Screening for pulmonary arterial hypertension in systemic sclerosis
Pulmonary arterial hypertension (PAH) is a dreaded complication of systemic sclerosis (SSc) that occurs in ~10% of patients. Most individuals present with severe symptoms, significant functional impairment and severe haemodynamics at diagnosis, and survival after PAH diagnosis is poor. Therefore, early diagnosis through systematic screening of asymptomatic patients has the potential to identify PAH at an early stage. Current evidence suggests that early diagnosis and treatment of PAH in patients with SSc may lead to better clinical outcomes. Annual screening may include echocardiography, but this can miss some patients due...
Source: European Respiratory Review - July 31, 2019 Category: Respiratory Medicine Authors: Weatherald, J., Montani, D., Jevnikar, M., Jaïs, X., Savale, L., Humbert, M. Tags: Pulmonary vascular disease Review Source Type: research

Does cytoreduction surgery and hyperthermic intrathoracic chemotherapy prolong survival in patients with N0-N1 nonsmall cell lung cancer and malignant pleural effusion?
Conclusion Although reported survival in this systematic review is encouraging, available evidence concerning debulking surgery and HITHOC in N0–N1 NSCLC with malignant pleural effusion is weak. Better evidence in the form of a randomised controlled trial is mandatory. (Source: European Respiratory Review)
Source: European Respiratory Review - July 31, 2019 Category: Respiratory Medicine Authors: Migliore, M., Nardini, M. Tags: Lung cancer Systematic review Source Type: research

Obstructive sleep apnoea in acute coronary syndrome
Obstructive sleep apnoea (OSA) syndrome affects about 13% of the male and 7–9% of the female population. Hypoxia, oxidative stress and systemic inflammation link OSA and cardiovascular and metabolic consequences, including coronary artery disease. Current research has identified several clinical phenotypes, and the combination of breathing disturbances during sleep, systemic effects and end-organ damage might help to develop personalised therapeutic approaches. It is unclear whether OSA is a risk factor for acute coronary syndrome (ACS) and might affect its outcome. On the one hand, OSA in patients with ACS may worse...
Source: European Respiratory Review - July 31, 2019 Category: Respiratory Medicine Authors: Randerath, W., Bonsignore, M. R., Herkenrath, S. Tags: Sleep medicine Sleep disordered breathing Source Type: research

Severe T2-high asthma in the biologics era: European experts' opinion
The European Respiratory Biologics Forum gathered participants from 21 countries in Madrid, Spain, to discuss the management and treatment of severe asthma in the era of biologics. The current insights on the pathophysiology of severe asthma were discussed, as well as the role of respiratory biologics in clinical practice and strategies for eliminating chronic use of oral corticosteroids. The participants also highlighted the key challenges in identifying patients with severe asthma based on phenotypes, biomarkers and treatable traits, and the existing problems in patient referral to specialist care. The monitoring of trea...
Source: European Respiratory Review - July 8, 2019 Category: Respiratory Medicine Authors: Pavord, I., Bahmer, T., Braido, F., Cosio, B. G., Humbert, M., Idzko, M., Adamek, L. Tags: Asthma and allergy Review Source Type: research

The contribution of infection and the respiratory microbiome in acute exacerbations of idiopathic pulmonary fibrosis
Idiopathic pulmonary fibrosis (IPF) arises in genetically susceptible individuals as a result of an aberrant wound-healing response following repetitive alveolar injury. The clinical course of the disease remains both variable and unpredictable with periods of more rapid decline, termed acute exacerbation of IPF (AE-IPF), often punctuating the disease trajectory. Exacerbations carry a significant morbidity and mortality, and their exact pathogenesis remains unclear. Given the emerging evidence that disruption and alteration in the lung microbiome plays a role in the pathogenesis and progression of IPF, this review discusse...
Source: European Respiratory Review - July 8, 2019 Category: Respiratory Medicine Authors: Invernizzi, R., Molyneaux, P. L. Tags: Interstitial and orphan lung disease Lung Science Conference Source Type: research

Influence of the lung microbiome on antibiotic susceptibility of cystic fibrosis pathogens
The lungs of patients with cystic fibrosis (CF) are colonised by a microbial community comprised of pathogenic species, such as Pseudomonas aeruginosa and Staphylococcus aureus, and microorganisms that are typically not associated with worse clinical outcomes (considered as commensals). Antibiotics directed at CF pathogens are often not effective and a discrepancy is observed between activity of these agents in vitro and in the patient. This review describes how interspecies interactions within the lung microbiome might influence the outcome of antibiotic treatment targeted at common CF pathogens. Protective mechanisms by ...
Source: European Respiratory Review - July 8, 2019 Category: Respiratory Medicine Authors: Vandeplassche, E., Tavernier, S., Coenye, T., Crabbe, A. Tags: CF and non-CF bronchiectasis Lung Science Conference Source Type: research

