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Rituximab and mycophenolate mofetil in interstitial lung disease (EVER-ILD): 1-year follow-up results of a randomised controlled trial Eur. Respir. J. (IF 16.6) Pub Date : 2024-09-12 Laurène Mansy, Agnès Caille, Martine Reynaud-Gaubert, Julien Bermudez, Philippe Bonniaud, Raphael Borie, Pierre-Yves Brillet, Jacques Cadranel, Isabelle Court-Fortune, Bruno Crestani, Marie-Pierre Debray, Mathilde Duprez, Anne Guillaumot, Sandrine Hirschi-Santelmo, Dominique Israel-Biet, Stéphane Jouneau, Karine Juvin, Mallorie Kerjouan, Julie Mankikian, Charles-Hugo Marquette, Jean-Marc Naccache,
Extract The nonspecific interstitial pneumonia (NSIP) pattern is a usual feature of interstitial lung disease (ILD) associated with connective tissue diseases (CTDs) [1], idiopathic interstitial pneumonia with autoimmune features (IPAF) [2] and several idiopathic ILDs (iILDs) [3]. The EVER-ILD trial randomised 126 patients between rituximab (1000 mg, on day 1 and day 15; n=65) versus placebo (n=61)
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Allergen challenge, eosinophils and the long road to asthma endotypes Eur. Respir. J. (IF 16.6) Pub Date : 2024-09-12 Sally E. Wenzel
Extract Inhaled allergen challenges (IAC) have been utilised as tools to understand the clinical, physiological and pathobiological responses to allergens in asthmatic and atopic individuals for close to 75 years [1, 2]. Sensitised individuals respond with an early/immediate fall in forced expiratory volume in 1 s (FEV1): the early asthmatic response (EAR). Following a return to baseline (within 60 min)
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10 pack-years of smoking: not the magic number for COPD risk and prognosis Eur. Respir. J. (IF 16.6) Pub Date : 2024-09-12 Helena Backman
Extract Threshold values play a critical role in clinical medicine, as they provide standardised criteria for diagnosis and treatment, ensuring consistency across different healthcare providers and settings. They help in maintaining uniformity in patient care, making sure that patients with similar characteristics receive similar evaluations and interventions. They also aid in risk stratification,
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Taking your mind off breathlessness Eur. Respir. J. (IF 16.6) Pub Date : 2024-09-12 Peter M.A. Calverley
Extract In May 2024, the songwriter Richard Sherman died at the age of 94. Together with his brother Robert, he wrote numerous memorable songs for the Disney film studios, including "Trust in Me" for the original Jungle Book animated film, during which the python Kaa hypnotises the suggestible man-cub Mowgli and takes control of his actions to great comic effect. Such is the popular view of hypnosis
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Benralizumab for allergic asthma: a randomised, double-blind, placebo-controlled trial Eur. Respir. J. (IF 16.6) Pub Date : 2024-09-12 Gail M. Gauvreau, Roma Sehmi, J. Mark FitzGerald, Richard Leigh, Donald W. Cockcroft, Beth E. Davis, Irvin Mayers, Louis-Philippe Boulet, Dhuha Al-Sajee, Brittany M. Salter, Ruth P. Cusack, Terence Ho, Christiane E. Whetstone, Nadia Alsaji, Imran Satia, Kieran J. Killian, Patrick D. Mitchell, Iain P. Magee, Celine Bergeron, Mohit Bhutani, Viktoria Werkström, Tomasz Durżyński, Kathryn Shoemaker, Rohit
Background Benralizumab induces rapid and near-complete depletion of eosinophils from blood and lung tissue. We investigated whether benralizumab could attenuate the allergen-induced late asthmatic response (LAR) in participants with allergic asthma. Methods Participants with allergic asthma who demonstrated increased sputum eosinophils and LAR at screening were randomised to benralizumab 30 mg or
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Medical hypnosis mitigates laboratory dyspnoea in healthy humans: a randomised, controlled experimental trial Eur. Respir. J. (IF 16.6) Pub Date : 2024-09-12 Capucine Morélot-Panzini, Cécile Arveiller-Carvallo, Isabelle Rivals, Nicolas Wattiez, Sophie Lavault, Agnès Brion, Laure Serresse, Christian Straus, Marie-Cécile Niérat, Thomas Similowski
Question Dyspnoea persisting despite treatments of underlying causes requires symptomatic approaches. Medical hypnosis could provide relief without the untoward effects of pharmacological approaches. We addressed this question through experimentally induced dyspnoea in healthy humans (inspiratory threshold loading (excessive inspiratory effort) and carbon dioxide stimulation (air hunger)). Material
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Low smoking exposure and development and prognosis of COPD over four decades: a population-based cohort study Eur. Respir. J. (IF 16.6) Pub Date : 2024-09-12 Yunus Çolak, Anders Løkke, Jacob L. Marott, Peter Lange, Jørgen Vestbo, Børge G. Nordestgaard, Shoaib Afzal
Background A diagnosis of COPD is mainly considered in individuals with >10 pack-years of smoking. We tested the hypothesis that low smoking exposure, below the critical threshold of 10 pack-years, increases risk of COPD and leads to poor prognosis. Methods We followed non-obstructed adult smokers from the Copenhagen City Heart Study for COPD, defined as a forced expiratory volume in 1 s (FEV1)/forced
