelines, their implementation is suboptimal. Effective strategies of guideline dissemination must be given more consideration, to ensure adequate implementation and improved patient care management in the future. info:eu-repo/semantics/publishedVersion

Pleural involvement is the most frequent thoracic complication of rheumatoid arthritis (RA), usually occurring in patients with known RA. Typical rheumatoid pleural effusion is an exudate characterized by low pH and glucose levels and high LDH activity. Rarely, it has features of pseudochylothorax. Other uncommon complications are pneumothorax, hydropneumothorax, empyema, and bronchopleural fistula. The case of a 51-year-oldman with a spontaneous, small, and asymptomatic hydropneumothorax with features of pseudochylothorax is presented. After careful clinical and laboratory evaluation, he was diagnosed with rheumatoid arthritis, and we admitted that the pleural changes were secondary to the connective tissue disease. He started immunosuppressive treatment and maintained stability duringfollow-up,without need of specific pleural treatment. We hypothesized that the pleural nodule found on the chest computed tomography scan was related with the simultaneous occurrence of pleural effusion and pneumothorax. This is a rare presentation and complication of RA, highlighting the utility of a comprehensive clinical and laboratory evaluation and focusing on the importance of pleural rheumatoid nodules in the pathogenesis of RA pleural disease.

Submitted by Ana Quininha (ana.quininha@chlc.min-saude.pt) on 2019-11-22T16:47:52Z No. of bitstreams: 1 Arch Broncon 576.pdf: 535595 bytes, checksum: e24f414601008814c58c873aeabbc402 (MD5) Made available in DSpace on 2019-11-22T16:47:52Z (GMT). No. of bitstreams: 1 Arch Broncon 576.pdf: 535595 bytes, checksum: e24f414601008814c58c873aeabbc402 (MD5) Previous issue date: 2018 info:eu-repo/semantics/publishedVersion

Introduction Sleepiness is a cardinal symptom in obstructive sleep apnoea (OSA) but most patients have unspecific symptoms. Arterial stiffness, evaluated by pulse wave velocity (PWV), is related to atherosclerosis and cardiovascular (CV) risk. Arterial stiffness was reported to be higher in patients with OSA, improving after treatment with continuous positive airway pressure (CPAP). This study aims to assess whether the same effect occurs in patients with OSA and without sleepiness. Methods and analysis This observational study assesses the CV effect of CPAP therapy on a cohort of patients with moderate-to-severe OSA; the effect on the subcohorts of sleepy and non-sleepy patients will be compared. A systematic and consecutive sample of patients advised CPAP therapy will be recruited from a single outpatient sleep clinic (Centro Hospitalar de Lisboa Central—CHLC, Portugal). Eligible patients are male, younger than 65 years, with confirmed moderate-to-severe OSA and apnoea–hypopnea index (AHI) above 15/hour. Other sleep disorders, diabetes or any CV disease other than hypertension are exclusion criteria. Clinical evaluation at baseline includes Epworth Sleepiness Scale (ESS), and sleepiness is defined as ESS above 10. OSA will be confirmed by polygraphic study (cardiorespiratory, level 3). Participants are advised to undertake an assessment of carotid-femoral PWV (cf-PWV) and 24 hours evaluation of ambulatory blood pressure monitoring (ABPM), at baseline and after 4 months of CPAP therapy. Compliance and effectiveness of CPAP will be assessed. The main outcome is the variation of cf-PWV over time. Ethics and dissemination This protocol was approved by the Ethics Committees of CHLC (reference number 84/2012) and NOVA Medical School (number36/2014/CEFCM), Lisbon. Informed, written consent will be obtained. Its results will be presented at conferences and published in peer-reviewed journals. Trial registration number NCT02273089; Pre-results.