Interdisciplinary multimodality management of stage III nonsmall cell lung cancer
Stage III nonsmall cell lung cancer (NSCLC) comprises about one-third of NSCLC patients and is very heterogeneous with varying and mostly poor prognosis. It is also called "locoregionally or locally advanced disease". Due to its heterogeneity a general schematic management approach is not appropriate. Usually a combination of local therapy (surgery or radiotherapy, depending on functional, technical and oncological operability) with systemic platinum-based doublet chemotherapy and, recently, followed by immune therapy is used. A more aggressive approach of triple agent chemotherapy or two local therapies (surgery...
Source: European Respiratory Review - July 8, 2019 Category: Respiratory Medicine Authors: Huber, R. M., De Ruysscher, D., Hoffmann, H., Reu, S., Tufman, A. Tags: Lung cancer Review Source Type: research

COPD beyond proximal bronchial obstruction: phenotyping and related tools at the bedside
Chronic obstructive pulmonary disease (COPD) is characterised by nonreversible proximal bronchial obstruction leading to major respiratory disability. However, patient phenotypes better capture the heterogeneously reported complaints and symptoms of COPD. Recent studies provided evidence that classical bronchial obstruction does not properly reflect respiratory disability, and symptoms now form the new paradigm for assessment of disease severity and guidance of therapeutic strategies. The aim of this review was to explore pathways addressing COPD pathogenesis beyond proximal bronchial obstruction and to highlight innovativ...
Source: European Respiratory Review - July 8, 2019 Category: Respiratory Medicine Authors: Capron, T., Bourdin, A., Perez, T., Chanez, P. Tags: COPD and smoking Review Source Type: research

Bariatric surgery: a potential cure for asthma?
Asthma incidence and severity are increased in obese populations. Systematic reviews have shown benefit from weight-loss interventions on asthma outcomes, but the role of bariatric surgery is still unclear. In this review, cohorts of obese asthmatic patients undergoing bariatric surgery were examined regarding different asthma outcomes. The available data on patients who were followed up showed improvements in asthma control, exacerbation risk, asthma-related hospitalisation, medication use and airway hyperresponsiveness, with some patients not requiring further treatment for asthma. Follow-up duration was variable, being ...
Source: European Respiratory Review - July 8, 2019 Category: Respiratory Medicine Authors: Chaaban, T. A. Tags: Asthma and allergy Mini-review Source Type: research

Sensors for detecting pulmonary diseases from exhaled breath
This review presents and discusses a new frontier for fast, risk-free and potentially inexpensive diagnostics of respiratory diseases by detecting volatile organic compounds (VOCs) present in exhaled breath. One part of the review is a didactic presentation of the overlaying concept and the chemistry of exhaled breath. The other part discusses diverse sensors that have been developed and used for the detection of respiratory diseases (e.g. chronic obstructive pulmonary disease, asthma, lung cancer, pulmonary arterial hypertension, tuberculosis, cystic fibrosis, obstructive sleep apnoea syndrome and pneumoconiosis) by analy...
Source: European Respiratory Review - June 26, 2019 Category: Respiratory Medicine Authors: Hashoul, D., Haick, H. Tags: Pulmonary pharmacology and therapeutics Review Source Type: research

Adipose tissue as a key player in obstructive sleep apnoea
This article aims to summarise the effects of IH on adipose tissue in experimental models in a comprehensive way. Data from well-designed controlled trials are also reported with the final goal of proposing new avenues for improving phenotyping and personalised care in OSA. (Source: European Respiratory Review)
Source: European Respiratory Review - June 26, 2019 Category: Respiratory Medicine Authors: Ryan, S., Arnaud, C., Fitzpatrick, S. F., Gaucher, J., Tamisier, R., Pepin, J.-L. Tags: Sleep medicine Sleep disordered breathin Source Type: research

A review of psychosocial factors and personality in the treatment of obstructive sleep apnoea
This article disseminates personality traits observed in patients with OSA and identifies vulnerable groups who may require additional support to increase treatment adherence. It summarises the current evidence for treatment barriers in patients with OSA. Low self-efficacy in relation to CPAP and weight loss adherence will be explored as well as the potential to predict treatment responders and enhance therapeutic uptake and adherence. Extending personality traits into research and clinical practice could potentially result in more successful CPAP therapy and weight loss treatment outcomes. (Source: European Respiratory Review)
Source: European Respiratory Review - June 26, 2019 Category: Respiratory Medicine Authors: Cayanan, E. A., Bartlett, D. J., Chapman, J. L., Hoyos, C. M., Phillips, C. L., Grunstein, R. R. Tags: Sleep medicine Sleep disordered breathin Source Type: research

Breath analysis of cancer in the present and the future
Most of the currently used diagnostics for cancerous diseases have yet to meet the standards of screening, as they are insufficiently accurate and/or invasive and risky. In this review, we describe the rationale, the progress made to date, and the potential of analysing the exhaled volatile organic compounds as a pathway for enabling early diagnosis of cancer and, therefore, for achieving better clinical prognosis and survival rates. The review highlights the major advancements made in this field, from fundamentals, up to translational phases and clinical trials, with a special emphasis on sensing ...
Source: European Respiratory Review - June 26, 2019 Category: Respiratory Medicine Authors: Einoch Amor, R., Nakhleh, M. K., Barash, O., Haick, H. Tags: Lung cancer Review Source Type: research

