Chronic hypersensitivity pneumonitis; an enigmatic and frequently fatal disease
In the current issue of the European Respiratory Review, Creamer and Barratt [1] review prognostic factors in chronic hypersensitivity pneumonitis (cHP). Their work provides a state-of-the-art overview on the topic; but in doing so, highlights many of the challenges confronting patients and clinicians dealing with this enigmatic condition. Many important knowledge gaps exist pertaining to cHP which impact everything from understanding of the mechanisms underpinning pathogenesis, disease natural history, optimal approaches to diagnosis, how to distinguish cHP from idiopathic pulmonary fibrosis (IPF) and best treatment. (Sou...
Source: European Respiratory Review - July 7, 2020 Category: Respiratory Medicine Authors: Maher, T. M. Tags: Interstitial and orphan lung disease Editorials Source Type: research

The ageing lung under stress
Healthy ageing of the lung involves structural changes but also numerous cell-intrinsic and cell-extrinsic alterations. Among them are the age-related decline in central cellular quality control mechanisms such as redox and protein homeostasis. In this review, we would like to provide a conceptual framework of how impaired stress responses in the ageing lung, as exemplified by dysfunctional redox and protein homeostasis, may contribute to onset and progression of COPD and idiopathic pulmonary fibrosis (IPF). We propose that age-related imbalanced redox and protein homeostasis acts, amongst others (e.g. cellular senescence)...
Source: European Respiratory Review - July 7, 2020 Category: Respiratory Medicine Authors: Korfei, M., MacKenzie, B., Meiners, S. Tags: Lung Science Conference Source Type: research

Ultra-rare disease: an European perspective
In the past few years, interest in rare diseases has grown, as demonstrated by the agendas of politicians and health authorities, but too little attention is still paid to ultra-rare diseases. Although no legal definition of an "ultra-rare" disease has yet been established, this subcategory was informally introduced by the National Institute for Health and Care Excellence for drugs with indications for diseases that have a prevalence
Source: European Respiratory Review - July 3, 2020 Category: Respiratory Medicine Authors: Harari, S., Humbert, M. Tags: Pulmonary vascular disease Editorials Source Type: research

The use of magnetic resonance imaging in pulmonary hypertension: why are we still waiting?
Pulmonary hypertension (PH) is a complex disease requiring a detailed assessment of haemodynamics, functional analysis and identification of the underlying cause. A typical patient with a suspicion of PH will undergo several noninvasive and invasive tests including right heart catheterisation before a definitive diagnosis can be made. Some of these tests, such as 6-min walking distance or N-terminal pro-brain natriuretic peptide can provide information on treatment response and prognosis. However, the extent of right ventricular (RV) adaptation to the increased afterload is the main determinant of outcome [1]. Because of t...
Source: European Respiratory Review - July 3, 2020 Category: Respiratory Medicine Authors: Wessels, J. N., de Man, F. S., Vonk Noordegraaf, A. Tags: Lung imaging, Pulmonary vascular disease Editorials Source Type: research

Therapeutic targeting of metabolic alterations in acute respiratory distress syndrome
Acute respiratory distress syndrome (ARDS) remains a significant source of mortality in critically ill patients. Characterised by acute, widespread alveolar inflammation and pulmonary oedema, its pathophysiological heterogeneity has meant that targeted treatments have remained elusive. Metabolomic analysis has made initial steps in characterising the underlying metabolic derangements of ARDS as an indicator of phenotypical class and has identified mitochondrial dysfunction as a potential therapeutic target. Mesenchymal stem cells and their derived extracellular vesicles have shown significant promise as potential therapies...
Source: European Respiratory Review - July 3, 2020 Category: Respiratory Medicine Authors: Robinson, M. J., Krasnodembskaya, A. D. Tags: Interstitial and orphan lung disease Lung Science Conference Source Type: research

Breathing fresh air into respiratory research with single-cell RNA sequencing
The complex cellular heterogeneity of the lung poses a unique challenge to researchers in the field. While the use of bulk RNA sequencing has become a ubiquitous technology in systems biology, the technique necessarily averages out individual contributions to the overall transcriptional landscape of a tissue. Single-cell RNA sequencing (scRNA-seq) provides a robust, unbiased survey of the transcriptome comparable to bulk RNA sequencing while preserving information on cellular heterogeneity. In just a few years since this technology was developed, scRNA-seq has already been adopted widely in respiratory research and has con...
Source: European Respiratory Review - July 3, 2020 Category: Respiratory Medicine Authors: Alexander, M. J., Budinger, G. R. S., Reyfman, P. A. Tags: Lung biology and experimental studies Reviews Source Type: research