A 68-year-old man with type 2 diabetes mellitus, hypertension and history of laryngeal cancer surgery with tracheostomy was operated on in 1993 and right chest rib chondrosarcoma surgery was performed in 1997. The patient presented with fever and acute right sided hypochondrium pain. Lung examination showed decreased breath sounds over the right posterior of the lower chest. Blood examination revealed elevation of inflammatory markers. Chest radiography showed right inferior lung lobe …

Resumo: A espirometria não atingiu ainda a divulgação que se justificaria em patologia respiratória, ou indivíduos que se encontram em risco relativamente a esta patologia, cujo diagnóstico é insuficiente, havendo um escasso conhecimento, e consequente controlo, dos custos atribuíveis a estas doenças, com destaque para a doença pulmonar obstrutiva crónica (DPOC).O PNEUMOBIL, iniciativa que visa esta divulgação entre fumadores e ex-fumadores, foi reactivado, após 10 anos de aplicação em Portugal, revelando agora, numa amostra de 5324 indivíduos, em que cerca de 50% ainda mantêm os hábitos tabágicos, sejam do sexo masculino ou feminino, que houve uma elevada prevalência de obstrução detectada por espirometria (30% e 25%, respecti-vamente) nas pessoas rastreadas perto de centros de saúde (grupo público) e em empresas (grupo privado). Este risco não se explica em regra por exposição ocu-pacional, nem se relaciona com a maioria dos sintomas respiratórios, muito frequentes nos rastreados. Apenas a dispneia (OR=1,28; p=0,02) e os episódios frequentes de expectoração (OR=1,21; p=0,008) ou de bronquite aguda (OR=1,31; p=0,05) revelam al-guma relação com a obstrução. O reconhecimento prévio da DPOC é muito reduzi-do e a presença de obstrução não se correlaciona (p=0,204) com o assumir da condição de portador.Rev Port Pneumol 2009; XV (5): 803-846 Abstract: The use of spirometry is not yet widespread enough in chronic respiratory or at-risk patients whose diagnosis is incomplete. There is scarce knowledge and inadequate management of the burden of these diseases, particularly chronic obstructive pulmonary disease (COPD).Pneumobil, an initiative aimed at raising awareness among smokers and ex-smokers, was reactivated 10 years after its launch in Portugal. It found a large prevalence of bronchial obstruction as measured by spirometry (30% and 25% in men and women respectively) in a sample of 5324 smoke-exposed individuals, 50% current smokers, screened at state or business (private company group) health institutions. This risk is neither mainly attributable to occupational exposure nor mainly related to respiratory symptoms, which were very common in our population. Only dyspnoea (OR=1.28; p=0.02) and frequent episodes of sputum production (OR=1.21; p=0.008) or acute bronchitis (OR=1.31; p=0.05) were somewhat related to bronchial obstruction. Prior knowledge of COPD is rare and bronchial obstruction is not correlated (p=0.204) to a possible diagnosis of COPD.Rev Port Pneumol 2009; XV (5): 803-846 Palavras-chave: Fumo do tabaco, obstrução brôn-quica, DPOC, PNEUMOBIL, Key-words: Tobacco smoke, bronchial obstruction, COPD, PNEUMOBIL

Submitted by Ana Quininha (ana.quininha@chlc.min-saude.pt) on 2011-06-07T15:32:59Z No. of bitstreams: 1 RPP 2008 1.pdf: 1184669 bytes, checksum: 614b8745af3b29362737aee635583def (MD5) Made available in DSpace on 2011-06-07T15:32:59Z (GMT). No. of bitstreams: 1 RPP 2008 1.pdf: 1184669 bytes, checksum: 614b8745af3b29362737aee635583def (MD5) Previous issue date: 2008