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A proposed approach to pulmonary long COVID: a viewpoint Eur. Respir. J. (IF 16.6) Pub Date : 2024-09-12 Firoozeh V. Gerayeli, Rachel L. Eddy, Don D. Sin
Extract Long COVID (also known as "post-acute sequelae of COVID-19") is a multi-system disorder that follows an acute bout of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection [1]. Although its exact prevalence is unknown, it is estimated to affect approximately 10% of SARS-CoV-2-infected individuals, though in reality the proportion is likely much higher owing to under-reporting
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Integrating hot topics and implementation of treatable traits in asthma. Eur. Respir. J. (IF 16.6) Pub Date : 2024-09-10 Peter G Gibson,Vanessa M McDonald
People with asthma experience many different problems related to their illness. The number and type of problems differs between patients. This results in asthma being a complex and heterogeneous disorder which mandates a personalised approach to management. These features pose very significant challenges for the effective implementation of evidence-based management. Treatable Traits is a model of care
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Cathepsin C (dipeptidyl peptidase 1) inhibition in adults with bronchiectasis: AIRLEAF®, a Phase II randomised, double-blind, placebo-controlled, dose-finding study. Eur. Respir. J. (IF 16.6) Pub Date : 2024-09-10 James D Chalmers,Michal Shteinberg,Marcus A Mall,Anne E O'Donnell,Henrik Watz,Abhya Gupta,Edith Frahm,Anastasia Eleftheraki,Johanna Rauch,Sanjay H Chotirmall,April W Armstrong,Peter Eickholz,Naoki Hasegawa,Wiebke Sauter,Pamela J McShane
Bronchiectasis is characterised by uncontrolled neutrophil serine protease (NSP) activity. Cathepsin C (CatC; dipeptidyl peptidase 1) activates NSPs during neutrophil maturation. CatC inhibitors can potentially reduce neutrophil-mediated lung damage. This Phase II, randomised, double-blind, placebo-controlled trial (AIRLEAF®; NCT05238675) evaluated efficacy, safety and optimal dosing of BI 1291583
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MK-5475, an inhaled soluble guanylate cyclase stimulator, for treatment of pulmonary arterial hypertension: the INSIGNIA-PAH study. Eur. Respir. J. (IF 16.6) Pub Date : 2024-09-10 Marc Humbert,Paul M Hassoun,Kelly M Chin,Guillermo Bortman,Mahesh J Patel,Carmen La Rosa,Wei Fu,Maria José Loureiro,Marius M Hoeper
BACKGROUND MK-5475 is an investigational inhaled soluble guanylate cyclase stimulator hypothesised to avoid most side-effects of systemic vasodilation. METHODS The phase 2 INSIGNIA-PAH (NCT04732221) trial randomised adults with pulmonary arterial hypertension (PAH) on stable background therapy 1:1:1:1 to once-daily dosing with placebo, MK-5475 32 µg, 100 µg or 380 µg via dry powder inhalation for 12 weeks
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Sleep disordered breathing in patients with acute myocardial infarction: finding the perfect window for saving the heart Eur. Respir. J. (IF 16.6) Pub Date : 2024-09-05 Christine Eulenburg, Claire Arnaud, Renaud Tamisier
Extract Sleep disordered breathing (SDB) encompasses two different conditions, obstructive sleep apnoea (OSA), which results from upper airway collapse during sleep, and central sleep apnoea (CSA), which is due to an altered ventilatory drive from the central nervous system [1, 2]. Both conditions stand out for interruptions of ventilation, lasting at least 10 s, that repeat all along sleep duration
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The younger, the better: lessons learned from real-world studies on CFTR modulators in young children Eur. Respir. J. (IF 16.6) Pub Date : 2024-09-05 Philippe Reix, Guillaume Chassagnon
Extract Prevention of cystic fibrosis (CF) lung disease by the early use of triple combination elexacaftor/tezacaftor/ivacaftor (ETI) will be key in the future standard of care of young children with CF [1, 2]. Almost every paediatrician agrees with this assumption, and evidence has been provided by clinical trials that it slows disease progression in school-aged children with CF [3, 4] and has a great
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Overweight and dysanapsis in childhood asthma Eur. Respir. J. (IF 16.6) Pub Date : 2024-09-05 Lies Lahousse
Extract Asthma is a chronic inflammatory disease with multiple genetic and environmental factors underpinning its various endotypes. To identify new opportunities for prevention and early asthma treatment, we need to detect, in advance, individuals on trajectories leading to obstructive lung disease and tackle associated risk factors. Besides genetic susceptibility, early risk factors for poor lung
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Safety of early rehabilitation after acute pulmonary embolism Eur. Respir. J. (IF 16.6) Pub Date : 2024-09-05 Karsten Keller, Lukas Hobohm
Extract Pulmonary embolism (PE) is a critical and potentially fatal condition with high rates of morbidity and mortality [1–3]. Despite a rise in incidence over the recent decades, the case fatality of PE decreased during the same period [1, 2]. Survival during the initial phase of hospitalisation and the short-term follow-up is closely linked to patients’ haemodynamic status, cardiac involvement including
