Extract Pulmonary arterial hypertension (PAH) is a severe progressive disease characterised by obliterative vascular remodelling and increased resistance in small and medium-sized pulmonary arteries (PAs). Contributing factors to PAH pathogenesis include genetic mutations such as those in bone morphogenetic protein receptor type 2 (BMPR2), perivascular inflammation, systemic immune dysregulation, and

Extract We commend Khor et al. [1] for their important article that proposes a framework for delivering comprehensive care in patients with interstitial lung disease (ILD) based on treatable traits.

Background In the phase 3 STELLAR trial, sotatercept, an investigational first-in-class activin signalling inhibitor, demonstrated beneficial effects on 6-min walk distance and additional efficacy endpoints in pre-treated participants with pulmonary arterial hypertension (PAH). Methods This post hoc analysis evaluated data from right heart catheterisation (RHC) and echocardiography (ECHO) obtained

Extract With increasing climate change, all sources of greenhouse gases must be considered. Hydrofluorocarbon (HFC) propellants are used in all pressurised metered dose inhalers (pMDIs) and, although they are not as ozone-depleting as the earlier chlorofluorocarbons (CFCs), they are potent greenhouse gases and there is a general ask for reduction in their use [1]. In this respect, respiratory doctors

Extract Efforts to curb the tuberculosis (TB) pandemic remain hindered by a lack of objective measures to quantify disease severity and track treatment success that are valid in both HIV-1-infected and -uninfected TB patients. Ralph et al. [1] developed a promising radiographic scoring system, with baseline scores being predictive of sputum smear conversion at 2 months, but it is reliant on skilled

Extract A hallmark of cystic fibrosis (CF) is build-up of thick tenacious mucus, leading to progressive cycles of infection and inflammation. Airway clearance techniques (ACTs) are a crucial component of CF management, designed to assist individuals in effectively clearing the thick and sticky mucus that accumulates in their airways. These techniques aim to enhance lung function, reduce the risk of

Extract The Global Initiative for Chronic Obstructive Lung Disease (GOLD) 2023 report on the diagnosis and management of COPD includes important changes to the pharmacological treatment recommendations [1]. The ABCD classification, a tool to guide inhaled treatment in newly diagnosed patients based on symptoms and exacerbation risk, has been replaced by ABE; the high exacerbation risk groups C and

Extract The intrinsic variability of asthma across time is a well-known feature of the disease and stratifying disease severity is an integral component of asthma management [1]. Severe asthma is estimated to comprise up to 10% of the asthmatic population and is associated with substantial socioeconomic burden, with more than half of asthma-related healthcare costs being attributed to this level of

Extract Good lung health is a critical component of overall health and well-being. However, determining what constitutes good lung health can be challenging, as it depends on both population-level and individual-level factors. Surprisingly, to the best of our knowledge, good lung health has not been yet defined [1, 2]. Here we explore different ways of defining good lung health and suggest some practical

Extract We read with interest the systematic review by Ivanova et al. [1], recently published in the European Respiratory Review, highlighting significant long-term lung function impairment experienced by people that survive tuberculosis (TB). Lung function impairment is a well-described characteristic of post-TB lung disease (PTLD), a condition that spans the spectrum of obstructive airway disease

Extract In the most recent 2023 report, the Global Initiative for Chronic Obstructive Lung Disease (GOLD) committee recommends to no longer consider the degree of dyspnoea when prescribing the very initial treatment of patients with COPD who have a history of exacerbations [1]. Thus, the former grades C and D are now regrouped into a single new "group E", defined exclusively in terms of prior exacerbations:

Extract Interstitial lung disease (ILD) is a heterogeneous group of pulmonary disorders, characterised by diffuse parenchymal lung infiltration. Some ILD patients can develop a progressive fibrosing phenotype characterised by worsening fibrotic changes, decline in lung function over time, worsening symptoms and quality of life, and early mortality. Patients with this inexorable progression of disease

Background Cystic fibrosis (CF) is commonly characterised by thick respiratory mucus. From diagnosis, people with CF are prescribed daily physiotherapy, including airway clearance techniques (ACTs). ACTs consume a large proportion of treatment time, yet the efficacy and effectiveness of ACTs are poorly understood. This study aimed to evaluate associations between the quality and quantity of ACTs and