Emerging role of cardiovascular magnetic resonance imaging in the management of pulmonary hypertension
Pulmonary hypertension (PH) is a clinical condition characterised by elevation of pulmonary arterial pressure (PAP) above normal range due to various aetiologies. While cardiac right-heart catheterisation (RHC) remains the gold standard and mandatory for establishing the diagnosis of PH, noninvasive imaging of the heart plays a central role in the diagnosis and management of all forms of PH. Although Doppler echocardiography (ECHO) can measure a range of haemodynamic and anatomical variables, it has limited utility for visualisation of the pulmonary artery and, oftentimes, the right ventricle. Cardiovascular magnetic reson...
Source: European Respiratory Review - July 3, 2020 Category: Respiratory Medicine Authors: Aryal, S. R., Sharifov, O. F., Lloyd, S. G. Tags: Lung imaging, Pulmonary vascular disease Reviews Source Type: research

Defining a role for exercise training in the management of asthma
The prevalence of asthma remains high worldwide, with increasing awareness of the morbidity and mortality from asthma in low-income countries. In the UK, despite the development of biological treatments, many patients remain suboptimally controlled, and mortality rates have been static for decades. Therefore, new approaches are needed to treat asthma that are scalable at minimal cost. Exercise immunology is an expanding field, and there is growing evidence that exercise can modulate inflammatory and immune processes in asthma. Whilst exercise is encouraged in current treatment guidelines, there are no specific recommendati...
Source: European Respiratory Review - July 3, 2020 Category: Respiratory Medicine Authors: Freeman, A. T., Staples, K. J., Wilkinson, T. M. A. Tags: Asthma and allergy Reviews Source Type: research

Desquamative interstitial pneumonia: still orphan and not always benign
Desquamative interstitial pneumonia (DIP) was originally described by Liebow et al. [1] in 1965, and so named because of the observation of cells filling the alveolar spaces and the belief that this feature was due to desquamation of alveolar epithelial cells. It has since been recognised that the dominant histologic feature of DIP represents accumulation of intra-alveolar macrophages, and sometimes of giant cells [2]. Although the more accurate terminology of "alveolar macrophage pneumonia" has been proposed, the term DIP has persisted [3]. (Source: European Respiratory Review)
Source: European Respiratory Review - June 23, 2020 Category: Respiratory Medicine Authors: Cottin, V. Tags: Pulmonary vascular disease Editorials Source Type: research

Desquamative interstitial pneumonia: a systematic review of its features and outcomes
Conclusion DIP remains an uncommon disease, frequently but not always related to smoking or other exposures. Furthermore, DIP behaves as a progressive disease more often than generally thought, possibly associated with different underlying aetiology. (Source: European Respiratory Review)
Source: European Respiratory Review - June 23, 2020 Category: Respiratory Medicine Authors: Hellemons, M. E., Moor, C. C., von der Thüsen, J., Rossius, M., Odink, A., Thorgersen, L. H., Verschakelen, J., Wuyts, W., Wijsenbeek, M. S., Bendstrup, E. Tags: Pulmonary vascular disease Reviews Source Type: research

COPD and acute myocardial infarction
COPD is strongly associated with cardiovascular disease, in particular acute myocardial infarction (AMI). Besides shared risk factors, COPD-related factors, such as systemic inflammation and hypoxia, underlie the pathophysiological interaction between COPD and AMI. The prevalence of COPD amongst AMI populations ranges from 7% to 30%, which is possibly even an underestimation due to underdiagnoses of COPD in general. Following the acute event, patients with COPD have an increased risk of mortality, heart failure and arrhythmias during follow-up. Adequate risk stratification can be performed using various imaging techniques,...
Source: European Respiratory Review - June 23, 2020 Category: Respiratory Medicine Authors: Goedemans, L., Bax, J. J., Delgado, V. Tags: COPD and smoking Reviews Source Type: research

Noninfectious pulmonary complications of haematopoietic stem cell transplantation
Haematopoietic stem cell transplantation (HSCT) is an established treatment for a variety of malignant and nonmalignant conditions. Pulmonary complications, both infectious and noninfectious, are a major cause of morbidity and mortality in patients who undergo HSCT. Recent advances in prophylaxis and treatment of infectious complications has increased the significance of noninfectious pulmonary conditions. Acute lung injury associated with idiopathic pneumonia syndrome remains a major acute complication with high morbidity and mortality. On the other hand, bronchiolitis obliterans syndrome is the most challenging chronic p...
Source: European Respiratory Review - June 23, 2020 Category: Respiratory Medicine Authors: Haider, S., Durairajan, N., Soubani, A. O. Tags: Pulmonary pharmacology and therapeutics Reviews Source Type: research

The radiological diagnosis of bronchiectasis: what's in a name?
Diagnosis of bronchiectasis is usually made using chest computed tomography (CT) scan, the current gold standard method. A bronchiectatic airway can show abnormal widening and thickening of its airway wall. In addition, it can show an irregular wall and lack of tapering, and/or can be visible in the periphery of the lung. Its diagnosis is still largely expert based. More recently, it has become clear that airway dimensions on CT and therefore the diagnosis of bronchiectasis are highly dependent on lung volume. Hence, control of lung volume is required during CT acquisition to standardise the evaluation of airways. Automate...
Source: European Respiratory Review - June 17, 2020 Category: Respiratory Medicine Authors: Tiddens, H. A. W. M., Meerburg, J. J., van der Eerden, M. M., Ciet, P. Tags: CF and non-CF bronchiectasis Controversies in bronchiectasis Source Type: research

