Risk assessment in patients with systemic sclerosis and pulmonary arterial hypertension
The current treatment of patients with pulmonary arterial hypertension (PAH) is founded on repeated risk stratification at diagnosis and during follow-up. The 2015 European pulmonary hypertension guidelines propose a multidimensional risk assessment strategy based on nine items and 13 distinct variables in order to predict the estimated 1-year mortality [1, 2]. Recently, several large registry studies have shown that simplified tools can be used to predict outcomes and guide treatment decisions [3–6]. These studies have focused largely on 6-min walking distance (6MWD), World Health Organization (WHO) functional class...
Source: European Respiratory Journal - October 18, 2018 Category: Respiratory Medicine Authors: Olsson, K. M., Hoeper, M. M. Tags: Pulmonary vascular disease Editorials Source Type: research

Whither pulmonary rehabilitation? Will alternative modes help or hurt?
Chronic obstructive pulmonary disease (COPD) causes great misery to those afflicted. While it would be nice to think that a cure is near at hand, this remains only a distant dream. Symptom relief is the best we can do for the time being. We look for therapies to provide important patient-centred benefits such as improved exercise tolerance, reduced dyspnoea and better health-related quality of life. When we consider available therapies, we might consider comparing the magnitude of benefits of bronchodilators with those of pulmonary rehabilitation. A recent informal comparison of these benefits gleaned from meta-analyses in...
Source: European Respiratory Journal - October 18, 2018 Category: Respiratory Medicine Authors: Casaburi, R. Tags: Editorials Source Type: research

The Severe Paediatric Asthma Collaborative in Europe (SPACE) ERS Clinical Research Collaboration: enhancing participation of children with asthma in therapeutic trials of new biologics and receptor blockers
Asthma is one of the most common chronic diseases of childhood. Fortunately, most children with asthma have a mild or moderate form of the disease [1], and symptom control can be achieved by ensuring adequate provision of care and continuously addressing the basics in asthma management through shared decision-making with patients and their families. However, asthma remains difficult to treat for a small proportion of children who may be symptomatic despite aggressive maintenance therapy and after management issues like adherence to treatment have been addressed [2, 3]. The expression "a small proportion" is anecd...
Source: European Respiratory Journal - October 18, 2018 Category: Respiratory Medicine Authors: Rusconi, F., Fernandes, R. M., Pijnenburg, M. W. H., Grigg, J., on behalf of the SPACE Clinical Research Collaboration Tags: Editorials Source Type: research

Baseline patient factors impact on the clinical efficacy of benralizumab for severe asthma
Benralizumab is an anti-eosinophilic monoclonal antibody that reduces exacerbations and improves lung function for patients with severe, uncontrolled asthma with eosinophilic inflammation. We evaluated the impact of baseline factors on benralizumab efficacy for patients with severe asthma. This analysis used pooled data from the SIROCCO (ClinicalTrials.gov identifier NCT01928771) and CALIMA (ClinicalTrials.gov identifier NCT01914757) Phase III studies. Patients aged 12–75 years with severe, uncontrolled asthma receiving high-dosage inhaled corticosteroids plus long-acting β2-agonists received benralizumab 3...
Source: European Respiratory Journal - October 18, 2018 Category: Respiratory Medicine Authors: Bleecker, E. R., Wechsler, M. E., FitzGerald, J. M., Menzies-Gow, A., Wu, Y., Hirsch, I., Goldman, M., Newbold, P., Zangrilli, J. G. Tags: Asthma and allergy Original Articles: Asthma Source Type: research

Haemodynamics and serial risk assessment in systemic sclerosis associated pulmonary arterial hypertension
The prognostic importance of follow-up haemodynamics and the validity of multidimensional risk assessment are not well established for systemic sclerosis (SSc)-associated pulmonary arterial hypertension (PAH). We assessed incident SSc-PAH patients to determine the association between clinical and haemodynamic variables at baseline and first follow-up right heart catheterisation (RHC) with transplant-free survival. RHC variables included cardiac index, stroke volume index (SVI), pulmonary arterial compliance and pulmonary vascular resistance. Risk assessment was performed according to the number of low-risk criteria: functi...
Source: European Respiratory Journal - October 18, 2018 Category: Respiratory Medicine Authors: Weatherald, J., Boucly, A., Launay, D., Cottin, V., Prevot, G., Bourlier, D., Dauphin, C., Chaouat, A., Savale, L., Jaïs, X., Jevnikar, M., Traclet, J., De Groote, P., Simonneau, G., Hachulla, E., Mouthon, L., Montani, D., Humbert, M., Sitbon, O. Tags: Original Articles: Pulmonary hypertension Source Type: research

Risk assessment in scleroderma patients with newly diagnosed pulmonary arterial hypertension: application of the ESC/ERS risk prediction model
Pulmonary arterial hypertension (PAH) is characterised by sustained pulmonary vasoconstriction and remodelling of the pulmonary circulation leading to progressive right ventricular (RV) dysfunction. Although recent registry data suggest improving outcomes, PAH still carries a high morbidity and mortality burden [1]. (Source: European Respiratory Journal)
Source: European Respiratory Journal - October 18, 2018 Category: Respiratory Medicine Authors: Mercurio, V., Diab, N., Peloquin, G., Housten-Harris, T., Damico, R., Kolb, T. M., Mathai, S. C., Hassoun, P. M. Tags: Original Articles: Research letters Source Type: research

