Issue 1 - "Update on adverse respiratory effects of outdoor air pollution" Part 2): Outdoor air pollution and respiratory diseases: Perspectives from Angola, Brazil, Canada, Iran, Mozambique and Portugal.

OBJECTIVE: To analyse the GARD perspective on the health effects of outdoor air pollution, and to synthesise the Portuguese epidemiological contribution to knowledge on its respiratory impact. RESULTS: Ambient air pollution has deleterious respiratory effects which are more apparent in larger, densely populated and industrialised countries, such as Canada, Iran, Brazil and Portugal, but it also affects people living in low-level exposure areas. While low- and middle-income countries (LMICs), are particularly affected, evidence based on epidemiological studies from LMICs is both limited and heterogeneous. While nationally, Portugal has a relatively low level of air pollution, many major cities face with substantial air pollution problems. Time series and cross-sectional epidemiological studies have suggested increased respiratory hospital admissions, and increased risk of respiratory diseases in people who live in urban areas and are exposed to even a relatively low level of air pollution. CONCLUSIONS: Adverse respiratory effects due to air pollution, even at low levels, have been confirmed by epidemiological studies. However, evidence from LMICs is heterogeneous and relatively limited. Furthermore, longitudinal cohort studies designed to study and quantify the link between exposure to air pollutants and respiratory diseases are needed. Worldwide, an integrated approach must involve multi-level stakeholders including governments (in Portugal, the Portuguese Ministry of Health, which hosts GARD-Portugal), academia, health professionals, scientific societies, patient associations and the community at large. Such an approach not only will garner a robust commitment, establish strong advocacy and clear objectives, and raise greater awareness, it will also support a strategy with adequate measures to be implemented to achieve better air quality and reduce the burden of chronic respiratory diseases (CRDs).

Translating Idiopathic pulmonary fibrosis guidelines into clinical practice.

INTRODUCTION AND OBJECTIVES: Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive fibrotic interstitial lung disease of unknown cause, which predominantly manifests in older males. IPF diagnosis is a complex, multi-step process and delay in diagnosis cause a negative impact on patient survival. Additionally, a multidisciplinary team of pulmonologists, radiologists and pathologists is necessary for an accurate IPF diagnosis. The present study aims to assess how diagnosis and treatment of IPF are followed in Portugal, as well as the knowledge and implementation of therapeutic guidelines adopted by the Portuguese Society of Pulmonology. MATERIALS AND METHODS: Seventy-eight practicing pulmonologists were enrolled (May-August 2019) in a survey developed by IPF expert pulmonologists comprised of one round of 31 questions structured in three parts. The first part was related to participant professional profile, the second part assessed participant level of knowledge and practice agreement with national consensus and international guidelines for IPF as well as their access to radiology and pathology for IPF diagnosis, and the third part was a self-evaluation of the guidelines adherence for diagnosis and treatment in their daily practice. RESULTS: Participants represented a wide spectrum of pulmonologists from 14 districts of Portugal and autonomous regions of Azores and Madeira. The majority were female (65%), with 5-19 years of experience (71%) and working in a public clinical center (83%). Importantly, the majority of pulmonologists follow their IPF patients (n=45) themselves, while 26% referred IPF patients to ILD experts in the same hospital and 22% to another center. Almost all pulmonologists (98%) agreed or absolutely agreed that multidisciplinary discussion is recommended to accurately diagnose IPF. No pulmonologists considered pulmonary biopsy as absolutely required to establish an IPF diagnosis. However, 87% agreed or absolutely agree with considering biopsy in a possible/probable UIP context. If a biopsy is necessary, 96% of pulmonologists absolutely agree or agree with considering criobiopsy as an option for IPF diagnosis. Regarding IPF treatment, 98% absolutely agreed or agreed that antifibrotic therapy should be started once the IPF diagnosis is established. Finally, 76% stated that 6 months is the recommended time for follow-up visit in IPF patients. CONCLUSIONS: Portuguese pulmonologists understand and agree with national consensus and international guidelines for IPF treatment but their implementation in Portugal is heterogeneous.

COPD exacerbations: management and hospital discharge.

Chronic Obstructive Pulmonary Disease (COPD) is a serious pulmonary condition. Many patients experience exacerbations and some require Emergency Room visits and hospitalization. In Portugal, hospitalizations due to COPD between 2009 and 2016 decreased by 8%, but they still represented 8049 hospitalized patients in 2016. Appropriate management of COPD exacerbations presents a clinical challenge and, in order to guide therapy, it is important to identify the underlying cause; however, this is not possible in about a third of severe COPD exacerbations. There are several diagnostic tools that can be used to assess an exacerbation and its severity, which will in turn guide treatment, and prognostic scores should be used to predict the risk of future exacerbations. After an exacerbation is appropriately managed, a suitable discharge plan should be prepared. This should generally include reclassification of the patient according to GOLD criteria, optimization of pharmacological therapy, management of comorbidities, patient (or caregiver) education on the correct use of medications, referral to a Pulmonology Outpatient Clinic, if they are not already attending one, and a smoking cessation and respiratory rehabilitation program. In this paper, we will focus on the pharmacological strategies for the management of COPD exacerbations, risk stratification and a hospital discharge plan proposal.