Interventional pulmonology: between ambition and wisdom
It is always useful to remember the past in order to understand and appreciate the present. In the past decades, we have experienced extraordinary changes and innovations in our discipline: interventional pulmonology. Almost all aspects of this sub-specialty have been transformed. Diagnostic bronchoscopy, previously limited to central airways, can now assess the periphery of the lungs thanks to new smaller bronchoscopes and innovative guiding techniques (virtual bronchoscopy, electromagnetic navigation, radial ultrasound, cone-bean computed tomography (CT)), allowing us to reach previously inaccessible nodules [1]. Mediast...
Source: European Respiratory Review - June 16, 2020 Category: Respiratory Medicine Authors: Dutau, H., Feller-Kopman, D. Tags: Editorials Source Type: research

Bronchoscopic intratumoural therapies for non-small cell lung cancer
The past decade has brought remarkable improvements in the treatment of non-small cell lung cancer (NSCLC) with novel therapies, such as immune checkpoint inhibitors, although response rates remain suboptimal. Direct intratumoural injection of therapeutic agents via bronchoscopic approaches poses the unique ability to directly target the tumour microenvironment and offers several theoretical advantages over systemic delivery including decreased toxicity. Increases in understanding of the tumour microenvironment and cancer immunology have identified many potential options for intratumoural therapy, especially combination im...
Source: European Respiratory Review - June 16, 2020 Category: Respiratory Medicine Authors: DeMaio, A., Sterman, D. Tags: Respiratory clinical practice, Lung cancer Interventional pulmonology Source Type: research

CFTR targeted therapies: recent advances in cystic fibrosis and possibilities in other diseases of the airways
Cystic fibrosis transmembrane conductance regulator (CFTR) is an ion transporter that regulates mucus hydration, viscosity and acidity of the airway epithelial surface. Genetic defects in CFTR impair regulation of mucus homeostasis, causing severe defects of mucociliary clearance as seen in cystic fibrosis. Recent work has established that CFTR dysfunction can be acquired in chronic obstructive pulmonary disease (COPD) and may also contribute to other diseases that share clinical features of cystic fibrosis, such as asthma, allergic bronchopulmonary aspergillosis and bronchiectasis. Protean causes of CFTR dysfunction have ...
Source: European Respiratory Review - June 16, 2020 Category: Respiratory Medicine Authors: Patel, S. D., Bono, T. R., Rowe, S. M., Solomon, G. M. Tags: CF and non-CF bronchiectasis Controversies in bronchiectasis Source Type: research

Preventing readmissions of COPD patients: more prospective studies are needed
Fortunately, less than one-third of patients with COPD experience frequent exacerbations [1]; however, these patients are the ones with a worse prognosis and the most challenging management. Particularly, those who experience severe exacerbations that require hospital admission are at increased risk of mortality [2], and therefore prevention of exacerbations in general and admissions in particular is one of the main objectives of COPD treatment [3]. (Source: European Respiratory Review)
Source: European Respiratory Review - June 3, 2020 Category: Respiratory Medicine Authors: Nunez, A., Miravitlles, M. Tags: COPD and smoking Editorial Source Type: research

Risk factors for all-cause hospital readmission following exacerbation of COPD: a systematic review and meta-analysis
Conclusions Comorbidities, previous exacerbations and hospitalisation, and increased length of stay were significant risk factors for 30- and 90-day all-cause readmission after an index hospitalisation with an exacerbation of COPD. (Source: European Respiratory Review)
Source: European Respiratory Review - June 3, 2020 Category: Respiratory Medicine Authors: Alqahtani, J. S., Njoku, C. M., Bereznicki, B., Wimmer, B. C., Peterson, G. M., Kinsman, L., Aldabayan, Y. S., Alrajeh, A. M., Aldhahir, A. M., Mandal, S., Hurst, J. R. Tags: COPD and smoking Reviews Source Type: research

Minimal clinically important difference for asthma endpoints: an expert consensus report
Minimal clinically important difference (MCID) can be defined as the smallest change or difference in an outcome measure that is perceived as beneficial and would lead to a change in the patient's medical management. The aim of the current expert consensus report is to provide a "state-of-the-art" review of the currently available literature evidence about MCID for end-points to monitor asthma control, in order to facilitate optimal disease management and identify unmet needs in the field to guide future research. A series of MCID cut-offs are currently available in literature and validated among populations of a...
Source: European Respiratory Review - June 3, 2020 Category: Respiratory Medicine Authors: Bonini, M., Di Paolo, M., Bagnasco, D., Baiardini, I., Braido, F., Caminati, M., Carpagnano, E., Contoli, M., Corsico, A., Del Giacco, S., Heffler, E., Lombardi, C., Menichini, I., Milanese, M., Scichilone, N., Senna, G., Canonica, G. W. Tags: Asthma and allergy Reviews Source Type: research