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The evolution of the European risk stratification system for pulmonary arterial hypertension Eur. Respir. J. (IF 16.6) Pub Date : 2024-09-05 Sandeep Sahay, Raymond L. Benza
Extract Pulmonary arterial hypertension (PAH) has remained a challenging disease with poor survival [1]. Accurate risk stratification in PAH is crucial for making treatment decisions and prognostication. Current European guidelines recommend routine risk assessment at baseline and follow-up evaluation [2]. Due to the complex nature of the disease and many factors influencing survival in PAH, it is
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European Respiratory Society statement on preschool wheezing disorders: updated definitions, knowledge gaps and proposed future research directions Eur. Respir. J. (IF 16.6) Pub Date : 2024-09-05 Heidi Makrinioti, Valentina Fainardi, Klaus Bonnelykke, Adnan Custovic, Lisa Cicutto, Courtney Coleman, Thomas Eiwegger, Claudia Kuehni, Alexander Moeller, Eva Pedersen, Marielle Pijnenburg, Hilary Pinnock, Sarath Ranganathan, Thomy Tonia, Padmaja Subbarao, Sejal Saglani
Since the publication of the European Respiratory Society (ERS) task force reports on the management of preschool wheezing in 2008 and 2014, a large body of evidence has accumulated suggesting that the clinical phenotypes that were proposed (episodic (viral) wheezing and multiple-trigger wheezing) do not relate to underlying airway pathology and may not help determine response to treatment. Specifically
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Host–microbial interactions differ with age of asthma onset Eur. Respir. J. (IF 16.6) Pub Date : 2024-09-05 Ali Versi, Adnan Azim, Fransiskus Xaverius Ivan, Mahmoud I. Abdel-Aziz, Stewart Bates, John Riley, Anke H. Maitland-Van Der Zee, Sven-Erik Dahlen, Ratko Djukanovic, Sanjay H. Chotirmall, Peter Howarth, Nazanin Zounemat Kermani, Kian Fan Chung, Ian M. Adcock
Extract Asthma is a heterogenous disease [1] and dichotomisation between childhood/early-onset (EO) and adult/late-onset (LO) disease [2] identified differences in lung function decline and response to anti-inflammatory therapies, including biologics [3]. This suggests distinct inflammatory mechanisms underpin EO and LO asthma. In parallel, a relationship exists between airway neutrophilia and the
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Risk stratification refinements with inclusion of haemodynamic variables at follow-up in patients with pulmonary arterial hypertension Eur. Respir. J. (IF 16.6) Pub Date : 2024-09-05 Athénaïs Boucly, Antoine Beurnier, Ségolène Turquier, Mitja Jevnikar, Pascal de Groote, Ari Chaouat, Céline Cheron, Xavier Jaïs, François Picard, Grégoire Prévot, Anne Roche, Sabina Solinas, Vincent Cottin, Fabrice Bauer, David Montani, Marc Humbert, Laurent Savale, Olivier Sitbon
Extract Pulmonary arterial hypertension (PAH) is a cardiovascular disorder characterised by pulmonary vascular remodelling resulting in increased pulmonary arterial pressure (PAP) and pulmonary vascular resistance (PVR), which can lead to progressive right heart failure [1, 2]. Right heart catheterisation (RHC) remains the gold standard for the diagnosis of pulmonary hypertension (PH) [1, 2]. Furthermore
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Electronically delivered rehabilitation after acute pulmonary embolism is safe and improves quality of life Eur. Respir. J. (IF 16.6) Pub Date : 2024-09-05 Daniel Lachant, Deborah Haight, Dominick Roto, Alyssa Williams, Kyle Norton, R. James White
Extract Anywhere from 16% to 50% of acute pulmonary embolism (PE) survivors have residual dyspnoea (post-PE impairment) [1, 2] and consume substantial healthcare resources, despite taking effective anti-coagulation. Cardiopulmonary exercise testing has demonstrated deconditioning as a common finding [3, 4], and anxiety may fuel sedentary behaviour that prevents recovery. Rehabilitation programmes >3 months
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Genetic predisposition to high BMI increases risk of early life respiratory infections and episodes of severe wheeze and asthma Eur. Respir. J. (IF 16.6) Pub Date : 2024-09-05 Signe Kjeldgaard Jensen, Casper-Emil Tingskov Pedersen, Kasper Fischer-Rasmussen, Mathias Elsner Melgaard, Nicklas Brustad, Julie Nyholm Kyvsgaard, Nilo Vahman, Ann-Marie Malby Schoos, Jakob Stokholm, Bo Chawes, Anders Eliasen, Klaus Bønnelykke
Extract There is a well-known association between obesity and asthma [1–8], but the underlying mechanisms are unclear. Some studies suggest asthma as a cause of obesity [1, 9]. However, more studies recognise overweight or obesity as a possible risk factor for childhood asthma [2, 5, 7, 10], and a meta-analysis found a positive linear relationship between body mass index (BMI) and asthma [11]. Obesity
