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

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

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

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

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

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

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

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

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

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

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]

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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;

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

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

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-

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

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

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

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

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

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

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

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

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

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

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

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.

Extract Airway inflammation drives both the development of bronchiectasis and results in the dominant clinical features. Many aetiologies of bronchiectasis, including previous infection, COPD, rheumatoid arthritis, allergic bronchopulmonary aspergillosis and toxic exposures, cause airway inflammation that damages the bronchial wall, which then allows chronic infection to be established. When bronchiectasis

Extract Based on current international guidelines, pulmonary arterial wedge pressure (PAWP) is critical for differentiating between pre- and post-capillary pulmonary hypertension (PH) and plays an important role in the diagnosis of left heart failure [1, 2]. The current PAWP threshold to define post-capillary PH is >15 mmHg, measured by right heart catheterisation (RHC) in the supine position [1].

Background Multiple host blood transcriptional signatures have been developed as non-sputum triage tests for tuberculosis (TB). We aimed to compare the diagnostic performance of 20 blood transcriptomic TB signatures for differentiating between symptomatic patients who have TB versus other respiratory diseases (ORD). Methods As part of a nested case–control study, individuals presenting with respiratory

Interstitial lung diseases (ILD) are a heterogeneous group of rare diffuse diseases affecting the lung parenchyma in children and adults. Childhood interstitial lung diseases (chILD) are often diagnosed at very young age, affect the developing lung, and can have different presentations and prognosis compared to adult forms of these diseases. In addition, chILD in many cases may apparently remit, and

Rationale The inflammasome is a key regulatory complex of the inflammatory response leading to interleukin-1β (IL-1β) release and activation. IL-1β amplifies inflammatory responses and induces mucus secretion and hyperconcentration in other diseases. The role of IL-1β in bronchiectasis has not been investigated. Objectives To characterise the role of airway IL-1β in bronchiectasis, including the association

Asthma is a prevalent pulmonary disease that affects more than 300 million people worldwide and imposes a substantial economic burden. While medication can effectively control symptoms in some patients, severe asthma attacks, driven by airway inflammation induced by environmental and infectious exposures, continue to be a major cause of asthma-related mortality. Heterogeneous phenotypes of asthma include

It is increasingly recognised that diverse genetic respiratory disorders present as severe pulmonary hypertension (PH) in the neonate and young infant, but many controversies and uncertainties persist regarding optimal strategies for diagnosis and management to maximize long-term outcomes. To better define the nature of PH in the setting of developmental lung disorders (DEVLD), in addition to the common

BACKGROUND Pulmonary alveolar proteinosis (PAP) is a rare syndrome caused by several distinct diseases leading to progressive dyspnoea, hypoxemia, risk of respiratory failure and early death due to accumulation of proteinaceous material in the lungs. Diagnostic strategies may include computed tomography (CT) of the lungs, bronchoalveolar lavage, evaluation of antibodies against granulocyte macrophage

BACKGROUND Prior exacerbation history is used to guide initial maintenance therapy in chronic obstructive pulmonary disease (COPD); however, the recommendations were derived from patients already diagnosed and treated. METHOD We assessed the rates of moderate (i.e. treated with antibiotics and/or systemic corticosteroids) and severe (i.e. hospitalised) exacerbations in the year following diagnosis

BACKGROUND The real-world consequences of a Philips/Respironics recall for positive airway pressure (PAP) devices distributed between 2009 and 2021 are unknown. METHODS We conducted a retrospective population-based study using health administrative databases (Ontario, Canada) on all new adult PAP users identified through the provincial funding system, free of cancer at baseline, who initiated (claimed)