Never-smoker African-American women suffer more from COPD compared with never-smoker Caucasian women
The article by Gut-Gobert et al. [1] presents an excellent review of evolving challenges in the management and prevention of COPD in women. (Source: European Respiratory Review)
Source: European Respiratory Review - May 27, 2020 Category: Respiratory Medicine Authors: Sumon, M., Mehari, A. Tags: COPD and smoking Correspondence Source Type: research

Pulmonary capillary haemangiomatosis: a distinct entity?
Pulmonary capillary haemangiomatosis (PCH) is a rare and incompletely understood histopathological finding characterised by abnormal capillary proliferation within the alveolar interstitium, which has long been noted to share many overlapping features with pulmonary veno-occlusive disease (PVOD). But are PCH and PVOD distinct entities that occur in isolation, or are they closely intertwined manifestations along a spectrum of the same disease? The classic clinical features of both PCH and PVOD include signs and symptoms related to pulmonary hypertension, hypoxaemia, markedly impaired diffusion capacity of the lung and abnor...
Source: European Respiratory Review - May 27, 2020 Category: Respiratory Medicine Authors: Weatherald, J., Dorfmüller, P., Perros, F., Ghigna, M.-R., Girerd, B., Humbert, M., Montani, D. Tags: Pulmonary vascular disease Ultra-rare lung disease Source Type: research

BNP/NT-proBNP in pulmonary arterial hypertension: time for point-of-care testing?
Despite the advent of new therapies and improved outcomes in patients with pulmonary arterial hypertension (PAH), it remains a life-shortening disease and the time to diagnosis remains unchanged. Strategies to improve outcomes are therefore currently focused on earlier diagnosis and a treatment approach aimed at moving patients with PAH into a category of low-risk of 1-year mortality. B-type natriuretic peptide (BNP; or brain natriuretic peptide) and N-terminal prohormone of BNP (NT-proBNP) are released from cardiac myocytes in response to mechanical load and wall stress. Elevated levels of BNP and NT-proBNP are incorporat...
Source: European Respiratory Review - May 15, 2020 Category: Respiratory Medicine Authors: Lewis, R. A., Durrington, C., Condliffe, R., Kiely, D. G. Tags: Pulmonary vascular disease Reviews Source Type: research

Prognostic factors in chronic hypersensitivity pneumonitis
Hypersensitivity pneumonitis (HP) is an immunologically mediated lung disease resulting from exposure to inhaled environmental antigens. Prognosis is variable, with a subset of patients developing progressive fibrosis leading to respiratory failure and death. Therefore, there is an urgent need to identify factors which predict prognosis and survival in patients with HP. We undertook a narrative review of existing evidence to identify prognostic factors in patients with chronic HP. Patient demographics, smoking history, extent of antigen exposure and comorbidities all have reported associations with disease outcome, and phy...
Source: European Respiratory Review - May 15, 2020 Category: Respiratory Medicine Authors: Creamer, A. W., Barratt, S. L. Tags: Interstitial and orphan lung disease Reviews Source Type: research

Measuring airway clearance outcomes in bronchiectasis: a review
While airway clearance techniques (ACTs) are recommended for individuals with bronchiectasis, many trials have demonstrated inconsistent benefits or failed to reach their primary outcome. This review determined the most common clinical and patient-reported outcome measures used to evaluate the efficacy of ACTs in bronchiectasis. A literature search of five databases using relevant keywords and filtering for studies published in English, up until the end of August 2019, was completed. Studies included randomised controlled trials, using crossover or any other trial design, and abstracts. Studies were included where the cont...
Source: European Respiratory Review - April 29, 2020 Category: Respiratory Medicine Authors: Franks, L. J., Walsh, J. R., Hall, K., Morris, N. R. Tags: CF and non-CF bronchiectasis Reviews Source Type: research

Chitotriosidase: a marker and modulator of lung disease
Chitotriosidase (CHIT1) is a highly conserved and regulated chitinase secreted by activated macrophages; it is a member of the 18-glycosylase family (GH18). CHIT1 is the most prominent chitinase in humans, can cleave chitin and participates in the body's immune response and is associated with inflammation, infection, tissue damage and remodelling processes. Recently, CHIT1 has been reported to be involved in the molecular pathogenesis of pulmonary fibrosis, bronchial asthma, COPD and pulmonary infections, shedding new light on the role of these proteins in lung pathophysiology. The potential roles of CHIT1 in lung diseases...
Source: European Respiratory Review - April 29, 2020 Category: Respiratory Medicine Authors: Chang, D., Sharma, L., Dela Cruz, C. S. Tags: Pulmonary pharmacology and therapeutics Reviews Source Type: research