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Impact of elexacaftor/tezacaftor/ivacaftor therapy on lung clearance index and magnetic resonance imaging in children with cystic fibrosis and one or two F508del alleles Eur. Respir. J. (IF 16.6) Pub Date : 2024-09-05 Mirjam Stahl, Martha Dohna, Simon Y. Graeber, Olaf Sommerburg, Diane M. Renz, Sophia T. Pallenberg, Andreas Voskrebenzev, Katharina Schütz, Gesine Hansen, Felix Doellinger, Eva Steinke, Stephanie Thee, Jobst Röhmel, Sandra Barth, Claudia Rückes-Nilges, Julian Berges, Susanne Hämmerling, Mark O. Wielpütz, Lutz Naehrlich, Jens Vogel-Claussen, Burkhard Tümmler, Marcus A. Mall, Anna-Maria Dittrich
Extract Clinical trials of the triple combination cystic fibrosis transmembrane conductance regulator (CFTR) modulator therapy elexacaftor/tezacaftor/ivacaftor (ETI) demonstrated unprecedented clinical benefits including improvements in lung function, respiratory symptoms and nutritional outcomes in patients with cystic fibrosis (CF) aged ≥2 years with at least one copy of the F508del allele [1–11]
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Treatment of sleep apnoea early after myocardial infarction with adaptive servo-ventilation: a proof-of-concept randomised controlled trial Eur. Respir. J. (IF 16.6) Pub Date : 2024-09-05 Michael Arzt, Henrik Fox, Stefan Stadler, Andrea Hetzenecker, Olaf Oldenburg, Okka W. Hamer, Florian Poschenrieder, Clemens Wiest, Radu Tanacli, Sebastian Kelle, Leonhard Bruch, Mirko Seidel, Michael Koller, Florian Zeman, Stefan Buchner
Extract Infarct size after acute myocardial infarction (AMI) determined using state-of-the-art techniques such as cardiac magnetic resonance (CMR) imaging is an important contributor to the development of heart failure [1, 2]. In turn, heart failure is a significant predictor of mortality and morbidity after AMI [3]. Intermittent hypoxia [4], increased cardiac workload and left ventricular mural pressure
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Phenotypes in pulmonary hypertension Eur. Respir. J. (IF 16.6) Pub Date : 2024-09-05 Jason Weatherald, Anna R. Hemnes, Bradley A. Maron, Lisa M. Mielniczuk, Christian Gerges, Laura C. Price, Marius M. Hoeper, Marc Humbert
The clinical classification of pulmonary hypertension (PH) has guided diagnosis and treatment of patients with PH for several decades. Discoveries relating to underlying mechanisms, pathobiology and responses to treatments for PH have informed the evolution in this clinical classification to describe the heterogeneity in PH phenotypes. In more recent years, advances in imaging, computational science
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Microenvironmental acidification by pneumococcal sugar consumption fosters barrier disruption and immune suppression in the human alveolus. Eur. Respir. J. (IF 16.6) Pub Date : 2024-09-04 Diana Fatykhova,Verena N Fritsch,Keerthana Siebert,Karen Methling,Michael Lalk,Tobias Busche,Jörn Kalinowski,January Weiner,Dieter Beule,Wilhelm Bertrams,Thomas P Kohler,Sven Hammerschmidt,Anna Löwa,Mara Fischer,Maren Mieth,Katharina Hellwig,Doris Frey,Jens Neudecker,Jens C Rueckert,Mario Toennies,Torsten T Bauer,Mareike Graff,Hong-Linh Tran,Stephan Eggeling,Achim D Gruber,Haike Antelmann,Stefan Hippenstiel
Streptococcus pneumoniae (S.p.) is the most common causative agent of community-acquired pneumonia worldwide. A key pathogenic mechanism that exacerbates severity of disease is the disruption of the alveolar-capillary barrier. However, the specific virulence mechanisms responsible for this in the human lung are not yet fully understood.In this study, we infected living human lung tissue with S.p. and
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Effect of Sotatercept on Circulating Proteomics in Pulmonary Arterial Hypertension. Eur. Respir. J. (IF 16.6) Pub Date : 2024-09-03 Laurent Savale,Ly Tu,Corinne Normand,Athénaïs Boucly,Olivier Sitbon,David Montani,Karen M Olsson,Da-Hee Park,Jan Fuge,Jan C Kamp,Marc Humbert,Marius M Hoeper,Christophe Guignabert
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Worsening dyspnoea as a predictor of progression of pulmonary fibrosis. Eur. Respir. J. (IF 16.6) Pub Date : 2024-09-03 Marlies S Wijsenbeek,Jeffrey J Swigris,Paolo Spagnolo,Martin Kolb,Michael Kreuter,Hilario Nunes,Wibke Stansen,Klaus B Rohr,Yoshikazu Inoue,
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Accuracy of Computer-Aided Detection for Tuberculosis ® on paediatric chest radiographs. Eur. Respir. J. (IF 16.6) Pub Date : 2024-09-03 Victory Fabian Edem,Esin Nkereuwem,Schadrac C Agbla,Sheila A Owusu,Abdou K Sillah,Binta Saidy,Musa B Jallow,Audrey G Forson,Uzochukwu Egere,Beate Kampmann,Toyin Togun
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Impact of the expanded label for elexacaftor-tezacaftor-ivacaftor in people with cystic fibrosis with no F508del variant in the United States. Eur. Respir. J. (IF 16.6) Pub Date : 2024-09-03 Elizabeth A Cromwell,Josh S Ostrenga,Don B Sanders,Wayne Morgan,Carlo Castellani,Rhonda Szczesniak,Pierre-Regis Burgel
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Bronchoconstriction with inhaled ATP in Healthy Volunteers. Eur. Respir. J. (IF 16.6) Pub Date : 2024-09-03 Jenny King,Rachel Dockry,Paul Marsden,Stephen Fowler,Jaclyn Smith