Regimens to treat multidrug-resistant tuberculosis: past, present and future perspectives
The objectives of this article are to review the main achievements in MDR-TB treatment through the description of the existing WHO strategies, to discuss the main ongoing trials and to shed light on potential future scenarios and revised definitions necessary to manage drug-resistant TB. (Source: European Respiratory Review)
Source: European Respiratory Review - May 29, 2019 Category: Respiratory Medicine Authors: Pontali, E., Raviglione, M. C., Migliori, G. B., and the writing group members of the Global TB Network Clinical Trials Committee Tags: Respiratory infections and tuberculosis Mini-review Source Type: research

Effectiveness of interventions for reducing TB incidence in countries with low TB incidence: a systematic review of reviews
Aims What is the evidence base for the effectiveness of interventions to reduce tuberculosis (TB) incidence in countries which have low TB incidence? Methods We conducted a systematic review of interventions for TB control and prevention relevant to low TB incidence settings (
Source: European Respiratory Review - May 29, 2019 Category: Respiratory Medicine Authors: Collin, S. M., Wurie, F., Muzyamba, M. C., de Vries, G., Lönnroth, K., Migliori, G. B., Abubakar, I., Anderson, S. R., Zenner, D. Tags: Respiratory infections and tuberculosis Review Source Type: research

The time-controlled adaptive ventilation protocol: mechanistic approach to reducing ventilator-induced lung injury
Airway pressure release ventilation (APRV) is a ventilator mode that has previously been considered a rescue mode, but has gained acceptance as a primary mode of ventilation. In clinical series and experimental animal models of extrapulmonary acute respiratory distress syndrome (ARDS), the early application of APRV was able to prevent the development of ARDS. Recent experimental evidence has suggested mechanisms by which APRV, using the time-controlled adaptive ventilation (TCAV) protocol, may reduce lung injury, including: 1) an improvement in alveolar recruitment and homogeneity; 2) reduction in alveolar and alveolar duc...
Source: European Respiratory Review - April 17, 2019 Category: Respiratory Medicine Authors: Kollisch-Singule, M., Andrews, P., Satalin, J., Gatto, L. A., Nieman, G. F., Habashi, N. M. Tags: Acute lung injury and critical care Review Source Type: research

Endobronchial valves for severe emphysema
The results of the randomised controlled trials investigating the bronchoscopic lung volume reduction treatment using endobronchial valves (EBV) are promising, and have led to their inclusion in treatment guidelines, US Food and Drug Administration approval and inclusion in routine care in an increasing number of countries. The one-way valve treatment has advanced and is now a regular treatment option. However, this new phase will lead to new challenges in terms of implementation. We believe that key issues in future research concern advanced patient selection, improved methods for target lobe selection, increased knowledg...
Source: European Respiratory Review - April 17, 2019 Category: Respiratory Medicine Authors: Hartman, J. E., Vanfleteren, L. E. G. W., van Rikxoort, E. M., Klooster, K., Slebos, D.-J. Tags: COPD and smoking Review Source Type: research

Dismantling the pathophysiology of asthma using imaging
Asthma remains an important disease worldwide, causing high burden to patients and healthcare systems and presenting a need for better management and ultimately prevention and cure. Asthma is a very heterogeneous condition, with many different pathophysiological processes. Better measurement of those pathophysiological processes are needed to better phenotype disease, and to go beyond the current, highly limited measurements that are currently used: spirometry and symptoms. Sophisticated three-dimensional lung imaging using computed tomography and ventilation imaging (single photon emission computed tomography and positron...
Source: European Respiratory Review - April 17, 2019 Category: Respiratory Medicine Authors: King, G. G., Farrow, C. E., Chapman, D. G. Tags: Lung imaging, Asthma and allergy Dismantling Airway Disease Source Type: research

Dismantling airway disease with the use of new pulmonary function indices
We describe their ability to assess detailed pulmonary mechanics for diagnostic and management purposes including response to bronchodilation and other treatments, relationship with symptoms, evaluation of acute exacerbations and recovery, and telemonitoring. The current limitations of both tests, as well as open questions/directions for further research, are also discussed. (Source: European Respiratory Review)
Source: European Respiratory Review - March 27, 2019 Category: Respiratory Medicine Authors: Zimmermann, S. C., Tonga, K. O., Thamrin, C. Tags: COPD and smoking, Lung imaging, Asthma and allergy Dismantling Airway Disease Source Type: research