Thoracic ultrasound in the modern management of pleural disease
Physician-led thoracic ultrasound (TUS) has substantially changed how respiratory disorders, and in particular pleural diseases, are managed. The use of TUS as a point-of-care test enables the respiratory physician to quickly and accurately diagnose pleural pathology and ensure safe access to the pleural space during thoracentesis or chest drain insertion. Competence in performing TUS is now an obligatory part of respiratory speciality training programmes in different parts of the world. Pleural physicians with higher levels of competence routinely use TUS during the planning and execution of more sophisticated diagnostic ...
Source: European Respiratory Review - April 29, 2020 Category: Respiratory Medicine Authors: Hassan, M., Mercer, R. M., Rahman, N. M. Tags: Lung imaging Reviews Source Type: research

The supportive care needs of people living with pulmonary fibrosis and their caregivers: a systematic review
Conclusion People with pulmonary fibrosis report many unmet needs for supportive care, particularly related to insufficient information and lack of psychosocial support. These data can inform the development of comprehensive care models for people with pulmonary fibrosis and their loved ones. (Source: European Respiratory Review)
Source: European Respiratory Review - April 29, 2020 Category: Respiratory Medicine Authors: Lee, J. Y. T., Tikellis, G., Corte, T. J., Goh, N. S., Keir, G. J., Spencer, L., Sandford, D., Khor, Y. H., Glaspole, I., Price, J., Hey-Cunningham, A. J., Maloney, J., Teoh, A. K. Y., Watson, A. L., Holland, A. E. Tags: Interstitial and orphan lung disease Reviews Source Type: research

Protecting healthcare workers from SARS-CoV-2 infection: practical indications
The World Health Organization has recently defined the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection a pandemic. The infection, that may cause a potentially very severe respiratory disease, now called coronavirus disease 2019 (COVID-19), has airborne transmission via droplets. The rate of transmission is quite high, higher than common influenza. Healthcare workers are at high risk of contracting the infection particularly when applying respiratory devices such as oxygen cannulas or noninvasive ventilation. The aim of this article is to provide evidence-based recommendations for the correct use of &...
Source: European Respiratory Review - April 3, 2020 Category: Respiratory Medicine Authors: Ferioli, M., Cisternino, C., Leo, V., Pisani, L., Palange, P., Nava, S. Tags: Respiratory infections and tuberculosis Frontiers in Clinical Practice Source Type: research

Short-course systemic corticosteroids in asthma: striking the balance between efficacy and safety
Short courses of systemic corticosteroids (SCS), both oral and injectable, are very effective for the resolution of acute asthma symptoms, including exacerbations. However, the benefits of SCS, even short courses, must be balanced against the impact of their side-effects. While the adverse consequences of long-term use are widely recognised, there appears to be a perception in the medical community that short courses of SCS are safe. Limited but growing evidence in the literature suggests that even very brief dosing periods (3–7 days) of SCS are enough to cause significantly negative outcomes for patients. Short...
Source: European Respiratory Review - April 3, 2020 Category: Respiratory Medicine Authors: Price, D., Castro, M., Bourdin, A., Fucile, S., Altman, P. Tags: Asthma and allergy Reviews Source Type: research

Modulator treatment for people with cystic fibrosis: moving in the right direction
In the past 10 years the incremental success of modulators of the cystic fibrosis transmembrane regulator (CFTR) protein have improved outcomes for people with cystic fibrosis (CF) [1]. Ivacaftor for individuals with sequence variants associated with class III mutations (e.g. G551D) and some other residual function mutations of the CFTR gene followed by lumacaftor/ivacaftor or tezacaftor/ivacaftor combinations in individuals homozygous for the F508del have achieved improvements in quality of life reduction, pulmonary exacerbations and variable improvement in forced expiratory volume in 1 s (FEV1). Highly effectiv...
Source: European Respiratory Review - March 20, 2020 Category: Respiratory Medicine Authors: Elborn, J. S. Tags: CF and non-CF bronchiectasis Editorial Source Type: research

Management of repository corticotrophin injection therapy for pulmonary sarcoidosis: a Delphi study
In patients treated with repository corticotrophin injection (RCI) for pulmonary sarcoidosis, effective management of adverse events may improve adherence. However, management of adverse events may be challenging due to limitations in real-world clinical experience with RCI and available published guidelines. We surveyed 12 physicians with a modified Delphi process using three questionnaires. Questionnaire 1 consisted of open-ended questions. Panellists' answers were developed into a series of statements for Questionnaires 2 and 3. In these, physicians rated their agreement with the statements using a Likert scale. Key con...
Source: European Respiratory Review - March 20, 2020 Category: Respiratory Medicine Authors: Rahaghi, F. F., Sweiss, N. J., Saketkoo, L. A., Scholand, M. B., Barney, J. B., Gerke, A. K., Lower, E. E., Mirsaeidi, M., O'Hare, L., Rumbak, M. J., Samavati, L., Baughman, R. P. Tags: Interstitial and orphan lung disease Reviews Source Type: research