Extensively drug-resistant tuberculosis in South Africa: genomic evidence supporting transmission in communities
Despite evidence that transmission is driving an extensively drug-resistant TB (XDR-TB) epidemic, our understanding of where and between whom transmission occurs is limited. We sought to determine whether there was genomic evidence of transmission between individuals without an epidemiologic connection. We conducted a prospective study of XDR-TB patients in KwaZulu-Natal, South Africa, during the 2011–2014 period. We collected sociodemographic and clinical data, and identified epidemiologic links based on person-to-person or hospital-based connections. We performed whole-genome sequencing (WGS) on the Mycobacterium t...
Source: European Respiratory Journal - October 18, 2018 Category: Respiratory Medicine Authors: Auld, S. C., Shah, N. S., Mathema, B., Brown, T. S., Ismail, N., Omar, S. V., Brust, J. C. M., Nelson, K. N., Allana, S., Campbell, A., Mlisana, K., Moodley, P., Gandhi, N. R. Tags: Original Articles: Tuberculosis Source Type: research

Long-term efficacy and effectiveness of a behavioural and community-based exercise intervention (Urban Training) to increase physical activity in patients with COPD: a randomised controlled trial
There is a need to increase and maintain physical activity in patients with chronic obstructive pulmonary disease (COPD). We assessed 12-month efficacy and effectiveness of the Urban Training intervention on physical activity in COPD patients. This randomised controlled trial (NCT01897298) allocated 407 COPD patients from primary and hospital settings 1:1 to usual care (n=205) or Urban Training (n=202). Urban Training consisted of a baseline motivational interview, advice to walk on urban trails designed for COPD patients in outdoor public spaces and other optional components for feedback, motivation, information and suppo...
Source: European Respiratory Journal - October 18, 2018 Category: Respiratory Medicine Authors: Arbillaga-Etxarri, A., Gimeno-Santos, E., Barberan-Garcia, A., Balcells, E., Benet, M., Borrell, E., Celorrio, N., Delgado, A., Jane, C., Marin, A., Martin-Cantera, C., Monteagudo, M., Montella, N., Munoz, L., Ortega, P., Rodriguez, D. A., Rodriguez-Roisi Tags: Original Articles: COPD Source Type: research

Physical activity: the key to cardiometabolic risk reduction in obstructive sleep apnoea
Obstructive sleep apnoea (OSA) is a prevalent sleep disorder, with recent estimates reporting prevalence ranging between 3% and 17% for moderate-severe OSA depending on age and sex [1]. (Source: European Respiratory Journal)
Source: European Respiratory Journal - October 10, 2018 Category: Respiratory Medicine Authors: Mendelson, M., Flore, P. Tags: Editorials Source Type: research

The European Sleep Apnoea Database (ESADA) ERS Clinical Research Collaboration: past, present and future
The European Sleep Apnoea Database (ESADA) study began in 2007 as a joint project within the European Union (EU) Cooperation in Science and Technology (COST) Action B26 on obstructive sleep apnoea (OSA) and cardiovascular risk, as a network of nationally appointed sleep apnoea experts. At that time, there were few cohorts of OSA patients, mainly in the context of epidemiological studies in the general population [1–6]. The COST Action only provided funding for networking, and each participating centre entered data into the web-based database on a voluntary basis. Collected data included anthropometrics, results of bl...
Source: European Respiratory Journal - October 10, 2018 Category: Respiratory Medicine Authors: Bonsignore, M. R., Hedner, J., on behalf of the ESADA study group Tags: Editorials Source Type: research

Myeloperoxidase oxidation of methionine associates with early cystic fibrosis lung disease
Cystic fibrosis (CF) lung disease progressively worsens from infancy to adulthood. Disease-driven changes in early CF airway fluid metabolites may identify therapeutic targets to curb progression. CF patients aged 12–38 months (n=24; three out of 24 later denoted as CF screen positive, inconclusive diagnosis) received chest computed tomography scans, scored by the Perth–Rotterdam Annotated Grid Morphometric Analysis for CF (PRAGMA-CF) method to quantify total lung disease (PRAGMA-%Dis) and components such as bronchiectasis (PRAGMA-%Bx). Small molecules in bronchoalveolar lavage fluid (BALF) were measured w...
Source: European Respiratory Journal - October 10, 2018 Category: Respiratory Medicine Authors: Chandler, J. D., Margaroli, C., Horati, H., Kilgore, M. B., Veltman, M., Liu, H. K., Taurone, A. J., Peng, L., Guglani, L., Uppal, K., Go, Y.-M., Tiddens, H. A. W. M., Scholte, B. J., Tirouvanziam, R., Jones, D. P., Janssens, H. M. Tags: Original Articles: Cystic fibrosis Source Type: research

Hands are vehicles for transmission of Streptococcus pneumoniae in novel controlled human infection study
Streptococcus pneumoniae (pneumococcus) is a major cause of acute otitis media, sinusitis, pneumonia and meningitis worldwide [1]. More than 1.2 million infant deaths are attributed to S. pneumoniae annually [2]. (Source: European Respiratory Journal)
Source: European Respiratory Journal - October 10, 2018 Category: Respiratory Medicine Authors: Connor, V., German, E., Pojar, S., Mitsi, E., Hales, C., Nikolaou, E., Hyder-Wright, A., Adler, H., Zaidi, S., Hill, H., Jochems, S. P., Burhan, H., French, N., Tobery, T., Rylance, J., Ferreira, D. M. Tags: Original Articles: Research letters Source Type: research

The Mechanical Ventilation-Respiratory Distress Observation Scale as a surrogate of self-reported dyspnoea in intubated patients
Intensive care unit (ICU) patients are exposed to many sources of discomfort, among which dyspnoea is one of the more severely distressing [1]. In invasively mechanically ventilated patients, dyspnoea is frequent (47% of intubated patients report breathing discomfort when they can first communicate with caregivers) and severe (median rating of 5 on a dyspnoea visual analogue scale (D-VAS); association with anxiety and neurovegetative signs of stress) [2]. It is often linked to ventilator settings and seems to be associated with poorer clinical outcomes (e.g. delayed extubation and post-traumatic stress disorders) [2, 3]. A...
Source: European Respiratory Journal - October 10, 2018 Category: Respiratory Medicine Authors: Decavele, M., Gay, F., Persichini, R., Mayaux, J., Morelot-Panzini, C., Similowski, T., Demoule, A. Tags: Original Articles: Research letters Source Type: research