Cancer-associated thrombosis: the when, how and why
Cancer-associated thrombosis (CAT) is a condition in which relevance has been increasingly recognised both for physicians that deal with venous thromboembolism (VTE) and for oncologists. It is currently estimated that the annual incidence of VTE in patients with cancer is 0.5% compared to 0.1% in the general population. Active cancer accounts for 20% of the overall incidence of VTE. Of note, VTE is the second most prevalent cause of death in cancer, second only to the progression of the disease, and cancer is the most prevalent cause of deaths in VTE patients. Nevertheless, CAT presents several peculiarities that distingui...
Source: European Respiratory Review - March 27, 2019 Category: Respiratory Medicine Authors: Fernandes, C. J., Morinaga, L. T. K., Alves, J. L., Castro, M. A., Calderaro, D., Jardim, C. V. P., Souza, R. Tags: Pulmonary vascular disease Review Source Type: research

Receptor for advanced glycation end-products and environmental exposure related obstructive airways disease: a systematic review
Conclusion RAGE is a mediator of inflammation associated end-organ dysfunction such as obstructive airways disease. Soluble RAGE, a decoy receptor, may have a protective effect in some pulmonary processes. Overall, RAGE is biologically relevant in environmental exposure associated lung disease. Future investigations should focus on further understanding the role and therapeutic potential of RAGE in particulate matter exposure associated lung disease. (Source: European Respiratory Review)
Source: European Respiratory Review - March 27, 2019 Category: Respiratory Medicine Authors: Haider, S. H., Oskuei, A., Crowley, G., Kwon, S., Lam, R., Riggs, J., Mikhail, M., Talusan, A., Veerappan, A., Kim, J. S., Caraher, E. J., Nolan, A. Tags: Epidemiology, occupational and environmental lung disease Review Source Type: research

A systematic review of pharmacotherapeutic clinical trial end-points for bronchiectasis in adults
Bronchiectasis is an increasing clinical problem, but multiple recent clinical trials have failed to reach their primary end-point. Difficulties in achieving "positive" bronchiectasis trials is reflected in a lack of agreement from trialists and regulators on what are the optimal end-points. To evaluate the use of end-points in bronchiectasis trials, we conducted a systematic review of published bronchiectasis trials from 2008 to 2018 and extracted end-points used, definitions, methods of analysis and responsiveness. Our analysis shows that quality of life and exacerbation end-points are most frequently used. Tri...
Source: European Respiratory Review - March 14, 2019 Category: Respiratory Medicine Authors: Crichton, M. L., Aliberti, S., Chalmers, J. D. Tags: CF and non-CF bronchiectasis Review Source Type: research

Prioritising children and adolescents in the tuberculosis response of the WHO European Region
In 2017, in recognition of the challenges faced by Member States in managing childhood and adolescent tuberculosis (TB) at a country level, the WHO Regional Office for Europe held a Regional Consultation. In total, 35 countries participated in the consultations representing both high- and low-incidence Member States. Here, we provide an overview of the existing World Health Organization (WHO) documents and guidelines on childhood and adolescent TB and describe the outcomes of this regional meeting. National childhood and adolescent TB guidelines are available in 25% of Member States, while 33% reported that no such guideli...
Source: European Respiratory Review - March 14, 2019 Category: Respiratory Medicine Authors: Gröschel, M. I., van den Boom, M., Migliori, G. B., Dara, M. Tags: Respiratory infections and tuberculosis, Paediatric pulmonology Review Source Type: research

Obesity hypoventilation syndrome
(OHS) is defined as a combination of obesity (body mass index ≥30 kg·m–2), daytime hypercapnia (arterial carbon dioxide tension ≥45 mmHg) and sleep disordered breathing, after ruling out other disorders that may cause alveolar hypoventilation. OHS prevalence has been estimated to be ~0.4% of the adult population. OHS is typically diagnosed during an episode of acute-on-chronic hypercapnic respiratory failure or when symptoms lead to pulmonary or sleep consultation in stable conditions. The diagnosis is firmly established after arterial blood gases and a sleep study. The presence of daytime hyp...
Source: European Respiratory Review - March 14, 2019 Category: Respiratory Medicine Authors: Masa, J. F., Pepin, J.-L., Borel, J.-C., Mokhlesi, B., Murphy, P. B., Sanchez-Quiroga, M. A. Tags: Sleep medicine Sleep Disordered Breathing Source Type: research

Telemedicine in the diagnosis and treatment of sleep apnoea
Telemedicine (TM) is a current tool in the landscape of medicine. It helps to address public health challenges such as increases in chronic disease in an ageing society and the associated burden in healthcare costs. Sleep TM refers to patient data exchange with the purpose of enhancing disease management. Obstructive sleep apnoea (OSA) syndrome is a chronic disorder associated with a significant morbidity, mainly cardiometabolic, and mortality. Obtaining adequate compliance to continuous positive airway pressure (CPAP) remains the greatest challenge related to OSA treatment, and the adoption of TM to support OSA management...
Source: European Respiratory Review - March 14, 2019 Category: Respiratory Medicine Authors: Bruyneel, M. Tags: Sleep medicine Sleep Disordered Breathing Source Type: research