RESUMO: ReJatamos três casos de doença de Hodgkin com envolvimento pulmonar. Três doentes tinham sintomas sistémicos e apenas dois respiratórios. Estudos imagiológicos revelaram uma massa sólida no lobo inferior esquerdo no primeiro caso, infiltrados nodulares com cavitação no segundo e uma consolidação pneumónica no terceiro. O diagnóstico de doençã de Hodgkin foi feito em biópsias: brônquica no caso urn, pulmonar por toracoscopia no caso dois e gangUonar no caso Três. Os achados histo1ógicos destes casos eram típlcos da doença. Todos os doentes foram tratados com quimioterapia. Responderam bern ao tratamento mostrando regressão franca das lesões na reavaliação passados três meses.REV PORT PNEUMOL 2000; VI (5): 373-381 ABSTRACT: We report three cases of Hodgkin disease with pulmonary involvement. There were systemic symptoms in all three, and respiratory symptoms in just two patients. lmageologic studies revealed a solid mass on the inferior left pulmonary lobe in the first case, nodular infiltrates with cavitation in the second, and a pneumonic consoUdation in the third. The diagnosis of Hodgkinâs disease was made on biopsies: bronchial in case one, pulmonary through thoracoscopy in case two and lymph node in case three. The histological features of these cases were very typical of the dJsease. All patients were treated with chemotherapy. They responded well to treatment, showing frank regression of the lesions on three month follow-up.REV PORT PNEUMOL 2000; VI (5): 373-381 Palavras-chave: Linfomas pulmonnres, Doença de Hodgkin, Key-words: Pulmonary Lymphomas, Hodgkinâs Disease

Submitted by Ana Quininha (ana.quininha@chlc.min-saude.pt) on 2011-06-09T14:55:28Z No. of bitstreams: 1 RPP 1997 429.pdf: 962729 bytes, checksum: 37987243944c705336850b9de2711240 (MD5) Made available in DSpace on 2011-06-09T14:55:28Z (GMT). No. of bitstreams: 1 RPP 1997 429.pdf: 962729 bytes, checksum: 37987243944c705336850b9de2711240 (MD5) Previous issue date: 1997

A broncofibroscopia (BF) é frequentemente efectuada em doentes com pneumonias de evolução arrastada com o objectivo de excluir patologia endobrónquica de etiologia neoplásica. Dado que a resolução radiográfica das pneumonias da comunidade é variável, sendo dependente de vários factores (agente etiológico, idade, doenças associadas) a decisão para efectuar uma BF é muitas vezes empírica. Com o objectivo de descrever a nossa experiência neste problema estudámos retrospectivamente 123 doentes com o diagnóstico de pneumonia da comunidade baseado em critérios clínicos e radiográficos e que, apesar da antibioferapia considerada adequada, não apresentavam melhoria radiográfica significativa ao fim de pelo menos 2 semanas. Obtivemós um diagnóstico histológico de neoplasia maligna do pulmão em 7 doentes (5,6° o), sendo todos do sexo masculino, com idade superior a 55 anos efuínadores de pelo menos 40 U.M.A. A análise comparativa com os restantes doentes mostrou diferenças com significado~estatístico em relação à idade e ao consumo tabágico. O tempo de evolução da doença,valores médios de hemoglobina, leucocitos, VS, existência ou não de alterações da função renal ou hepática não foram significativamente diferentes nos dois grupos. Concluimos que a BF deve ser efectuada precocemente nos doentes pertencentes ao grupo de risco identificado (fumadores, com mais de 55 anos) não se justificando a sua realização nos restantes doentes antes das 4 a 8 semanas de evolução, a menos que estejam presentes critérios clínicos objectivos tais como progressão da doença ou agravamento do estado geral.

Submitted by Ana Quininha (ana.quininha@chlc.min-saude.pt) on 2014-10-10T16:43:56Z No. of bitstreams: 1 AMP 1995 493.pdf: 385006 bytes, checksum: f6e80e1f4d4f2394a32caa528a3d080d (MD5) Made available in DSpace on 2014-10-10T16:43:56Z (GMT). No. of bitstreams: 1 AMP 1995 493.pdf: 385006 bytes, checksum: f6e80e1f4d4f2394a32caa528a3d080d (MD5) Previous issue date: 1995