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The Seventh World Symposium on Pulmonary Hypertension: our journey to Barcelona. Eur. Respir. J. (IF 16.6) Pub Date : 2024-09-02 Marc Humbert,Nazzareno Galiè,Lewis J Rubin,Gérald Simonneau,Vallerie V McLaughlin
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COVID-19 changed the world – without changing CTEPH Eur. Respir. J. (IF 16.6) Pub Date : 2024-08-29 Ioannis T. Farmakis, Stavros V. Konstantinides
Extract The coronavirus disease 2019 (COVID-19) pandemic, although no longer a global public health emergency since 2023, is formally still ongoing, and as of May 2024, over 775 million confirmed cases and more than seven million deaths have been reported worldwide. An association between COVID-19 infection and thrombosis was observed very early in the beginning of the pandemic [1]. Severe acute respiratory
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Reply to: Sputum colour matters: haemoptysis in a bronchiectasis registry Eur. Respir. J. (IF 16.6) Pub Date : 2024-08-29 Stefano Aliberti, James D. Chalmers
Extract We thank S. Yamamoto and co-workers for their letter, in which they argue that sputum colour assessment and sputum characterisation should include assessment of haemoptysis. Our study showed that the use of a four-point sputum colour chart to assess patients in stable state, which has been validated in bronchiectasis, could predict clinically relevant outcomes such as exacerbations and severe
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Systems biology approaches to utilise polygenic risk scores for chronic diseases Eur. Respir. J. (IF 16.6) Pub Date : 2024-08-29 Ayoung Jeong
Extract The ever-growing body of genome-wide association studies (GWASs) enables development of polygenic risk scores (PRSs). Unlike Mendelian disorders with high penetrance, the effects of common genetic variants are often incremental and non-deterministic for chronic diseases. Despite the generally small effect size, recent research findings indicate that PRSs – a large number of genetic variants
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Sputum colour matters: haemoptysis in a bronchiectasis registry Eur. Respir. J. (IF 16.6) Pub Date : 2024-08-29 Shota Yamamoto, Hideo Ishikawa, Keita Takeda, Masahiro Kawashima
Extract We read with great interest the paper by Aliberti et al. [1] on the utility of sputum colour assessment in bronchiectasis. Through this large-scale analysis of the EMBARC (European Multicentre Bronchiectasis Audit and Research Collaboration) registry and a four-category sputum colour chart, the authors demonstrate that sputum purulence is strongly associated with disease severity, bacterial
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Epigenomic partitioning of a polygenic risk score for asthma reveals distinct genetically driven disease pathways Eur. Respir. J. (IF 16.6) Pub Date : 2024-08-29 Bernard Stikker, Lianne Trap, Bahar Sedaghati-Khayat, Marjolein J.W. de Bruijn, Wilfred F.J. van Ijcken, Emmely de Roos, Arfan Ikram, Rudi W. Hendriks, Guy Brusselle, Jeroen van Rooij, Ralph Stadhouders
Background Individual differences in susceptibility to developing asthma, a heterogeneous chronic inflammatory lung disease, are poorly understood. Whether genetics can predict asthma risk and how genetic variants modulate the complex pathophysiology of asthma are still debated. Aim To build polygenic risk scores for asthma risk prediction and epigenomically link predictive genetic variants to pathophysiological
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Chronic thromboembolic pulmonary hypertension is an uncommon complication of COVID-19: UK national surveillance and observational screening cohort studies Eur. Respir. J. (IF 16.6) Pub Date : 2024-08-29 S. Ashwin Reddy, Joseph Newman, Olivia C. Leavy, Hakim Ghani, Joanna Pepke-Zaba, John E. Cannon, Karen K. Sheares, Dolores Taboada, Katherine Bunclark, Allan Lawrie, Cathie L. Sudlow, Colin Berry, James M. Wild, Jane A. Mitchell, Jennifer Quint, Jennifer Rossdale, Laura Price, Luke S. Howard, Martin Wilkins, Naveed Sattar, Philip Chowienczyk, Roger Thompson, Louise V. Wain, Alexander Horsley, Ling-Pei
Background Pulmonary embolism (PE) is a well-recognised complication of coronavirus disease 2019 (COVID-19) infection, and chronic thromboembolic pulmonary disease with and without pulmonary hypertension (CTEPD/CTEPH) are potential life-limiting consequences. At present the burden of CTEPD/CTEPH is unclear and optimal and cost-effective screening strategies yet to be established. Methods We evaluated
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ERJ Podcast August 2024: Titration of anti-IL-5 biologics in severe asthma Eur. Respir. J. (IF 16.6) Pub Date : 2024-08-29 European Respiratory Society
As part of the August issue, the European Respiratory Journal presents the latest in its series of podcasts. Deputy Chief Editor Don Sin interviews Marianne Baastrup Soendergaard (Department of Respiratory Medicine, Copenhagen University Hospital – Bispebjerg, Copenhagen, Denmark) about the OPTIMAL study: an open-label randomised controlled trial of titration of anti-interleukin-5 biologic therapy