Respiratory viral infection: a potential "missing link" in the pathogenesis of COPD
Chronic obstructive pulmonary disease (COPD) is currently the third most common cause of global mortality. Acute exacerbations of COPD frequently necessitate hospital admission to enable more intensive therapy, incurring significant healthcare costs. COPD exacerbations are also associated with accelerated lung function decline and increased risk of mortality. Until recently, bacterial pathogens were believed to be responsible for the majority of disease exacerbations. However, with the advent of culture-independent molecular diagnostic techniques it is now estimated that viruses are detected during half of all COPD exacerb...
Source: European Respiratory Review - March 14, 2019 Category: Respiratory Medicine Authors: Linden, D., Guo-Parke, H., Coyle, P. V., Fairley, D., McAuley, D. F., Taggart, C. C., Kidney, J. Tags: COPD and smoking Review Source Type: research

Fibrosing interstitial lung diseases: knowns and unknowns
Patients with certain types of fibrosing interstitial lung disease (ILD) are at risk of developing a progressive phenotype characterised by self-sustaining fibrosis, decline in lung function, worsening quality of life, and early mortality. It has been proposed that such progressive fibrosing ILDs, which show commonalities in clinical behaviour and in the pathogenetic mechanisms that drive progressive fibrosis, may be "lumped" together for the purposes of clinical research and, potentially, for treatment. At present, no drugs are approved for the treatment of ILDs other than nintedanib and pirfenidone for the trea...
Source: European Respiratory Review - February 27, 2019 Category: Respiratory Medicine Authors: Cottin, V., Wollin, L., Fischer, A., Quaresma, M., Stowasser, S., Harari, S. Tags: Interstitial and orphan lung disease Review Source Type: research

Women and COPD: do we need more evidence?
The increasingly female face of chronic obstructive pulmonary disease (COPD) prevalence among women has equalled that of men since 2008, due in part to increased tobacco use among women worldwide and exposure to biomass fuels. This finding is supported by a number of characteristics. There is evidence of susceptibility to smoking and other airborne contaminants, along with epidemiological and phenotypic manifestations. COPD has thus become the leading cause of death in women in the USA. The clinical presentation is characterised by increasingly pronounced dyspnoea with a marked tendency towards anxiety and depression, unde...
Source: European Respiratory Review - February 27, 2019 Category: Respiratory Medicine Authors: Gut-Gobert, C., Cavailles, A., Dixmier, A., Guillot, S., Jouneau, S., Leroyer, C., Marchand-Adam, S., Marquette, D., Meurice, J.-C., Desvigne, N., Morel, H., Person-Tacnet, C., Raherison, C. Tags: COPD and smoking Review Source Type: research

European Respiratory Review, list of peer reviewers 2018
The European Respiratory Review is voluntarily reviewed. We are most grateful to the hard work and dedication of those listed below, who reviewed articles for the ERR in 2018 (Source: European Respiratory Review)
Source: European Respiratory Review - February 6, 2019 Category: Respiratory Medicine Tags: Acknowledgement Source Type: research

The European Respiratory Review: continuing the success and targeting new challenges
The European Respiratory Review (ERR) is now over 20 years old, and during those years it has become a source of current and relevant information in our discipline. The overall number of visits to the ERR website and the number of downloaded review articles is constantly increasing, not only in Europe but all over the world, with more than 800 000 visits and 2.75 million HTML/PDF downloads in 2018. The ERR is already included in many international indexes and databases, and an important landmark was achieved in November 2018 when the ERR was accepted for indexing by Clarivate Analytics in the Science Citation Index Ex...
Source: European Respiratory Review - February 6, 2019 Category: Respiratory Medicine Authors: Adir, Y. Tags: Editorial Source Type: research

Tumoral pulmonary hypertension
This article will describe subtypes of tumoral PH, their pathophysiology, investigation and management options in turn. (Source: European Respiratory Review)
Source: European Respiratory Review - February 6, 2019 Category: Respiratory Medicine Authors: Price, L. C., Seckl, M. J., Dorfmüller, P., Wort, S. J. Tags: Pulmonary vascular disease Group 5 Pulmonary Hypertension Source Type: research

Beyond idiopathic pulmonary fibrosis: the world of progressive-fibrosing interstitial lung disease
In this issue, the European Respiratory Review publishes a series of articles on fibrosing interstitial lung diseases (ILDs) that present with a progressive phenotype. Progressive-fibrosing ILD (PF-ILD) is a terminology recently used to describe these patients. In addition to patients with idiopathic pulmonary fibrosis (IPF), there is a large and more diverse group of patients who develop PF-ILD. These patients are suffering from a disorder that shares a number of clinical features with IPF including: increasing respiratory symptoms; decline of the physiological functions; limited response to immunomodulatory therapy; prem...
Source: European Respiratory Review - December 21, 2018 Category: Respiratory Medicine Authors: Harari, S. Tags: Interstitial and orphan lung disease Editorial Source Type: research