Asthma-COPD overlap: A Portuguese survey.

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 200mL and 12%) on more than 1 occasion) plus 1 minor criteria (history of atopy; age ≥40 years; peripheral eosinophilia (>300eosinophils/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.

Migration, TB control and elimination: Whom to screen and treat.

Tuberculosis (TB) in migrants represents an important clinical and public health threat, particularly in low TB incidence countries. The current review is aimed to assess issues related to screening and treatment of migrants with latent TB infection or TB disease.

Idiopathic pulmonary fibrosis in the era of antifibrotic therapy: Searching for new opportunities grounded in evidence.

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.

Erratum to: Scaling up strategies of the chronic respiratory disease programme of the European Innovation Partnership on Active and Healthy Ageing (Action Plan B3: Area 5).

[This corrects the article DOI: 10.1186/s13601-016-0116-9.].

Scaling up strategies of the chronic respiratory disease programme of the European Innovation Partnership on Active and Healthy Ageing (Action Plan B3: Area 5).

Action Plan B3 of the European Innovation Partnership on Active and Healthy Ageing (EIP on AHA) focuses on the integrated care of chronic diseases. Area 5 (Care Pathways) was initiated using chronic respiratory diseases as a model. The chronic respiratory disease action plan includes (1) AIRWAYS integrated care pathways (ICPs), (2) the joint initiative between the Reference site MACVIA-LR (Contre les MAladies Chroniques pour un VIeillissement Actif) and ARIA (Allergic Rhinitis and its Impact on Asthma), (3) Commitments for Action to the European Innovation Partnership on Active and Healthy Ageing and the AIRWAYS ICPs network. It is deployed in collaboration with the World Health Organization Global Alliance against Chronic Respiratory Diseases (GARD). The European Innovation Partnership on Active and Healthy Ageing has proposed a 5-step framework for developing an individual scaling up strategy: (1) what to scale up: (1-a) databases of good practices, (1-b) assessment of viability of the scaling up of good practices, (1-c) classification of good practices for local replication and (2) how to scale up: (2-a) facilitating partnerships for scaling up, (2-b) implementation of key success factors and lessons learnt, including emerging technologies for individualised and predictive medicine. This strategy has already been applied to the chronic respiratory disease action plan of the European Innovation Partnership on Active and Healthy Ageing.

Consensus document for the diagnosis and treatment of idiopathic pulmonary fibrosis: Joint Consensus of Sociedade Portuguesa de Pneumologia, Sociedade Portuguesa de Radiologia e Medicina Nuclear e Sociedade Portuguesa de Anatomia Patológica.

Idiopathic pulmonary fibrosis is a rare interstitial lung disease included in the Idiopathic Interstitial Pneumonias group. Although several potential risk factors have been described, it is a progressive fibrosing disease of unknown cause affecting mainly adults over 50 years and associated with a poor prognosis, reflected in a median survival of 2-3 years after diagnosis. The concept of a multidisciplinary working group for the diagnosis of idiopathic pulmonary fibrosis is based on the need to have experienced pulmonologists, radiologists and pathologists in the evaluation and correct treatment of the disease, and requires the use of all available data about individual patients, standardized (largely through High Resolution Computed Tomography and pathology when needed) as well as non-standardized data (laboratory, serology and biomarkers). This approach helps to increase diagnostic accuracy and is an internationally accepted recommendation. In regard to therapy, the situation has changed radically since the publication of the ATS/ERS/JRS/ALAT 2011 guidelines on the diagnosis and management of idiopathic pulmonary fibrosis where it was stressed that no proven therapy exists for this disease. Currently besides non-pharmacological treatment, therapy of complications and comorbidities and palliative care, nintedanib and pirfenidone, two compounds with pleiotropic mechanisms of action, are to date, the two drugs with confirmed efficacy in slowing functional decline and disease progression in idiopathic pulmonary fibrosis patients.

Optimal treatment sequence in COPD: Can a consensus be found?

There is currently no consensus on the treatment sequence in chronic obstructive pulmonary disease (COPD), although it is recognized that early diagnosis is of paramount importance to start treatment in the early stages of the disease. Although it is fairly consensual that initial treatment should be with an inhaled short-acting beta agonist, a short-acting muscarinic antagonist, a long-acting beta-agonist or a long-acting muscarinic antagonist. As the disease progresses, several therapeutic options are available, and which to choose at each disease stage remains controversial. When and in which patients to use dual bronchodilation? When to use inhaled corticosteroids? And triple therapy? Are the existing non-inhaled therapies, such as mucolytic agents, antibiotics, phosphodiesterase-4 inhibitors, methylxanthines and immunostimulating agents, useful? If so, which patients would benefit? Should co-morbidities be taken into account when choosing COPD therapy for a patient? This paper reviews current guidelines and available evidence and proposes a therapeutic scheme for COPD patients. We also propose a treatment algorithm in the hope that it will help physicians to decide the best approach for their patients. The authors conclude that, at present, a full consensus on optimal treatment sequence in COPD cannot be found, mainly due to disease heterogeneity and lack of biomarkers to guide treatment. For the time being, and although some therapeutic approaches are consensual, treatment of COPD should be patient-oriented.