Delphi consensus recommendations for a treatment algorithm in pulmonary sarcoidosis
Pulmonary sarcoidosis presents substantial management challenges, with limited evidence on effective therapies and phenotypes. In the absence of definitive evidence, expert consensus can supply clinically useful guidance in medicine. An international panel of 26 experts participated in a Delphi process to identify consensus on pharmacological management in sarcoidosis with the development of preliminary recommendations. The modified Delphi process used three rounds. The first round focused on qualitative data collection with open-ended questions to ensure comprehensive inclusion of expert concepts. Rounds 2 and 3 applied q...
Source: European Respiratory Review - March 20, 2020 Category: Respiratory Medicine Authors: Rahaghi, F. F., Baughman, R. P., Saketkoo, L. A., Sweiss, N. J., Barney, J. B., Birring, S. S., Costabel, U., Crouser, E. D., Drent, M., Gerke, A. K., Grutters, J. C., Hamzeh, N. Y., Huizar, I., Ennis James, W., Kalra, S., Kullberg, S., Li, H., Lower, E. Tags: Interstitial and orphan lung disease Reviews Source Type: research

Clinical phenotyping: role in treatment decisions in sarcoidosis
A variety of phenotypic categorisations have been developed for sarcoidosis. Phenotyping has been used for genetics studies and to guide treatment selection. The authors participated in a Delphi expert consensus panel to develop a proposed phenotype categorisation and treatment recommendations for pulmonary sarcoidosis patients. Panellists reached consensus that asymptomatic patients with normal pulmonary function and adenopathy alone or normal chest imaging do not require therapy, while symptomatic patients with impaired pulmonary function or infiltrates should be treated. The panel did not reach consensus on asymptomatic...
Source: European Respiratory Review - March 20, 2020 Category: Respiratory Medicine Authors: Baughman, R. P., Scholand, M. B., Rahaghi, F. F. Tags: Interstitial and orphan lung disease Reviews Source Type: research

Impact of CFTR modulator use on outcomes in people with severe cystic fibrosis lung disease
Drug compounds that augment the production and activity of the cystic fibrosis (CF) transmembrane regulator (CFTR) have revolutionised CF care. Many adults and some children with CF suffer advanced and severe lung disease or await lung transplantation. While the hope is that these drug compounds will prevent lung damage when started early in life, there is an ongoing need to care for people with advanced lung disease. The focus of this review is the accumulating data from clinical trials and case series regarding the benefits of CFTR modulator therapy in people with advanced pulmonary disease. We address the impact of trea...
Source: European Respiratory Review - March 20, 2020 Category: Respiratory Medicine Authors: Shteinberg, M., Taylor-Cousar, J. L. Tags: CF and non-CF bronchiectasis Reviews Source Type: research

Shared mechanisms of multimorbidity in COPD, atherosclerosis and type-2 diabetes: the neutrophil as a potential inflammatory target
Multimorbidity is increasingly common and current healthcare strategies are not always aligned to treat this complex burden of disease. COPD, type-2 diabetes mellitus (T2D) and cardiovascular disease, especially atherosclerosis, occur more frequently together than expected, even when risk factors such as smoking, obesity, inactivity and poverty are considered. This supports the possibility of unifying mechanisms that contribute to the pathogenesis or progression of each condition. Neutrophilic inflammation is causally associated with COPD, and increasingly recognised in the pathogenesis of atherosclerosis and T2D, potentia...
Source: European Respiratory Review - March 20, 2020 Category: Respiratory Medicine Authors: Hughes, M. J., McGettrick, H. M., Sapey, E. Tags: COPD and smoking Reviews Source Type: research

Natural enemy or friend? Pneumonia in the very elderly critically ill patient
Pneumonia has been a serious cause of morbidity and mortality for many years and, more than 100 years ago, Sir William Osler referred to it as "the natural enemy of the old man" [1]. Many studies have documented that elderly individuals have a higher frequency of illness, increased mortality and more subtle clinical features compared to younger populations. This too was known by Osler who commented that "it is not improbable that debility lowers the vitality and renders the individual susceptible" and when the illness occurs, it can be "without chill; the cough and expectoration are slight&quo...
Source: European Respiratory Review - February 19, 2020 Category: Respiratory Medicine Authors: Niederman, M. S. Tags: Epidemiology, occupational and environmental lung disease, Respiratory infections and tuberculosis Editorial Source Type: research

Community-acquired pneumonia in critically ill very old patients: a growing problem
Very old (aged ≥80 years) adults constitute an increasing proportion of the global population. Currently, this subgroup of patients represents an important percentage of patients admitted to the intensive care unit. Community-acquired pneumonia (CAP) frequently affects very old adults. However, there are no specific recommendations for the management of critically ill very old CAP patients. Multiple morbidities, polypharmacy, immunosenescence and frailty contribute to an increased risk of pneumonia in this population. CAP in critically ill very old patients is associated with higher short- and long-term mortality; ...
Source: European Respiratory Review - February 19, 2020 Category: Respiratory Medicine Authors: Cilloniz, C., Dominedo, C., Pericas, J. M., Rodriguez-Hurtado, D., Torres, A. Tags: Epidemiology, occupational and environmental lung disease, Respiratory infections and tuberculosis Reviews Source Type: research