Chronic blood exchange transfusions in the management of pre-capillary pulmonary hypertension complicating sickle cell disease
The long-term effects of chronic blood exchange transfusions (BETs) on pre-capillary pulmonary hypertension complicating sickle cell disease (SCD) are unknown. 13 homozygous SS SCD patients suffering from pre-capillary pulmonary hypertension and treated by chronic BETs were evaluated retrospectively. Assessments included haemodynamics, New York Heart Association Functional Class (NYHA FC), 6-min walk distance (6MWD) and blood tests. Before initiating BETs, all patients were NYHA FC III or IV, median (range) 6MWD was 223 (0–501) m and median (range) pulmonary vascular resistance (PVR) was 3.7 (2–12.5)&...
Source: European Respiratory Journal - October 10, 2018 Category: Respiratory Medicine Authors: Turpin, M., Chantalat-Auger, C., Parent, F., Driss, F., Lionnet, F., Habibi, A., Maitre, B., Huertas, A., Jaïs, X., Weatherald, J., Montani, D., Sitbon, O., Simonneau, G., Galacteros, F., Humbert, M., Bartolucci, P., Savale, L. Tags: Original Articles: Pulmonary hypertension Source Type: research

Physical activity as a moderator for obstructive sleep apnoea and cardiometabolic risk in the EPISONO study
In this study we aimed to examine the incidence of OSA in relation to physical activity, and its role as a protective factor in individuals with OSA on the incidence of cardiometabolic diseases, in an 8–9-year follow-up study. We analysed data of 658 volunteers from the São Paulo Epidemiologic Sleep Study (EPISONO), a cohort study of individuals aged 20–80 years, collected through polysomnography, the International Physical Activity Questionnaire and an assessment of cardiometabolic profile. Active subjects had a lower risk of developing OSA compared with nonactive subjects (relative risk 0.877, 95%...
Source: European Respiratory Journal - October 10, 2018 Category: Respiratory Medicine Authors: Monico-Neto, M., Moreira Antunes, H. K., dos Santos, R. V. T., D'Almeida, V., Alves Lino de Souza, A., Azeredo Bittencourt, L. R., Tufik, S. Tags: Original Articles: Sleep apnoea Source Type: research

"Safety of nintedanib added to pirfenidone treatment for idiopathic pulmonary fibrosis." Kevin R. Flaherty, Charlene D. Fell, J. Terrill Huggins, Hilario Nunes, Robert Sussman, Claudia Valenzuela, Ute Petzinger, John L. Stauffer, Frank Gilberg, Monica Bengus and Marlies Wijsenbeek. Eur Respir J 2018; 52: 1800230.
(Source: European Respiratory Journal)
Source: European Respiratory Journal - October 4, 2018 Category: Respiratory Medicine Tags: Author Correction Source Type: research

Breaking news: DNA damage and repair pathways in COPD and implications for pathogenesis and treatment
Cigarette smoke exposure is the major driver of chronic obstructive pulmonary disease (COPD) and lung cancer in the developed world [1]. COPD affects over 450 million people worldwide whilst lung cancer is the biggest cause of death in people with cancer [2, 3]. Cigarette smoke-mediated DNA damage causes apoptosis, cellular senescence, inflammation and mutagenesis, which have all been implicated in the pathogenesis of COPD [4, 5]. Reduced components of DNA damage and repair pathways have previously been reported in COPD lung tissues (with or without associated pulmonary emphysema) and blood (including stem cells), and airw...
Source: European Respiratory Journal - October 4, 2018 Category: Respiratory Medicine Authors: Adcock, I. M., Mumby, S., Caramori, G. Tags: Editorials Source Type: research

Risk factors for lung disease progression in children with cystic fibrosis
We read with interest the recent paper by van Horck et al. [1], which studied 545 children followed for 5 years with longitudinal data from the Dutch Cystic Fibrosis registry. Data from 2009 to 2014 showed that proton pump inhibitor (PPI) use was associated with annual decline of % predicted forced expiratory volume in 1 s and future pulmonary exacerbation rates. In a discussion of potential mechanisms, the authors considered that bacteria are normally killed by acid conditions in the stomach but that gastric pH is raised following PPI use. It was therefore hypothesised that with extra-oesophageal reflux, survivi...
Source: European Respiratory Journal - October 4, 2018 Category: Respiratory Medicine Authors: Al Momani, H., Perry, A., Jones, R., McDonnell, M., Krishnan, A., Robertson, A., Griffin, M., Rutherford, R., Brodlie, M., Pearson, J., Bourke, S., Ward, C. Tags: Original Articles: Correspondence Source Type: research

A new era for treatment of drug-resistant tuberculosis
For three decades, drug-resistant tuberculosis (TB) has posed grave challenges to patients, communities and global TB control efforts. Treatment of multidrug-resistant (MDR)-TB (i.e. TB resistant to at least isoniazid and rifampicin) and extensively drug-resistant (XDR)-TB (i.e. MDR-TB with additional resistance to fluoroquinolones and second-line injectable TB drugs) has relied on medications that are less potent and more toxic than first-line TB therapy. Consequently, drug-resistant TB treatment takes 2 years to complete and has been associated with frequent and severe side-effects. Treatment outcomes remain abysmal...
Source: European Respiratory Journal - October 4, 2018 Category: Respiratory Medicine Authors: Gandhi, N. R., Brust, J. C. M., Shah, N. S. Tags: Editorials Source Type: research