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Embracing the challenges of neonatal and paediatric pulmonary hypertension. Eur. Respir. J. (IF 16.6) Pub Date : 2024-08-29 Dunbar Ivy,Erika B Rosenzweig,Steven H Abman,Maurice Beghetti,Damien Bonnet,Johannes Menno Douwes,Alessandra Manes,Rolf M F Berger
Paediatric pulmonary arterial hypertension (PAH) shares common features with adult disease, but is associated with several additional disorders and challenges that require unique approaches. This article discusses recent advances, ongoing challenges and distinct approaches for caring for infants and children with PAH, as presented by the paediatric task force of the 7th World Symposium on Pulmonary
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Emerging multimodality imaging techniques for the pulmonary circulation. Eur. Respir. J. (IF 16.6) Pub Date : 2024-08-29 Sudarshan Rajagopal,Harm J Bogaard,Mohammed S M Elbaz,Benjamin H Freed,Martine Remy-Jardin,Edwin J R van Beek,Deepa Gopalan,David G Kiely
Pulmonary hypertension (PH) remains a challenging condition to diagnose, classify and treat. Current approaches to the assessment of PH include echocardiography, ventilation/perfusion scintigraphy, cross-sectional imaging using computed tomography and magnetic resonance imaging, and right heart catheterisation. However, these approaches only provide an indirect readout of the primary pathology of the
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Pathophysiology of the right ventricle and its pulmonary vascular interaction. Eur. Respir. J. (IF 16.6) Pub Date : 2024-08-29 Anna R Hemnes,David S Celermajer,Michele D'Alto,Francois Haddad,Paul M Hassoun,Kurt W Prins,Robert Naeije,Anton Vonk Noordegraaf
The right ventricle and its stress response is perhaps the most important arbiter of survival in patients with pulmonary hypertension of many causes. The physiology of the cardiopulmonary unit and definition of right heart failure proposed in the 2018 World Symposium on Pulmonary Hypertension have proven useful constructs in subsequent years. Here, we review updated knowledge of basic mechanisms that
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Genetics and precision genomics approaches to pulmonary hypertension. Eur. Respir. J. (IF 16.6) Pub Date : 2024-08-29 Eric D Austin,Micheala A Aldred,Mona Alotaibi,Stefan Gräf,William C Nichols,Richard C Trembath,Wendy K Chung
Considerable progress has been made in the genomics of pulmonary arterial hypertension (PAH) since the 6th World Symposium on Pulmonary Hypertension, with the identification of rare variants in several novel genes, as well as common variants that confer a modest increase in PAH risk. Gene and variant curation by an expert panel now provides a robust framework for knowing which genes to test and how
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Exploring the patient perspective in pulmonary hypertension. Eur. Respir. J. (IF 16.6) Pub Date : 2024-08-29 H James Ford,Colleen Brunetti,Pisana Ferrari,Gergely Meszaros,Victor M Moles,Hall Skaara,Adam Torbicki,J Simon R Gibbs
The global impacts of pulmonary hypertension (PH) were formally recognised in 1973 at the 1st World Health Organization meeting dedicated to primary pulmonary hypertension, held in Geneva. Investigations into disease pathogenesis and classification led to the development of numerous therapies over the ensuing decades. While the impacts of the disease have been lessened due to treatments, the symptoms
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Risk stratification and treatment goals in pulmonary arterial hypertension. Eur. Respir. J. (IF 16.6) Pub Date : 2024-08-29 Fabio Dardi,Athénaïs Boucly,Raymond Benza,Robert Frantz,Valentina Mercurio,Horst Olschewski,Göran Rådegran,Lewis J Rubin,Marius M Hoeper
Risk stratification has gained an increasing role in predicting outcomes and guiding the treatment of patients with pulmonary arterial hypertension (PAH). The most predictive prognostic factors are three noninvasive parameters (World Health Organization functional class, 6-min walk distance and natriuretic peptides) that are included in all currently validated risk stratification tools. However, suffering
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Management of pulmonary hypertension in special conditions. Eur. Respir. J. (IF 16.6) Pub Date : 2024-08-29 Ioana R Preston,Luke S Howard,David Langleben,Mona Lichtblau,Tomas Pulido,Rogerio Souza,Karen M Olsson
Care of pulmonary hypertension (PH) patients in special situations requires insightful knowledge of the pathophysiology of the cardiopulmonary system and close interaction with different specialists, depending on the situation. The role of this task force was to gather knowledge about five conditions that PH patients may be faced with. These conditions are 1) perioperative care; 2) management of pregnancy;
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Treatment algorithm for pulmonary arterial hypertension. Eur. Respir. J. (IF 16.6) Pub Date : 2024-08-29 Kelly M Chin,Sean P Gaine,Christian Gerges,Zhi-Cheng Jing,Stephen C Mathai,Yuichi Tamura,Vallerie V McLaughlin,Olivier Sitbon