Healthcare utilisation and costs in the diagnosis and treatment of progressive-fibrosing interstitial lung diseases
There are over 200 interstitial lung diseases (ILDs). In addition to patients with idiopathic pulmonary fibrosis (IPF), a percentage of patients with other ILDs also develop progressive fibrosis of the lung during their disease course. Patients with progressive-fibrosing ILDs may show limited response to immunomodulatory therapy, worsening symptoms and lung function and, ultimately, early mortality. There are few data for ILDs that may present a progressive fibrosing phenotype specifically, but we believe the burden and healthcare costs associated with these conditions may be comparable to those reported in IPF. This revie...
Source: European Respiratory Review - December 21, 2018 Category: Respiratory Medicine Authors: Holtze, C., Flaherty, K., Kreuter, M., Luppi, F., Moua, T., Vancheri, C., Scholand, M. B. Tags: Interstitial and orphan lung disease Review Source Type: research

The epidemiology of idiopathic pulmonary fibrosis and interstitial lung diseases at risk of a progressive-fibrosing phenotype
The availability of epidemiological data relating to interstitial lung diseases (ILDs) has increased over recent years, but information on the prevalence and incidence of ILDs of different aetiologies remains limited. Despite global distribution, the proportion of patients who develop a progressive phenotype across different ILDs is not well known. Disease behaviour is well documented in idiopathic pulmonary fibrosis but idiosyncratic in other ILDs that may present a progressive fibrosing phenotype. Possible reasons may include the heterogeneous nature of the aetiology, the complexity of diagnosis (and subsequent documenta...
Source: European Respiratory Review - December 21, 2018 Category: Respiratory Medicine Authors: Olson, A. L., Gifford, A. H., Inase, N., Fernandez Perez, E. R., Suda, T. Tags: Interstitial and orphan lung disease Review Source Type: research

Presentation, diagnosis and clinical course of the spectrum of progressive-fibrosing interstitial lung diseases
Although these conditions are rare, a proportion of patients with interstitial lung diseases (ILDs) may develop a progressive-fibrosing phenotype. Progressive fibrosis is associated with worsening respiratory symptoms, lung function decline, limited response to immunomodulatory therapies, decreased quality of life and, potentially, early death. Idiopathic pulmonary fibrosis may be regarded as a model for other progressive-fibrosing ILDs. Here we focus on other ILDs that may present a progressive-fibrosing phenotype, namely idiopathic nonspecific interstitial pneumonia, unclassifiable idiopathic interstitial pneumonia, conn...
Source: European Respiratory Review - December 21, 2018 Category: Respiratory Medicine Authors: Cottin, V., Hirani, N. A., Hotchkin, D. L., Nambiar, A. M., Ogura, T., Otaola, M., Skowasch, D., Park, J. S., Poonyagariyagorn, H. K., Wuyts, W., Wells, A. U. Tags: Interstitial and orphan lung disease Review Source Type: research

Patients' perceptions and patient-reported outcomes in progressive-fibrosing interstitial lung diseases
The effects of interstitial lung disease (ILD) create a significant burden on patients, unsettling almost every domain of their lives, disrupting their physical and emotional well-being and impairing their quality of life (QoL). Because many ILDs are incurable, and there are limited reliably-effective, life-prolonging treatment options available, the focus of many therapeutic interventions has been on improving or maintaining how patients with ILD feel and function, and by extension, their QoL. Such patient-centred outcomes are best assessed by patients themselves through tools that capture their perceptions, which inheren...
Source: European Respiratory Review - December 21, 2018 Category: Respiratory Medicine Authors: Swigris, J. J., Brown, K. K., Abdulqawi, R., Buch, K., Dilling, D. F., Koschel, D., Thavarajah, K., Tomic, R., Inoue, Y. Tags: Interstitial and orphan lung disease Review Source Type: research

Pharmacological management of progressive-fibrosing interstitial lung diseases: a review of the current evidence
A proportion of patients with interstitial lung diseases (ILDs) are at risk of developing a progressive-fibrosing phenotype, which is associated with a deterioration in lung function and early mortality. In addition to idiopathic pulmonary fibrosis (IPF), fibrosing ILDs that may present a progressive phenotype include idiopathic nonspecific interstitial pneumonia, connective tissue disease-associated ILDs, hypersensitivity pneumonitis, unclassifiable idiopathic interstitial pneumonia, ILDs related to other occupational exposures and sarcoidosis. Corticosteroids and/or immunosuppressive therapies are sometimes prescribed to...
Source: European Respiratory Review - December 21, 2018 Category: Respiratory Medicine Authors: Richeldi, L., Varone, F., Bergna, M., de Andrade, J., Falk, J., Hallowell, R., Jouneau, S., Kondoh, Y., Morrow, L., Randerath, W., Strek, M., Tabaj, G. Tags: Interstitial and orphan lung disease Review Source Type: research