Reducing the need for carbon dioxide monitoring in the investigation of paediatric sleep disordered breathing
There is increasing interest in the use of home sleep studies to diagnose paediatric sleep disordered breathing (SDB) as they are potentially more cost-effective, convenient and representative of the child's typical nights' sleep [1–3]. One concern is that most home sleep study equipment does not include measurement of carbon dioxide. (Source: European Respiratory Journal)
Source: European Respiratory Journal - October 4, 2018 Category: Respiratory Medicine Authors: Trucco, F., Bush, A., Tan, H.-L. Tags: Original Articles: Research letters Source Type: research

Anti-tumour effect of low molecular weight heparin in localised lung cancer: a phase III clinical trial
The anti-tumour and anti-metastatic properties of heparins have not been tested in patients with early stage cancer. Whether adjuvant low molecular weight heparin (LMWH) tinzaparin impacts the survival of patients with resected non-small cell lung cancer (NSCLC) was investigated. Patients with completely resected stage I, II or IIIA NSCLC were randomly allocated to receive subcutaneous tinzaparin 100 IU·kg–1 once a day for 12 weeks or no treatment in addition to standard of care. The trial was open-label with blinded central adjudication of study outcomes. The primary outcome was overall survival. In...
Source: European Respiratory Journal - October 4, 2018 Category: Respiratory Medicine Authors: Meyer, G., Besse, B., Doubre, H., Charles-Nelson, A., Aquilanti, S., Izadifar, A., Azarian, R., Monnet, I., Lamour, C., Descourt, R., Oliviero, G., Taillade, L., Chouaid, C., Giraud, F., Falcoz, P.-E., Revel, M.-P., Westeel, V., Dixmier, A., Tredaniel, J. Tags: Lung cancer Original Articles: Lung cancer Source Type: research

Nuclear magnetic resonance-based metabolomics in respiratory medicine
Metabolomics investigates the chemical end products of biological processes in living systems. It combines high-throughput analytical techniques with bioinformatics to investigate and quantify, in a global (untargeted) and/or specific (targeted) manner, the metabolites present in living systems in response to any exposure (including therapeutics), lifestyle, environmental and genetic stress [1]. The unique profile obtained can provide direct information on a physiological status or on a sequence of events in an organism. Since the metabolome (i.e. all the endogenous metabolites of molecular weight
Source: European Respiratory Journal - October 4, 2018 Category: Respiratory Medicine Authors: Paris, D., Maniscalco, M., Motta, A. Tags: ERJ Methods Source Type: research

Effect of the CXCR2 antagonist danirixin on symptoms and health status in COPD
We report the results of a 52-week Phase 2 study conducted in Germany and the USA (GSK protocol 200163; ClinicalTrials.gov identifier NCT02130193) assessing the effects of danirixin when added to standard-of-care inhaled medications in participants with symptomatic COPD. Participants with a forced expiratory volume in 1 s (FEV1) ≥50% of predicted normal and a history of two exacerbations in the prior 12 months, or one exacerbation and elevated plasma fibrinogen ≥3 mg·mL–1, as well as a history of chronic cough and/or mucus hypersecretion on most days for at least the previous 3 months prio...
Source: European Respiratory Journal - October 4, 2018 Category: Respiratory Medicine Authors: Lazaar, A. L., Miller, B. E., Tabberer, M., Yonchuk, J., Leidy, N., Ambery, C., Bloomer, J., Watz, H., Tal-Singer, R. Tags: Original Articles: Research letters Source Type: research

SPLUNC1 degradation by the cystic fibrosis mucosal environment drives airway surface liquid dehydration
The multi-organ disease cystic fibrosis (CF) is caused by mutations in the cystic fibrosis transmembrane regulator gene (CFTR) that lead to diminished transepithelial anion transport. CF lungs are characterised by airway surface liquid (ASL) dehydration, chronic infection/inflammation and neutrophilia. Dysfunctional CFTR may upregulate the epithelial Na+ channel (ENaC), further exacerbating dehydration. We previously demonstrated that short palate lung and nasal epithelial clone 1 (SPLUNC1) negatively regulates ENaC in normal airway epithelia. Here, we used pulmonary tissue samples, sputum and human bronchial epithelial ce...
Source: European Respiratory Journal - October 4, 2018 Category: Respiratory Medicine Authors: Webster, M. J., Reidel, B., Tan, C. D., Ghosh, A., Alexis, N. E., Donaldson, S. H., Kesimer, M., Ribeiro, C. M. P., Tarran, R. Tags: CF and non-CF bronchiectasis Original Articles: Cystic fibrosis Source Type: research

The DNA repair transcriptome in severe COPD
Inadequate DNA repair is implicated in the pathogenesis of chronic obstructive pulmonary disease (COPD). However, the mechanisms that underlie inadequate DNA repair in COPD are poorly understood. We applied an integrative genomic approach to identify DNA repair genes and pathways associated with COPD severity. We measured the transcriptomic changes of 419 genes involved in DNA repair and DNA damage tolerance that occur with severe COPD in three independent cohorts (n=1129). Differentially expressed genes were confirmed with RNA sequencing and used for patient clustering. Clinical and genome-wide transcriptomic differe...
Source: European Respiratory Journal - October 4, 2018 Category: Respiratory Medicine Authors: Sauler, M., Lamontagne, M., Finnemore, E., Herazo-Maya, J. D., Tedrow, J., Zhang, X., Morneau, J. E., Sciurba, F., Timens, W., Pare, P. D., Lee, P. J., Kaminski, N., Bosse, Y., Gomez, J. L. Tags: COPD and smoking, Lung biology and experimental studies Original Articles: Basic science and COPD Source Type: research