Pulmonary arterial hypertension leads to significant impairment in haemodynamics, right heart function, exercise capacity, quality of life and survival. Current therapies have mechanisms of action involving signalling via one of four pathways: endothelin-1, nitric oxide, prostacyclin and bone morphogenetic protein/activin signalling. Efficacy has generally been greater with therapeutic combinations
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Pulmonary hypertension associated with lung diseases. Eur. Respir. J. (IF 16.6) Pub Date : 2024-08-29 Oksana A Shlobin,Yochai Adir,Joan A Barbera,Vincent Cottin,Sergio Harari,Etienne-Marie Jutant,Joanna Pepke-Zaba,Hossein-Ardeschir Ghofrani,Richard Channick
Pulmonary hypertension (PH) associated with chronic lung disease (CLD) is both common and underrecognised. The presence of PH in the setting of lung disease has been consistently shown to be associated with worse outcomes. Recent epidemiological studies have advanced understanding of the heterogeneity of this patient population and shown that defining both the specific type of CLD as well as the severity
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Definition, classification and diagnosis of pulmonary hypertension. Eur. Respir. J. (IF 16.6) Pub Date : 2024-08-29 Gabor Kovacs,Sonja Bartolome,Christopher P Denton,Michael A Gatzoulis,Sue Gu,Dinesh Khanna,David Badesch,David Montani
Pulmonary hypertension (PH) is a haemodynamic condition characterised by elevation of mean pulmonary arterial pressure (mPAP) >20 mmHg, assessed by right heart catheterisation. Pulmonary arterial wedge pressure (PAWP) and pulmonary vascular resistance (PVR) distinguish pre-capillary PH (PAWP ≤15 mmHg, PVR >2 Wood Units (WU)), isolated post-capillary PH (PAWP >15 mmHg, PVR ≤2 WU) and combined post-
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Pathology and pathobiology of pulmonary hypertension: current insights and future directions. Eur. Respir. J. (IF 16.6) Pub Date : 2024-08-29 Christophe Guignabert,Jurjan Aman,Sébastien Bonnet,Peter Dorfmüller,Andrea J Olschewski,Soni Pullamsetti,Marlene Rabinovitch,Ralph T Schermuly,Marc Humbert,Kurt R Stenmark
In recent years, major advances have been made in the understanding of the cellular and molecular mechanisms driving pulmonary vascular remodelling in various forms of pulmonary hypertension, including pulmonary arterial hypertension, pulmonary hypertension associated with left heart disease, pulmonary hypertension associated with chronic lung disease and hypoxia, and chronic thromboembolic pulmonary
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Transplantation, bridging, and support technologies in pulmonary hypertension. Eur. Respir. J. (IF 16.6) Pub Date : 2024-08-29 Laurent Savale,Alberto Benazzo,Paul Corris,Shaf Keshavjee,Deborah Jo Levine,Olaf Mercier,R Duane Davis,John T Granton
Despite the progress made in medical therapies for treating pulmonary hypertension (PH), a subset of patients remain susceptible to developing a maladaptive right ventricular phenotype. The effective management of end-stage PH presents substantial challenges, necessitating a multidisciplinary approach and early identification of patients prone to acute decompensation. Identifying potential transplant
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Clinical trial design, end-points, and emerging therapies in pulmonary arterial hypertension. Eur. Respir. J. (IF 16.6) Pub Date : 2024-08-29 Jason Weatherald,Thomas R Fleming,Martin R Wilkins,Thomas M Cascino,Mitchell A Psotka,Roham Zamanian,Werner Seeger,Nazzareno Galiè,Mardi Gomberg-Maitland
Clinical trials in pulmonary arterial hypertension (PAH) have led to the approval of several effective treatments that improve symptoms, exercise capacity and clinical outcomes. In phase 3 clinical trials, primary end-points must reflect how a patient "feels, functions or survives". In a rare disease like PAH, with an ever-growing number of treatment options and numerous candidate therapies being studied
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Idiopathic pulmonary fibrosis mortality: update on trends in the modern treatment era Eur. Respir. J. (IF 16.6) Pub Date : 2024-08-22 Anna J. Podolanczuk, Ganesh Raghu
Extract Idiopathic pulmonary fibrosis (IPF) is a progressive lung disease caused by a combination of genetic and environmental factors [1]. Studies indicate that the global incidence and prevalence of IPF are increasing [2–8]. The reasons for this are not fully understood, but contributors include more widespread use of computed tomography (CT) imaging, improved recognition of the disease, an ageing
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Stepping down biologics in asthma: is it time to challenge the status quo? Eur. Respir. J. (IF 16.6) Pub Date : 2024-08-22 Sandhya Khurana, Steve N. Georas
Extract Rapid advances in biologic therapy have truly revolutionised the management of severe asthma in the past decade. The efficacy of these drugs has been undeniably demonstrated in randomised controlled trials (RCTs) and real-world studies, with reassuring safety data. Many patients with severe asthma have had their lives transformed after starting a biologic, which is an extremely gratifying result