Role of imaging in progressive-fibrosing interstitial lung diseases
Imaging techniques are an essential component of the diagnostic process for interstitial lung diseases (ILDs). Chest radiography is frequently the initial indicator of an ILD, and comparison of radiographs taken at different time points can show the rate of disease progression. However, radiography provides only limited specificity and sensitivity and is primarily used to rule out other diseases, such as left heart failure. High-resolution computed tomography (HRCT) is a more sensitive method and is considered central in the diagnosis of ILDs. Abnormalities observed on HRCT can help identify specific ILDs. HRCT also can be...
Source: European Respiratory Review - December 21, 2018 Category: Respiratory Medicine Authors: Walsh, S. L. F., Devaraj, A., Enghelmayer, J. I., Kishi, K., Silva, R. S., Patel, N., Rossman, M. D., Valenzuela, C., Vancheri, C. Tags: Interstitial and orphan lung disease Review Source Type: research

Acute exacerbations of progressive-fibrosing interstitial lung diseases
Acute exacerbation of interstitial lung disease (ILD) is associated with a poor prognosis and high mortality. Numerous studies have documented acute exacerbation in idiopathic pulmonary fibrosis (IPF), but less is known about these events in other ILDs that may present a progressive-fibrosing phenotype. We propose defining acute exacerbation as an acute, clinically significant respiratory deterioration, typically less than 1 month in duration, together with computerised tomography imaging showing new bilateral glass opacity and/or consolidation superimposed on a background pattern consistent with fibrosing ILDs. Drawi...
Source: European Respiratory Review - December 21, 2018 Category: Respiratory Medicine Authors: Kolb, M., Bondue, B., Pesci, A., Miyazaki, Y., Song, J. W., Bhatt, N. Y., Huggins, J. T., Oldham, J. M., Padilla, M. L., Roman, J., Shapera, S. Tags: Interstitial and orphan lung disease Review Source Type: research

The changing face of the European Respiratory Review
Three years have now passed since I took up the post of Chief Editor of the European Respiratory Review (ERR), one of the best experiences in my professional career. When Vincent Cottin handed over the helm to me, the ERR was already in excellent condition after his brilliant leadership and that of his predecessor Marc Humbert. Throughout my term of office I have tried to further open up the ERR to professionals from other areas of medicine and to involve countries that in the past have been less sensitive to our publication; all this thanks to an editorial board made up of junior and senior experts with very different ski...
Source: European Respiratory Review - December 19, 2018 Category: Respiratory Medicine Authors: Harari, S. Tags: Editorial Source Type: research

Cyst-related primary lung malignancies: an important and relatively unknown imaging appearance of (early) lung cancer
It is well known that lung cancer can manifest itself in imaging as solid and subsolid nodules or masses. However, in this era of increased computed tomography use another morphological computed tomography appearance of lung cancer is increasingly being recognised, presenting as a malignancy in relation to cystic airspaces. Despite the fact that it seems to be a relatively common finding in daily practice, literature on this entity is scarce and presumably the overall awareness is limited. This can lead to misinterpretation and delay in diagnosis and, therefore, increased awareness is urgently needed. This review aims to i...
Source: European Respiratory Review - December 19, 2018 Category: Respiratory Medicine Authors: Mets, O. M., Schaefer-Prokop, C. M., de Jong, P. A. Tags: Lung imaging, Lung cancer Review Source Type: research

Palliative care in pulmonary arterial hypertension: an underutilised treatment
Pulmonary arterial hypertension (PAH) is a condition characterised by increased pulmonary vascular resistance which can lead to right heart failure and premature death. It imposes a significant burden on patients' lives, affecting their physical, emotional and social wellbeing. Pharmacological therapies are the mainstay of treatment; while they are not curative, they can alleviate patient suffering, improve quality of life and delay disease progression. Despite these therapies, disease progresses in a significant number of patients, who are faced with the debilitating symptoms of PAH and treatment adverse effects. Palliati...
Source: European Respiratory Review - December 19, 2018 Category: Respiratory Medicine Authors: Khirfan, G., Tonelli, A. R., Ramsey, J., Sahay, S. Tags: Review Source Type: research

The use of chest magnetic resonance imaging in interstitial lung disease: a systematic review
Thin-slices multi-detector computed tomography (MDCT) plays a key role in the differential diagnosis of interstitial lung disease (ILD). However, thin-slices MDCT has a limited ability to detect active inflammation, which is an important target of newly developed ILD drug therapy. Magnetic resonance imaging (MRI), thanks to its multi-parameter capability, provides better tissue characterisation than thin-slices MDCT. Our aim was to summarise the current status of MRI applications in ILD and to propose an ILD-MRI protocol. A systematic literature search was conducted for relevant studies on chest MRI in patients with ILD. W...
Source: European Respiratory Review - December 19, 2018 Category: Respiratory Medicine Authors: Romei, C., Turturici, L., Tavanti, L., Miedema, J., Fiorini, S., Marletta, M., Wielopolski, P., Tiddens, H., Falaschi, F., Ciet, P. Tags: Interstitial and orphan lung disease, Lung imaging Review Source Type: research