"How can we minimise the use of regular oral corticosteroids in asthma?" Arnaud Bourdin, Ian Adcock, Patrick Berger, Philippe Bonniaud, Philippe Chanson, Cecile Chenivesse, Jacques de Blic, Antoine Deschildre, Philippe Devillier, Gilles Devouassoux, Alain Didier, Gilles Garcia, Antoine Magnan, Yan Martinat, Thierry Perez, Nicolas Roche, Camille Taille, Pierre Val and Pascal Chanez. Eur Respir Rev 2020; 29: 190085.
(Source: European Respiratory Review)
Source: European Respiratory Review - February 12, 2020 Category: Respiratory Medicine Tags: Author correction Source Type: research

"Is bronchiectasis really a disease?" Michal Shteinberg, Patrick A. Flume and James D. Chalmers. Eur Respir Rev 2020; 29: 190051.
(Source: European Respiratory Review)
Source: European Respiratory Review - February 12, 2020 Category: Respiratory Medicine Tags: Author correction Source Type: research

Subglottic secretion drainage for preventing ventilator-associated pneumonia: an overview of systematic reviews and an updated meta-analysis
Although several guidelines recommend subglottic secretion drainage as a strategy for prevention of ventilator-associated pneumonia (VAP), its use is not widespread. With the aim to assess the effectiveness of subglottic secretion drainage for preventing VAP and to improve other outcomes such as mortality, duration of mechanical ventilation and length of stay in the intensive care unit (ICU) or hospital, an electronic search of the Cochrane Library, MEDLINE, Web of Science and Embase was undertaken. Nine systematic reviews with meta-analysis (in the overview of reviews) and 20 randomised controlled trials (in the updated m...
Source: European Respiratory Review - February 12, 2020 Category: Respiratory Medicine Authors: Pozuelo-Carrascosa, D. P., Herraiz-Adillo, A., Alvarez-Bueno, C., Anon, J. M., Martinez-Vizcaino, V., Cavero-Redondo, I. Tags: Respiratory infections and tuberculosis Review Source Type: research

{alpha}1-Antitrypsin deficiency and chronic respiratory disorders
α1-antitrypsin deficiency (AATD) is a hereditary disorder associated with a risk of developing liver disease and pulmonary emphysema, and other chronic respiratory disorders (mainly asthma and bronchiectasis); Z variant is the commonest deficient variant of AAT. Determining AAT concentration in serum or plasma and identifying allelic variants by phenotyping or genotyping are fundamental in the diagnosis of AATD. Initial evaluation and annual follow-up measurement of lung function, including post-bronchodilator forced expiratory volume in 1 s and gas transfer inform on disease progression. Lung densitometry is th...
Source: European Respiratory Review - February 12, 2020 Category: Respiratory Medicine Authors: Cazzola, M., Stolz, D., Rogliani, P., Matera, M. G. Tags: COPD and smoking, Genetics Review Source Type: research

Current and future applications of liquid biopsy in nonsmall cell lung cancer from early to advanced stages
Liquid biopsy refers to the analysis of any tumour-derived material circulating in the blood or any other body fluid. This concept is particularly relevant in lung cancer as the tumour is often difficult to reach and may need an invasive and potentially harmful procedure. Moreover, the multitude of anticancer drugs and their sequential use underline the importance of conducting an iterative assessment of tumour biology. Liquid biopsies can noninvasively detect any targetable genomic alteration and guide corresponding targeted therapy, in addition to monitoring response to treatment and exploring the genetic changes at resi...
Source: European Respiratory Review - February 12, 2020 Category: Respiratory Medicine Authors: Guibert, N., Pradines, A., Favre, G., Mazieres, J. Tags: Lung cancer Review Source Type: research

Schistosomiasis-associated pulmonary arterial hypertension: a systematic review
We examined the hypothesis that there are pronounced similarities in pathophysiology, haemodynamics, and survival of Sch-PAH and idiopathic PAH (iPAH). This systematic review and meta-analysis was registered in the PROSPERO database (identifier CRD42018104066). A systematic search and review of the literature was performed according to PRISMA guidelines for studies published between 01 January 1990 and 29 June 2018. For Sch-PAH, 18 studies evaluating pathophysiological mechanisms, eight studies on haemodynamics (n=277), and three studies on survival (n=191) were identified. 16 clinical registries reporting data on haemodyn...
Source: European Respiratory Review - February 5, 2020 Category: Respiratory Medicine Authors: Knafl, D., Gerges, C., King, C. H., Humbert, M., Bustinduy, A. L. Tags: Pulmonary vascular disease Review Source Type: research