Consensus document for the prevention of respiratory infections in adults.

Infectious diseases are one of the principle causes of morbidity, mortality and drain on health resources worldwide. In recent years there has been an increase in the impact of respiratory infections, particularly in the Portuguese population. It is for this reason that the Portuguese Respiratory Society has presented a series of recommendations for the prevention of respiratory infections in adults. These recommendations include both general measures and vaccinations for flu and pneumococcal pneumonia.

Environmental tobacco smoke exposure at home and smoking prevalence in the general Portuguese population--the INAsma study.

BACKGROUND: We aimed to: 1) estimate the prevalence of exposure to environmental tobacco smoke (ETS) at home in the Portuguese population; 2) estimate tobacco smoking prevalence in Portugal; 3) identify social and personal characteristics associated with smoking or exposure to ETS. METHODS: Nationwide, cross-sectional, population-based telephone survey. Overall, 6003 individuals completed the interview. ETS exposure at home was defined as exposure to at least one current smoker at home. A smoker was defined as someone with 15 years or older, smoking at least 1 cigarette per day during a year; a current smoker (CS) smoked in the last month. RESULTS: Exposure to ETS at home was reported by 26.6% (95%CI 25.5-27.7) of the participants. Living in households with ≥4 persons (OR=2.31; 95%CI[1.81-2.96]), being a current smoker (OR=7.29; 95%CI[5.74-9.26]) or having current asthma (OR=2.06; 95%CI[1.45-2.94]) were factors positively associated with ETS exposure. When analyzed by gender, the effect of current asthma was only relevant to females. Currently 19.0% (95%CI 18.0-20.0) of the Portuguese population smokes tobacco and 17.2% (95%CI 16.2-18.2) are ex-smokers. CS prevalence is higher in males than females (26.5%versus 12.2%, p<0,001). The odds of being a CS were higher for males, the more educated, and those exposed to ETS at home. When analyzed by gender, school education only affected females. CONCLUSION: Exposure to ETS at home was higher than previously reported. Children/adolescents and asthma patients may have a higher risk of exposure. This report endorses a decreasing trend in the prevalence of tobacco smoking in Portuguese males, but a tendency to increase in females.

Nanoparticles, nanotechnology and pulmonary nanotoxicology.

The recently emergent field of Nanotechnology involves the production and use of structures at the nanoscale. Research at atomic, molecular or macromolecular levels, has led to new materials, systems and structures on a scale consisting of particles less than 100 nm and showing unique and unusual physical, chemical and biological properties, which has enabled new applications in diverse fields, creating a multimillion-dollar high-tech industry. Nanotechnologies have a wide variety of uses from nanomedicine, consumer goods, electronics, communications and computing to environmental applications, efficient energy sources, agriculture, water purification, textiles, and aerospace industry, among many others. The different characteristics of nanoparticles such as size, shape, surface charge, chemical properties, solubility and degree of agglomeration will determine their effects on biological systems and human health, and the likelihood of respiratory hazards. There are a number of new studies about the potential occupational and environmental effects of nanoparticles and general precautionary measures are now fully justified. Adverse respiratory effects include multifocal granulomas, peribronchial inflammation, progressive interstitial fibrosis, chronic inflammatory responses, collagen deposition and oxidative stress. The authors present an overview of the most important studies about respiratory nanotoxicology and the effects of nanoparticles and engineered nanomaterials on the respiratory system.

[Necrotizing pneumonia - a rare complication]. / Pneumonia Necrotizante - Uma Complicação Rara.

Necrotizing pneumonia is a rare complication of community-acquired pneumonia associated with destruction of the lung tissue during the infection and rise of necrotic foci in consolidated areas. Staphylococcus aureus, Streptococcus pyogenes, Nocardia, Klebsiella pneumoniae and Streptococcus pneumoniae are the most common causative agents. Risk factors for developing necrotizing pneumonia include smoking, alcoholism, old age, diabetes mellitus, chronic lung diseases or liver disease. Diagnosis of necrotizing pneumonia requires supportive care, use of broad-spectrum antibiotics and monitoring for sepsis and respiratory failure. Hemoptysis, abscess, empyema and gangrene are possible complications and surgical intervention may be required. The authors present a clinical case of pneumococcal necrotizing pneumonia in a patient without important risk factors and favourable progression with medical therapy. In this regard, a brief bibliographic review about this pathology is also made.

Selected clinical highlights from the 2010 ERS Congress in Barcelona.

This article reviews a selection of presentations at the 2010 annual meeting of the European Respiratory Society held in Barcelona, Spain, which was the largest congress ever in the field of respiratory medicine. The best abstracts from the groups of the Clinical Assembly (Clinical Problems, Rehabilitation and Chronic Care, Imaging, Interventional Pulmonology, Diffuse Parenchymal Lung Disease, and General Practice and Primary Care) are presented in the context of the current literature.