Background Current guidelines recommend 20–40 mg·day–1 of oral prednisolone for treating pulmonary sarcoidosis. Whether the higher dose (40 mg·day–1) can improve outcomes remains unknown. Methods We conducted an investigator-initiated, single-centre, open-label, parallel-group, randomised controlled trial (ClinicalTrials.gov identifier NCT03265405). Consecutive subjects with pulmonary sarcoidosis were

Background There is limited evidence on the pathways leading to severe asthma and we are presently unable to effectively predict the progression of the disease. We aimed to describe the longitudinal trajectories leading to severe asthma and to describe clinical events preceding disease progression in a nationwide population of patients with severe asthma. Methods We conducted an observational study

One of the author names was presented …

As part of the August issue, the European Respiratory Journal presents the latest in its series of podcasts. Deputy Chief Editor Don Sin interviews Prof. Bartolome Celli about his editorial on simplifying pharmacotherapy for patients with COPD.

Extract The World Health Organization (WHO) has warned that climate change is the biggest global threat to humanity in the 21st century [1, 2]. By 2050, climate change is expected to cause at least 250 000 deaths every year globally due to climate-related heat stress, malnutrition, malaria and diarrhoea [3]. An additional health burden will arise from more indirect climate-related paths, including

Extract The heart and lungs must work in close harmony for the cardiopulmonary system to function effectively. Numerous physiological pathways connect these two organs, leading to frequent coexistence of cardiovascular and pulmonary illnesses. In particular, it is now recognised that individuals with COPD have considerably higher rates (typically two to five times) of cardiovascular comorbidities,

Extract Tuberculosis remains a global problem, and the COVID-19 pandemic and ongoing conflicts, such as the war in Ukraine, negatively impact the progress made and milestones set in the End TB Strategy [1]. Therefore, additional efforts and resources are required to mitigate the impact of these events and to ensure that global tuberculosis targets are achieved [2]. One of the research priorities set

Background: Phase 2a trials in tuberculosis typically use early bactericidal activity (EBA), the decline in sputum CFU over 14 days, as the primary end-point for testing the efficacy of drugs as monotherapy. However, the cost of phase 2a trials can range from USD 7 million to USD 19.6 million on average, while >30% of drugs fail to progress to phase 3. Better utilising pre-clinical data to predict

Background Despite COPD being a risk factor for cardiovascular disease (CVD) and knowing that risk stratification for CVD primary prevention is important, little is known about the real-world risk of CVD among people with COPD with no history of CVD. This knowledge would inform CVD management for people with COPD. The current study aimed to examine the risk of major adverse cardiovascular events (MACE)

Extract COPD and interstitial lung diseases (ILDs) are increasing in prevalence and are associated with a high disease burden and mortality [1, 2]. Chronic respiratory diseases are the third leading cause of death according to global health metrics [3], with COPD being the primary cause. It should be realised that, in COPD, many patients die of comorbid conditions and not of COPD [4]. ILD, which comprises

Several current guidelines/strategies outline a treatment approach to asthma, which primarily consider the goals of improving lung function and quality of life and reducing symptoms and exacerbations. They suggest a strategy of stepping up or down treatment, depending on the patient's overall current asthma symptom control and future risk of exacerbation. While this stepwise approach is undeniably

Frailty is a complex, multidimensional syndrome characterised by a loss of physiological reserves that increases a person's susceptibility to adverse health outcomes. Most knowledge regarding frailty originates from geriatric medicine; however, awareness of its importance as a treatable trait for people with chronic respiratory disease (including asthma, COPD and interstitial lung disease) is emerging

Review of simple clinical and physiological data can help stratify COPD severity and guide the pharmacotherapy most likely to improve patient outcomes. Supervision of response, adherence and correct use of medications helps adjust therapy. https://bit.ly/3P3J5gf

There is increased awareness of palliative care needs in people with COPD or interstitial lung disease (ILD). This European Respiratory Society (ERS) task force aimed to provide recommendations for initiation and integration of palliative care into the respiratory care of adult people with COPD or ILD. The ERS task force consisted of 20 members, including representatives of people with COPD or ILD

Extract Pulmonary arterial hypertension (PAH) is a rare disease characterised by remodelling of small pulmonary vessels, which results in a progressive increase in right ventricular afterload [1]. Most of the symptoms and signs of PAH are attributable to impaired right heart function. However, patients with PAH also show variable alterations of their lung diffusion capacity for carbon monoxide (DLCO)