How can we minimise the use of regular oral corticosteroids in asthma?
Options to achieve oral corticosteroid (OCS)-sparing have been triggering increasing interest since the 1970s because of the side-effects of OCSs, and this has now become achievable with biologics. The Société de Pneumologie de Langue Francaise workshop on OCSs aimed to conduct a comprehensive review of the basics for OCS use in asthma and issue key research questions. Pharmacology and definition of regular use were reviewed by the first working group (WG1). WG2 examined whether regular OCS use is associated with T2 endotype. WG3 reported on the specificities of the paediatric area. Key "research stateme...
Source: European Respiratory Review - February 5, 2020 Category: Respiratory Medicine Authors: Bourdin, A., Adcock, I., Berger, P., Bonniaud, P., Chanson, P., Chenivesse, C., de Blic, J., Deschildre, A., Devilliers, P., Devouassoux, G., Didier, A., Garcia, G., Magnan, A., Martinat, Y., Perez, T., Roche, N., Taille, C., Val, P., Chanez, P. Tags: Asthma and allergy Review Source Type: research

European Respiratory Review, list of peer reviewers 2019
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 2019 (Source: European Respiratory Review)
Source: European Respiratory Review - January 29, 2020 Category: Respiratory Medicine Tags: Acknowledgement Source Type: research

The effects of pharmacological interventions on quality of life and fatigue in sarcoidosis: a systematic review
Conclusion More randomised, double-blind and placebo-controlled trials are needed to expand the evidence base on these important outcome parameters. (Source: European Respiratory Review)
Source: European Respiratory Review - January 29, 2020 Category: Respiratory Medicine Authors: Vis, R., van de Garde, E. M. W., Grutters, J. C., Korenromp, I. H. E. Tags: Pulmonary pharmacology and therapeutics Review Source Type: research

Is bronchiectasis really a disease?
The definition of a disease requires that distinguishing signs and symptoms are present that are common, and that the constellation of signs and symptoms differentiate the condition from other causes. In bronchiectasis, anatomical changes, airways inflammation and airway infection are the distinguishing features that are common to this disease. However, bronchiectasis is a heterogenous disease: signs and symptoms are shared with other airway diseases, there are multiple aetiologies and certain phenotypes of bronchiectasis have distinct clinical and laboratory features that are not common to all people with bronchiectasis. ...
Source: European Respiratory Review - January 29, 2020 Category: Respiratory Medicine Authors: Shteinberg, M., Flume, P. A., Chalmers, J. D. Tags: CF and non-CF bronchiectasis Controversies in bronchiectasis Source Type: research

"National and regional asthma programmes in Europe." Olof Selroos, Maciej Kupczyk, Piotr Kuna, Piotr Łacwik, Jean Bousquet, David Brennan, Susanna Palkonen, Javier Contreras, Mark FitzGerald, Gunilla Hedlin, Sebastian L. Johnston, Renaud Louis, Leanne Metcalf, Samantha Walker, Antonio Moreno-Galdo, Nikolaos G. Papadopoulos, Jose Rosado-Pinto, Pippa Powell and Tari Haahtela. Eur Respir Rev 2015; 24: 474-483.
(Source: European Respiratory Review)
Source: European Respiratory Review - December 23, 2019 Category: Respiratory Medicine Tags: Erratum Source Type: research

The future of bronchodilation: looking for new classes of bronchodilators
Available bronchodilators can satisfy many of the needs of patients suffering from airway disorders, but they often do not relieve symptoms and their long-term use raises safety concerns. Therefore, there is interest in developing new classes that could help to overcome the limits that characterise the existing classes. At least nine potential new classes of bronchodilators have been identified: 1) selective phosphodiesterase inhibitors; 2) bitter-taste receptor agonists; 3) E-prostanoid receptor 4 agonists; 4) Rho kinase inhibitors; 5) calcilytics; 6) agonists of peroxisome proliferator-activated receptor-; 7) agonists of...
Source: European Respiratory Review - December 23, 2019 Category: Respiratory Medicine Authors: Cazzola, M., Rogliani, P., Matera, M. G. Tags: Pulmonary pharmacology and therapeutics Reviews Source Type: research

Thoracic ultrasound competence for ultrasound-guided pleural procedures
Focused thoracic ultrasound has become essential in the guidance and direction of pleural interventions to reduce unwanted complications and as a result now forms a crucial component of physician training. Current training standards along with assessment methods vary widely, and are often not robust enough to ensure adequate competence. This review assesses the current state of training and assessment of thoracic ultrasound competence in various settings, allowing comparison with alternative competency based programmes. Future directions for training and assessment of thoracic ultrasound competence are discussed. (Source: ...
Source: European Respiratory Review - December 23, 2019 Category: Respiratory Medicine Authors: McCracken, D. J., Laursen, C. B., Barker, G., Gleeson, F. V., Cullen, K. M., Rahman, N. M. Tags: Lung imaging Reviews Source Type: research