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Titration of anti-IL-5 biologics in severe asthma: an open-label randomised controlled trial (the OPTIMAL study) Eur. Respir. J. (IF 16.6) Pub Date : 2024-08-22 Marianne Baastrup Soendergaard, Anne-Sofie Bjerrum, Linda Makowska Rasmussen, Sofie Lock-Johansson, Ole Hilberg, Susanne Hansen, Anna von Bulow, Celeste Porsbjerg
Extract The treatment of severe eosinophilic asthma has been revolutionised with the introduction of anti-interleukin (IL)-5 biological treatment. Anti-IL-5 biologics include mepolizumab, benralizumab and reslizumab, all of which target the IL-5 pathway; regulatory randomised controlled trials (RCTs) have shown that they are efficient in reducing exacerbation rates and the need for maintenance oral
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Mortality trends in idiopathic pulmonary fibrosis in Europe between 2013 and 2018 Eur. Respir. J. (IF 16.6) Pub Date : 2024-08-22 Francesca Gonnelli, Martina Bonifazi, Richard Hubbard
Extract Previous studies in the UK [1] and Europe [2] have shown that the mortality from idiopathic pulmonary fibrosis (IPF) has increased over time, but up-to-date studies using consistent methodology are limited. The last reliable European estimate of IPF mortality was provided by Marshall et al. [2] using World Health Organization (WHO) data. Their study showed that the mortality rate from IPF increased
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Reassessing Halm's clinical stability criteria in community-acquired pneumonia management. Eur. Respir. J. (IF 16.6) Pub Date : 2024-08-22 Simone Bastrup Israelsen,Markus Fally,Pernille Brok Nielsen,Lilian Kolte,Kasper Karmark Iversen,Pernille Ravn,Thomas Benfield
INTRODUCTION Halm's clinical stability criteria have long guided antibiotic treatment and hospital discharge decisions for patients hospitalised with community-acquired pneumonia (CAP). Originally introduced in 1998, these criteria were established based on a relatively small and select patient population. Consequently, our study aims to reassess their applicability in management of CAP in a contemporary
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The treatment of latent tuberculosis infection in migrants in primary care versus secondary care. Eur. Respir. J. (IF 16.6) Pub Date : 2024-08-22 Matthew Burman,Dominik Zenner,Andrew J Copas,Lara Goscé,Hassan Haghparast-Bidgoli,Peter J White,Vicky Hickson,Opal Greyson,Duncan Trathen,Richard Ashcroft,Adrian R Martineau,Ibrahim Abubakar,Christopher J Griffiths,Heinke Kunst
BACKGROUND Control of latent tuberculosis infection (LTBI) is a priority in the World Health Organization strategy to eliminate tuberculosis (TB). Many high-income low TB incidence countries have prioritised LTBI screening and treatment in recent migrants. We tested whether a novel model of care, based entirely within primary care, was effective and safe as compared to secondary care. METHODS This
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Pharmacological and pre-clinical safety profile of rSIV.F/HN, a hybrid lentiviral vector for cystic fibrosis gene therapy. Eur. Respir. J. (IF 16.6) Pub Date : 2024-08-22 Alena Moiseenko,Anthony Sinadinos,Ana Sergijenko,Kyriel Pineault,Aarash Saleh,Konradin Nekola,Nathalie Strang,Anastasia Eleftheraki,A Christopher Boyd,Jane C Davies,Deborah R Gill,Stephen C Hyde,Gerry McLachlan,Tim Rath,Michael Rothe,Axel Schambach,Silke Hobbie,Michael Schuler,Udo Maier,Matthew J Thomas,Detlev Mennerich,Manfred Schmidt,Uta Griesenbach,Eric W F W Alton,Sebastian Kreuz
RATIONALE AND OBJECTIVE Cystic fibrosis (CF) is caused by mutations in the CF Transmembrane Conductance Regulator (CFTR) gene. CFTR modulators offer significant improvements, but approximately 10% of patients remain nonresponsive or are intolerant. This study provides an analysis of rSIV.F/HN, a lentiviral vector optimized for lung delivery, including CFTR protein expression, functional correction
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The end of the road for blood RNA biomarkers as triage tests for symptomatic pulmonary tuberculosis among spontaneous sputum producers? Eur. Respir. J. (IF 16.6) Pub Date : 2024-08-15 James Greenan-Barrett, Rishi K. Gupta, Mahdad Noursadeghi
Extract In 2022, an estimated 3.1 million people with tuberculosis (TB) remained undiagnosed [1] despite the global roll-out of rapid molecular tests for Mycobacterium tuberculosis, such as Xpert Ultra and Truenat MTB plus [2]. In pulmonary TB, these tests rely on the availability of a respiratory sample. Sputum induction or invasive sampling are required in sputum-scarce individuals, but often unavailable
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Lunging through the pollution–exercise paradox: clean air during Olympic and Paralympic Games and beyond Eur. Respir. J. (IF 16.6) Pub Date : 2024-08-15 Christopher Carlsten, Isabella Annesi-Maesano
Extract The 2024 Olympic Games and Paralympic Games lead us, as pulmonologists, to reflect on the sport, air pollution and respiratory health triad, and potential compromises at their intersection. Prompted by the scrutiny for any factor that can tip the highly competitive scale of the Games, this theme is of interest to all citizens well beyond the spotlight and glory of these dramatic events.