Extract Lower respiratory tract infections caused by viruses are the most common reason for hospitalisation in young children [1]. Respiratory virus infections have also been associated with increased risk for diagnosis of asthma later in childhood [2, 3]. Investigating the mechanistic underpinnings that link early life viral infections and subsequent development of asthma remains an important and

Extract All smokers develop lung inflammation, but patients with COPD suffer from profound alterations of both their innate and adaptive immune compartments [1]. Such alterations predispose to acute exacerbations of COPD, which not only lead to an acute worsening of respiratory symptoms but are also associated with increased lung function decline and increased morbidity and mortality [2, 3]. These

Extract In people with cystic fibrosis (CF), CF transmembrane conductance regulator (CFTR) protein dysfunction in the lower airways results in dehydrated airway secretions and impaired mucociliary clearance [1]. This facilitates proliferation and persistence of bacteria in the airways and the resultant cycle of chronic infection and inflammation drives structural lung damage and decline in lung function

Background Single-inhaler dual bronchodilators are now recommended as initial treatment of COPD for patients with multiple exacerbations or with moderate or severe dyspnoea. It is unclear whether there are differences in effectiveness among commonly used dual bronchodilators. Methods We identified a cohort of COPD patients, aged ≥40 years, treated during 2017–2020, from the UK Clinical Practice Research

Background Severe bronchiolitis (i.e. bronchiolitis requiring hospitalisation) during infancy is a major risk factor for childhood asthma. However, the exact mechanism linking these common conditions remains unclear. We examined the longitudinal relationship between nasal airway miRNAs during severe bronchiolitis and the risk of developing asthma. Methods In a 17-centre prospective cohort study of

Extract Asthma and obesity are prevalent disorders with significant impact on public health. Obesity is a reported risk factor for the development of asthma and is associated with increased exacerbation rates, medication usage and higher symptoms scores in patients with asthma. It should therefore be addressed and treated as an important asthma related comorbidity [1]. Oral corticosteroids (OCS) are

Background Cardiac magnetic resonance (CMR) is the gold standard technique to assess biventricular volumes and function, and is increasingly being considered as an end-point in clinical studies. Currently, with the exception of right ventricular (RV) stroke volume and RV end-diastolic volume, there is only limited data on minimally important differences (MIDs) reported for CMR metrics. Our study aimed

Background Recent studies demonstrated that the triple combination cystic fibrosis transmembrane conductance regulator (CFTR) modulator therapy elexacaftor/tezacaftor/ivacaftor (ETI) improves lung function and reduces pulmonary exacerbations in cystic fibrosis (CF) patients with at least one F508del allele. However, effects of ETI on downstream consequences of CFTR dysfunction, i.e. abnormal viscoelastic

Background Virus infections drive COPD exacerbations and progression. Antiviral immunity centres on the activation of virus-specific CD8+ T-cells by viral epitopes presented on major histocompatibility complex (MHC) class I molecules of infected cells. These epitopes are generated by the immunoproteasome, a specialised intracellular protein degradation machine, which is induced by antiviral cytokines

Interstitial lung disease (ILD) is a diverse group of inflammatory and fibrotic lung conditions causing significant morbidity and mortality. A multitude of factors beyond the lungs influence symptoms, health-related quality of life, disease progression and survival in patients with ILD. Despite an increasing emphasis on multidisciplinary management in ILD, the absence of a framework for assessment

Background It is currently unknown if disease severity modifies response to therapy in pulmonary arterial hypertension (PAH). We aimed to explore if disease severity, as defined by established risk-prediction algorithms, modified response to therapy in randomised clinical trials in PAH. Methods We performed a meta-analysis using individual participant data from 18 randomised clinical trials of therapy

In the Materials and methods, Data collection and variables subsection of this manuscript, the formulae used to calculate the Model …

As part of the July issue, the European Respiratory Journal presents the latest in its series of podcasts. Chief Editor James Chalmers interviews Prof. Daiana Stolz about HISTORIC, a randomised, placebo-controlled, double-blind, investigator-initiated trial of airway smooth muscle area to predict steroid responsiveness in COPD patients receiving triple therapy.

Extract We thank J.J. Lawrence and R. Kumar for their comments, which express concerns regarding our findings of a causal effect of gastro-oesophageal reflux disease (GORD) on idiopathic pulmonary fibrosis (IPF) [1], as this gives us the opportunity to further discuss the issue of confounding in Mendelian randomisation (MR).