The mode of action of anticholinergics in asthma
Acetylcholine binds to muscarinic receptors to play a key role in the pathophysiology of asthma, leading to bronchoconstriction, increased mucus secretion, inflammation and airway remodelling. Anticholinergics are muscarinic receptor antagonists that are used in the treatment of chronic obstructive pulmonary disease and asthma. Recent in vivo and in vitro data have increased our understanding of how acetylcholine contributes to the disease manifestations of asthma, as well as elucidating the mechanism of action of anticholinergics. This review assesses the latest literature on acetylcholine in asthma pathophysiology, with ...
Source: European Respiratory Journal - October 4, 2018 Category: Respiratory Medicine Authors: Gosens, R., Gross, N. Tags: Back to Basics Source Type: research

Exercise pulmonary haemodynamic response predicts outcomes in fibrotic lung disease
Pulmonary hypertension is diagnosed by an elevated mean pulmonary arterial pressure (mPAP) during resting right heart catheterisation (RHC) [1]. Although "exercise-induced pulmonary hypertension" is no longer a distinct clinical entity, recently, there has been renewed interest in the prognostic value of pulmonary haemodynamic responses to exercise, particularly in patients with fibrotic lung disease [1–4]. As the optimal use of exercise RHC in this cohort remains unknown, our main objective in this study was to study the relationship between exercise haemodynamics and outcomes in fibrotic lung disease pati...
Source: European Respiratory Journal - September 27, 2018 Category: Respiratory Medicine Authors: Jose, A., King, C. S., Shlobin, O. A., Brown, A. W., Wang, C., Nathan, S. D. Tags: Original Articles: Research letters Source Type: research

Identifying obstructive sleep apnoea patients responsive to supplemental oxygen therapy
A possible precision-medicine approach to treating obstructive sleep apnoea (OSA) involves targeting ventilatory instability (elevated loop gain) using supplemental inspired oxygen in selected patients. Here we test whether elevated loop gain and three key endophenotypic traits (collapsibility, compensation and arousability), quantified using clinical polysomnography, can predict the effect of supplemental oxygen on OSA severity. 36 patients (apnoea–hypopnoea index (AHI)>20 events·h–1) completed two overnight polysomnographic studies (single-blinded randomised-controlled crossover) on supplementa...
Source: European Respiratory Journal - September 27, 2018 Category: Respiratory Medicine Authors: Sands, S. A., Edwards, B. A., Terrill, P. I., Butler, J. P., Owens, R. L., Taranto-Montemurro, L., Azarbarzin, A., Marques, M., Hess, L. B., Smales, E. T., de Melo, C. M., White, D. P., Malhotra, A., Wellman, A. Tags: Sleep medicine Original Articles: Sleep apnoea Source Type: research

Does early onset asthma increase childhood obesity risk? A pooled analysis of 16 European cohorts
This study includes 21 130 children born from 1990 to 2008 in Denmark, France, Germany, Greece, Italy, The Netherlands, Spain, Sweden and the UK. We followed non-obese children at 3–4 years of age for incident obesity up to 8 years of age. Physician-diagnosed asthma, wheezing and allergic rhinitis were assessed up to 3–4 years of age. Children with physician-diagnosed asthma had a higher risk for incident obesity than those without asthma (adjusted hazard ratio (aHR) 1.66, 95% CI 1.18–2.33). Children with active asthma (wheeze in the last 12 months and physician-diagnosed asthma) exhib...
Source: European Respiratory Journal - September 27, 2018 Category: Respiratory Medicine Authors: Contreras, Z. A., Chen, Z., Roumeliotaki, T., Annesi-Maesano, I., Baïz, N., von Berg, A., Bergström, A., Crozier, S., Duijts, L., Ekström, S., Eller, E., Fantini, M. P., Kjaer, H. F., Forastiere, F., Gerhard, B., Gori, D., Harskamp Tags: Paediatric pulmonology Original Articles: Paediatric lung disease Source Type: research

Air pollution exposure and lung function until age 16 years: the PIAMA birth cohort study
Evidence for the effects of air pollution exposure on lung function growth into adolescence is scarce. We investigated associations of air pollution exposure with lung function and lung function growth until age 16. We conducted both longitudinal (n=915) and cross-sectional (n=721) analyses of associations of air pollution exposure with forced expiratory volume in 1 s (FEV1) and forced vital capacity (FVC) growth from ages eight to 16 and FEV1 and FVC at age 16. We estimated residential concentrations of nitrogen dioxide (NO2), "soot" and particulate matter (PMx, where x is the 50% cut-off aerodynamic diamet...
Source: European Respiratory Journal - September 27, 2018 Category: Respiratory Medicine Authors: Milanzi, E. B., Koppelman, G. H., Smit, H. A., Wijga, A. H., Oldenwening, M., Vonk, J. M., Brunekreef, B., Gehring, U. Tags: Paediatric pulmonology Original Articles: Paediatric lung disease Source Type: research

Don't forget about neuromuscular disorders!
Respiratory medicine ostensibly targets diseases of the airways and the lungs, while respiratory problems arising from other entities are suggested to only play a minor role in this branch of medicine. However, diseases of the respiratory pump, particularly those associated with neuromuscular disorders (NMD), bear significant relevance to respiratory physicians, both in clinical practice and in terms of the scientific evolution of respiratory medicine and mechanical ventilation [1, 2]. During the 1952 Copenhagen polio epidemic, polio victims with severe respiratory acidosis secondary to respiratory paralysis were the first...
Source: European Respiratory Journal - September 18, 2018 Category: Respiratory Medicine Authors: Windisch, W. Tags: Editorials Source Type: research