Fatal recurrent VTE after anticoagulant treatment for unprovoked VTE: a systematic review
Current guidelines recommend long-term anticoagulant therapy in patients with unprovoked venous thromboembolism (VTE). The risk of fatal recurrent VTE after treatment discontinuation (versus that of fatal bleeding during anticoagulation) is of particular relevance in the decision to continue or stop anticoagulation after the first 3 months. Our primary aim was to provide a point-estimate of the yearly rate of fatal recurrent VTE and VTE case-fatality rate in patients with unprovoked VTE after anticoagulation cessation. Data were extracted from both randomised controlled trials and observational studies published befor...
Source: European Respiratory Review - November 28, 2018 Category: Respiratory Medicine Authors: van der Wall, S. J., van der Pol, L. M., Ende-Verhaar, Y. M., Cannegieter, S. C., Schulman, S., Prandoni, P., Rodger, M., Huisman, M. V., Klok, F. A. Tags: Lung structure and function Review Source Type: research

Sustained inflations and avoiding mechanical ventilation to prevent death or bronchopulmonary dysplasia: a meta-analysis
Sustained inflations and avoidance of endotracheal mechanical ventilation (eMV) are delivery room interventions aimed at preventing bronchopulmonary dysplasia (BPD). Their effectiveness is the subject of the present meta-analysis. The databases MEDLINE, EMBASE and CENTRAL were searched for randomised controlled trials (RCTs) of preterm infants that compared: 1) sustained inflations with intermittent positive-pressure ventilation; and 2) a non-intubated strategy of respiratory support with one that prescribed eMV at an earlier stage. Data extraction and analysis followed the standard methods of the Cochrane Collaboration. T...
Source: European Respiratory Review - November 28, 2018 Category: Respiratory Medicine Authors: Fischer, H. S., Schmölzer, G. M., Cheung, P.-Y., Bührer, C. Tags: Respiratory clinical practice, Paediatric pulmonology Review Source Type: research

"Cardiovascular disease and COPD: dangerous liaisons?" Klaus F. Rabe, John R. Hurst and Samy Suissa. Eur Respir Rev 2018; 27: 180057.
(Source: European Respiratory Review)
Source: European Respiratory Review - November 21, 2018 Category: Respiratory Medicine Tags: Erratum Source Type: research

"Cardiovascular disease and COPD: dangerous liaisons." Klaus F. Rabe, John R. Hurst and Samy Suissa. Eur Respir Rev 2018; 27: 180057.
(Source: European Respiratory Review)
Source: European Respiratory Review - November 21, 2018 Category: Respiratory Medicine Tags: Erratum Source Type: research

Risk factors for mortality and mortality rates in interstitial lung disease patients in the intensive care unit
Data on interstitial lung disease (ILD) outcomes in the intensive care unit (ICU) is of limited value due to population heterogeneity. The aim of this study was to examine risk factors for mortality and ILD mortality rates in the ICU. We performed a systematic review using five databases. 50 studies were identified and 34 were included: 17 studies on various aetiologies of ILD (mixed-ILD) and 17 on idiopathic pulmonary fibrosis (IPF). In mixed-ILD, elevated APACHE score, hypoxaemia and mechanical ventilation are risk factors for mortality. No increased mortality was found with steroid use. Evidence is inconclusive on advan...
Source: European Respiratory Review - November 21, 2018 Category: Respiratory Medicine Authors: Huapaya, J. A., Wilfong, E. M., Harden, C. T., Brower, R. G., Danoff, S. K. Tags: Interstitial and orphan lung disease Review Source Type: research

Current opinions for the management of asthma associated with ear, nose and throat comorbidities
Ear, nose and throat (ENT) comorbidities are common in patients with asthma and are frequently associated with poorer asthma outcomes. All these comorbidities are "treatable traits" in asthma. Identification and management of these disorders may spare medication usage and contribute to improved asthma control and quality of life, and a decrease in exacerbation rates. This review summarises recent data about the prevalence, clinical impact and treatment effects of ENT comorbidities in asthma including allergic rhinitis, chronic rhinosinusitis with and without nasal polyposis, aspirin-exacerbated respiratory diseas...
Source: European Respiratory Review - November 21, 2018 Category: Respiratory Medicine Authors: Tiotiu, A., Plavec, D., Novakova, S., Mihaicuta, S., Novakova, P., Labor, M., Bikov, A. Tags: Asthma and allergy Review Source Type: research

Cardiovascular disease and COPD: dangerous liaisons?
Chronic obstructive pulmonary disease (COPD) and cardiovascular disease (CVD) frequently occur together and their coexistence is associated with worse outcomes than either condition alone. Pathophysiological links between COPD and CVD include lung hyperinflation, systemic inflammation and COPD exacerbations. COPD treatments may produce beneficial cardiovascular (CV) effects, such as long-acting bronchodilators, which are associated with improvements in arterial stiffness, pulmonary vasoconstriction, and cardiac function. However, data are limited regarding whether these translate into benefits in CV outcomes. Some studies ...
Source: European Respiratory Review - October 3, 2018 Category: Respiratory Medicine Authors: Rabe, K. F., Hurst, J. R., Suissa, S. Tags: COPD and smoking Review Source Type: research