A randomised trial of voriconazole and prednisolone monotherapy in acute-stage allergic bronchopulmonary aspergillosis complicating asthma
Glucocorticoids are the most widely used agents in the treatment of allergic bronchopulmonary aspergillosis (ABPA), a disorder characterised by immunologic reactions mounted against Aspergillus fumigatus colonising the airways of patients with asthma and cystic fibrosis [1, 2]. Unfortunately, the use of glucocorticoids is associated with several adverse reactions [3]. A novel treatment strategy in ABPA would be the use of antifungal triazoles as monotherapy. Recently, we have shown that itraconazole was effective as monotherapy in acute-stage ABPA [4]. Whether voriconazole monotherapy is also efficacious in acute-stage ABP...
Source: European Respiratory Journal - September 18, 2018 Category: Respiratory Medicine Authors: Agarwal, R., Dhooria, S., Sehgal, I. S., Aggarwal, A. N., Garg, M., Saikia, B., Chakrabarti, A. Tags: Original Articles: Research letters Source Type: research

Patterns of healthcare utilisation for respiratory complications of adults with neuromuscular disease: a population study
Our objective was to quantify health service utilisation including monitoring and treatment of respiratory complications for adults with neuromuscular disease (NMD), identifying practice variation and adherence to guideline recommendations at a population level. We conducted a population-based longitudinal cohort study (2003–2015) of adults with NMD using hospital diagnostic and health insurance billing codes within administrative health databases. We identified 185 586 adults with NMD. Mean age 52 years, 59% female. 41 173 (22%) went to an emergency department for respiratory complications on average 1.6 times ...
Source: European Respiratory Journal - September 18, 2018 Category: Respiratory Medicine Authors: Rose, L., McKim, D., Leasa, D., Nonoyama, M., Tandon, A., Bai, Y. Q., Amin, R., Katz, S., Goldstein, R., Gershon, A. Tags: Respiratory clinical practice Original Articles: Neuromuscular disease Source Type: research

Increasing burden of noninfectious lung disease in persons living with HIV: a 7-year study using the French nationwide hospital administrative database
An overall reduction in the incidence of AIDS and a change in the spectrum of lung disease have been noticed in persons living with HIV (PLHIV). Our aim was to provide an epidemiological update regarding the prevalence of lung diseases in PLHIV hospitalised in France. We analysed the prevalence of lung disease in PLHIV hospitalised in France from 2007 to 2013, from the French nationwide hospital medical information database, and assessed the association between HIV and incident noninfectious disease over 4 years of follow-up. A total of 52 091 PLHIV were hospitalised in France between 2007 and 2013. Among PLHIV hospit...
Source: European Respiratory Journal - September 18, 2018 Category: Respiratory Medicine Authors: Maitre, T., Cottenet, J., Beltramo, G., Georges, M., Blot, M., Piroth, L., Bonniaud, P., Quantin, C. Tags: Original Articles: Rare lung diseases Source Type: research

ERJ September Podcast: Challenges and perspectives in obstructive sleep apnoea
As part of the September issue, the European Respiratory Journal presents the latest in its series of podcasts. Section Editor David Gozal interviews Walter McNicholas (St Vincent's University Hospital and University College Dublin, Ireland) about a report by an ad hoc working group of the Sleep Disordered Breathing Group of the European Respiratory Society and the European Sleep Research Society. (Source: European Respiratory Journal)
Source: European Respiratory Journal - September 18, 2018 Category: Respiratory Medicine Tags: Electronic Resources Source Type: research

Advancing into the details of pulmonary haemodynamics during exercise
Pulmonary haemodynamics during exercise may help us to understand why patients are severely limited despite relatively moderate pathological findings at rest. Recently, a European Respiratory Society task force presented the expert consensus on clinical experience and published studies over the past 50 years in this field [1], and suggested a definition of "exercise pulmonary hypertension" (exercise PH). In contrast to previous guidelines [2], this expert statement does not consider an isolated increase of mean pulmonary arterial pressure (MPAP) over a certain threshold during exercise as pathological. Instead, &...
Source: European Respiratory Journal - September 17, 2018 Category: Respiratory Medicine Authors: Kovacs, G., Olschewski, H. Tags: Editorials Source Type: research

Quantitative high-resolution computed tomography fibrosis score: performance characteristics in idiopathic pulmonary fibrosis
We evaluated performance characteristics and estimated the minimal clinically important difference (MCID) of data-driven texture analysis (DTA), a high-resolution computed tomography (HRCT)-derived measurement of lung fibrosis, in subjects with idiopathic pulmonary fibrosis (IPF). The study population included 141 subjects with IPF from two interventional clinical trials who had both baseline and nominal 54- or 60-week follow-up HRCT. DTA scores were computed and compared with forced vital capacity (FVC), diffusing capacity of the lung for carbon monoxide, distance covered during a 6-min walk test and St George's Respirato...
Source: European Respiratory Journal - September 17, 2018 Category: Respiratory Medicine Authors: Humphries, S. M., Swigris, J. J., Brown, K. K., Strand, M., Gong, Q., Sundy, J. S., Raghu, G., Schwarz, M. I., Flaherty, K. R., Sood, R., O'Riordan, T. G., Lynch, D. A. Tags: Original Articles: Interstitial lung disease and radiology Source Type: research

Global Lung Initiative equations for pulmonary hypertension screening in systemic sclerosis
Systemic sclerosis (SSc) is a connective tissue disease characterised by widespread vasculopathy and excessive fibrosis in multiple organs, including the lungs [1]. The most frequent pulmonary involvement in SSc is interstitial lung disease (ILD), but the most harmful is pulmonary hypertension (PH), a complication found in about 10% of SSc patients [2]. In patients with SSc, early diagnosis of and prompt therapy for PH (either isolated or associated with ILD) are beneficial from a prognostic standpoint and recommendations for active screening of PH in SSc have therefore been established [3]. (Source: European Respiratory Journal)
Source: European Respiratory Journal - September 17, 2018 Category: Respiratory Medicine Authors: Soumagne, T., Guillien, A., Chambellan, A., Hua-Huy, T., Dinh-Xuan, A. T., Degano, B. Tags: Original Articles: Research letters Source Type: research

Exercise right heart catheterisation before and after pulmonary endarterectomy in patients with chronic thromboembolic disease
We report on the impact of pulmonary endarterectomy (PEA) on pulmonary haemodynamics in a prospective series of 32 consecutive CTED patients who underwent PEA. All patients had a comprehensive diagnostic work-up including right heart catheterisation at baseline and 12 months after PEA. Furthermore, in 12 patients exercise right heart catheterisation was performed before and after PEA. After PEA, MPAP was lower at rest (20±3 versus 17±3 mmHg; p=0.008) and during maximal exercise (39±8 versus 31±6 mmHg; p=0.016). The mean total pulmonary resistance (TPR) decreased from 3.6±0....
Source: European Respiratory Journal - September 17, 2018 Category: Respiratory Medicine Authors: Guth, S., Wiedenroth, C. B., Rieth, A., Richter, M. J., Gruenig, E., Ghofrani, H. A., Arlt, M., Liebetrau, C., Prüfer, D., Rolf, A., Hamm, C. W., Mayer, E. Tags: Pulmonary vascular disease Original Articles: Pulmonary vascular diseases Source Type: research

Stratifying infants with cystic fibrosis for disease severity using intestinal organoid swelling as a biomarker of CFTR function
Forskolin-induced swelling (FIS) of intestinal organoids from individuals with cystic fibrosis (CF) measures function of the cystic fibrosis transmembrane conductance regulator (CFTR), the protein mutated in CF. We investigated whether FIS corresponds with clinical outcome parameters and biomarkers of CFTR function in 34 infants diagnosed with CF. Relationships with FIS were studied for indicators of pulmonary and gastrointestinal disease. Children with low FIS had higher levels of immunoreactive trypsinogen (p=0.030) and pancreatitis-associated protein (p=0.039), more often had pancreatic insufficiency (p
Source: European Respiratory Journal - September 17, 2018 Category: Respiratory Medicine Authors: de Winter-de Groot, K. M., Janssens, H. M., van Uum, R. T., Dekkers, J. F., Berkers, G., Vonk, A., Kruisselbrink, E., Oppelaar, H., Vries, R., Clevers, H., Houwen, R. H. J., Escher, J. C., Elias, S. G., de Jonge, H. R., de Rijke, Y. B., Tiddens, H. A. W. Tags: Original Articles: Cystic fibrosis Source Type: research

Budesonide/formoterol MDI with co-suspension delivery technology in COPD: the TELOS study
TELOS compared budesonide (BD)/formoterol fumarate dihydrate (FF) metered dose inhaler (BFF MDI), formulated using innovative co-suspension delivery technology that enables consistent aerosol performance, with its monocomponents and budesonide/formoterol fumarate dihydrate dry powder inhaler (DPI) in patients with moderate to very severe chronic obstructive pulmonary disease (COPD), without a requirement for an exacerbation history. In this phase III, double-blind, parallel-group, 24-week study (NCT02766608), patients were randomised to BFF MDI 320/10 µg (n=664), BFF MDI 160/10 µg (n=649), FF MDI 10&n...
Source: European Respiratory Journal - September 16, 2018 Category: Respiratory Medicine Authors: Ferguson, G. T., Papi, A., Anzueto, A., Kerwin, E. M., Cappelletti, C., Duncan, E. A., Nyberg, J., Dorinsky, P. Tags: COPD and smoking Original Articles: COPD Source Type: research

Pulmonary endarterectomy and the cost of patient refusal
Without effective treatment, patients with chronic thromboembolic pulmonary hypertension (CTEPH) will struggle and ultimately die of right heart failure [1]. The key pathological features of CTEPH consist of obstructive chronic thrombi (resistant to thrombolysis and despite adherence to anticoagulation) in the proximal elastic pulmonary arteries combined with varying degrees of microvascular remodelling [2, 3]. Both processes contribute to pulmonary hypertension, but the successful removal of the proximal mechanical component with pulmonary endarterectomy (PEA) often leads to dramatic improvement in pulmonary hypertension ...
Source: European Respiratory Journal - September 16, 2018 Category: Respiratory Medicine Authors: Kim, N. H., Mayer, E. Tags: Editorials Source Type: research

Weight loss as a predictor of mortality in patients with interstitial lung disease
The interstitial lung diseases (ILDs) are a diverse group of diffuse parenchymal lung disorders that commonly result in pulmonary fibrosis. ILDs are broadly classified according to known and unknown aetiologies. Connective tissue disease-associated ILD (CTD-ILD) and chronic hypersensitivity pneumonitis (CHP) are among the most common ILDs of known aetiology, while idiopathic pulmonary fibrosis (IPF) and unclassifiable ILD (U-ILD) are among the most common of unknown aetiology [1–4]. (Source: European Respiratory Journal)
Source: European Respiratory Journal - September 16, 2018 Category: Respiratory Medicine Authors: Pugashetti, J., Graham, J., Boctor, N., Mendez, C., Foster, E., Juarez, M., Harper, R., Morrissey, B., Kadoch, M., Oldham, J. M. Tags: Original Articles: Research letters Source Type: research

New steps forward for obstructive sleep apnoea in the era of precision medicine
Obstructive sleep apnoea (OSA) is a progressive disorder characterised by repeated upper-airway collapse during sleep that leads to intermittent hypoxia and hypercapnia, fragmented sleep, fluctuations in blood pressure and increased sympathetic nervous system activity [1]. Population studies in the early 1990s found OSA (defined by an apnoea–hypopnoea index (AHI)>5 events·h–1) in 9% of middle-aged women and 24% of middle-aged men [2]. (Source: European Respiratory Journal)
Source: European Respiratory Journal - September 16, 2018 Category: Respiratory Medicine Authors: Peker, Y., Strollo, P. J. Tags: Sleep medicine Editorials Source Type: research

The impact of patient choice on survival in chronic thromboembolic pulmonary hypertension
Pulmonary endarterectomy (PEA) is the gold standard treatment for operable chronic thromboembolic pulmonary hypertension (CTEPH). However, a proportion of patients with operable disease decline surgery. There are currently no published data on this patient group. The aim of this study was to identify outcomes and prognostic factors in a large cohort of consecutive patients with CTEPH. Data were collected for consecutive, treatment-naive CTEPH patients at the Pulmonary Vascular Disease Unit of the Royal Hallamshire Hospital (Sheffield, UK) between 2001 and 2014. Of 550 CTEPH patients (mean±sd age 63±15 ye...
Source: European Respiratory Journal - September 16, 2018 Category: Respiratory Medicine Authors: Quadery, S. R., Swift, A. J., Billings, C. G., Thompson, A. A. R., Elliot, C. A., Hurdman, J., Charalampopoulos, A., Sabroe, I., Armstrong, I. J., Hamilton, N., Sephton, P., Garrad, S., Pepke-Zaba, J., Jenkins, D. P., Screaton, N., Rothman, A. M., Lawrie, Tags: Pulmonary vascular disease Original Articles: Pulmonary vascular diseases Source Type: research

Challenges and perspectives in obstructive sleep apnoea: Report by an ad hoc working group of the Sleep Disordered Breathing Group of the European Respiratory Society and the European Sleep Research Society
Obstructive sleep apnoea (OSA) is a major challenge for physicians and healthcare systems throughout the world. The high prevalence and the impact on daily life of OSA oblige clinicians to offer effective and acceptable treatment options. However, recent evidence has raised questions about the benefits of positive airway pressure therapy in ameliorating comorbidities. An international expert group considered the current state of knowledge based on the most relevant publications in the previous 5 years, discussed the current challenges in the field, and proposed topics for future research on epidemiology, phenotyping, ...
Source: European Respiratory Journal - September 16, 2018 Category: Respiratory Medicine Authors: Randerath, W., Bassetti, C. L., Bonsignore, M. R., Farre, R., Ferini-Strambi, L., Grote, L., Hedner, J., Kohler, M., Martinez-Garcia, M.-A., Mihaicuta, S., Montserrat, J., Pepin, J.-L., Pevernagie, D., Pizza, F., Polo, O., Riha, R., Ryan, S., Verbraecken, Tags: Reviews Source Type: research

ERS Clinical Research Collaborations: underpinning research excellence
The European Respiratory Society (ERS) mission is to promote respiratory health in order to alleviate and prevent suffering from respiratory disease. To achieve this mission there remains a number of challenges to be addressed. We need: 1) a better understanding of the burden of lung disease and its natural history, as well as of the fundamental mechanisms underpinning disease; 2) validation and application of accurate diagnostic tests, patient-centred outcomes and biomarkers; and 3) development and testing of new pharmacological and non-pharmacological interventions and their optimal implementation in clinical practice. T...
Source: European Respiratory Journal - September 15, 2018 Category: Respiratory Medicine Authors: Brightling, C., Genton, C., Bill, W., Welte, T., Gaga, M., Heuvelin, E., Brusselle, G. Tags: Editorials Source Type: research

Dissecting respiratory disease heterogeneity through the genetics of diffusing capacity
Genome-wide association studies (GWASs) have allowed the robust and replicable identification of novel genomic regions associated with respiratory diseases. For instance, in chronic obstructive pulmonary disease (COPD), nearly all of the described genetic risk regions were not previously known to play a role in COPD pathogenesis [1–9]. However, where sample size is critical to discovery of new genetic risk regions in GWASs, large GWASs of lung function (and lung function extremes) in the general population have made great strides in describing the genetic risk regions contributing to the observed population variabili...
Source: European Respiratory Journal - September 15, 2018 Category: Respiratory Medicine Authors: Hobbs, B. D., Cho, M. H. Tags: Editorials Source Type: research

Chronic breathlessness: time for Cinderella to go to the ball!
Breathlessness is a peculiar issue. The normal physiological response to exertion (physical or emotional) is clearly not a "symptom", defined as a physical or mental manifestation of disease apparent to the patient (English Oxford Living Dictionary). However, breathlessness is also one of the most common, serious and distressing symptoms caused by disease(s). Perhaps these extremes explain partly why the change from "normal" to "symptom" is often unrecognised by the breathless patient, their family and friends and their healthcare professionals, particularly when this shift occurs insidiously ...
Source: European Respiratory Journal - September 15, 2018 Category: Respiratory Medicine Authors: Johnson, M. J., Fallon, M. Tags: Editorials Source Type: research

Bridging the gap in knowledge between dyspnoea scientists and clinicians
Dyspnoea can be defined as "a subjective experience of breathing discomfort that consists of qualitatively distinct sensations that vary in intensity" [1]. It derives from interactions among multiple physiological, psychological, social and environmental factors, and may induce secondary physiological and behavioural responses. The current definition of dyspnoea highlights the importance of the multidimensional nature of dyspnoea (sensory–perceptual, affective distress and impact domains), which in turn has led to the development of multidimensional tools to assess dyspnoea [2, 3]. Our current understanding...
Source: European Respiratory Journal - September 15, 2018 Category: Respiratory Medicine Authors: Adler, D. Tags: Editorials Source Type: research