Extract Picture yourself in a seat at the movies. The room is dark. The screen is also dark or shows a benign scene. There is no music, or a gentle melody. Before you see anybody on the screen, you hear someone breathing. Depending on the sound of this respiration, you might feel relaxed, aroused or threatened. You might identify breathlessness. You might worry for the person who breathes. You might

Extract Despite decades of research and clinical practice, we continue to debate the age-old question of how inhaled corticosteroids (ICS) fit into the treatment algorithms for patients living with COPD. For each patient, the benefits of reducing their exacerbation frequency must be weighed against their risk of pneumonia and oropharyngeal complications. Current clinical guidelines suggest restricting

Extract A group of researchers revealed a strong correlation between the sales of ice cream and the number of deaths by drowning, and asserted that they searched the data and did not discover any confounding variables. They went on to conclude that the relationship was causal, indicating that ice cream sales could be altered to reduce the number of drowning incidents. Most readers concurred with the

Extract There are flaws to this analysis that threaten the claim of causality. The main one being the fact that many of the single nucleotide polymorphisms used to instrument GORD are strongly associated with body composition.

Extract The recently published technology assessment of continuous positive airway pressure (CPAP), prepared for the Agency for Healthcare Research and Quality (AHRQ) of the US Department of Health and Human Services and entitled Long-Term Health Outcomes in Obstructive Sleep Apnea, has created considerable angst within the sleep medicine community with its conclusion that randomised trials "do not

Extract The threat of tropical cyclones in East Asia is a critical public health issue and a key reason for constructing adaptation strategies for the challenges of climate change. Some studies have indicated general evidence of the relationship between tropical cyclone exposure and the increased incidences of various kinds of diseases [1–3]. Specifically, there are several pathways for the impact

Extract Pulmonary hypertension (PH) is a frequent complication of COPD, with a poor prognosis, especially in its severe form [1]. Accordingly, current guidelines distinguish patients with severe PH from those with moderate PH [2]. Patients with COPD and severe PH often present with worse hypoxaemia than those with moderate PH, despite having milder airflow obstruction [3–5]. The mechanisms underlying

Background Although inhaled corticosteroids (ICS) are highly effective in asthma, they provide significant, but modest, clinical benefit in COPD. Here, we tested the hypothesis that high bronchial airway smooth muscle cell (ASMC) area in COPD is associated with ICS responsiveness. Methods In this investigator-initiated and -driven, double-blind, randomised, placebo-controlled trial (HISTORIC), 190

Extract Growing evidence supports the contention that immune dysregulation and autoimmunity predispose and contribute to the pathological remodelling that characterise pulmonary arterial hypertension (PAH) [1, 2]. PAH is a common complication of connective tissue diseases (CTDs), especially systemic sclerosis (SSc) and systemic lupus erythematosus (SLE) [3]. In addition, idiopathic PAH (IPAH) patients

Extract A recently published report prepared for the Agency for Healthcare Research and Quality (AHRQ) of the US Department of Health and Human Services concluded that the published evidence from randomised controlled trials (RCTs) mostly does not support a benefit from continuous positive airway pressure (CPAP) therapy for obstructive sleep apnoea (OSA) in outcomes that include cardiometabolic comorbidity

Airway clearance techniques (ACTs) are part of the main management strategy for patients with bronchiectasis. Despite being a priority for patients, accessibility, implementation and reporting of ACTs are variable in clinical settings and research studies. This European Respiratory Society statement summarises current knowledge about ACTs in adults with bronchiectasis and makes recommendations to improve

Extract Breathlessness is a common and persistent symptom for those living with chronic lung conditions and advanced disease [1]. It can be distressing for those who experience it, and often results in anxiety, physical inactivity and a poorer quality of life [2, 3]. Additionally, it impacts significantly on those who are close, including friends and family, and is associated with a considerable care

BACKGROUND Epithelial damage, repair and remodelling are critical features of chronic airway diseases including chronic obstructive pulmonary disease (COPD). Interleukin (IL)-33 released from damaged airway epithelia causes inflammation via its receptor, serum stimulation-2 (ST2). Oxidation of IL-33 (IL-33ox) to a non-ST2-binding form is thought to limit its activity. We investigated whether IL-33ox

Extract Ageing is often associated with chronic remodelling, which is a key feature of many human diseases, including chronic respiratory diseases such as lung fibrosis. Idiopathic pulmonary fibrosis (IPF) is the most common form of pulmonary fibrosis and is defined as a specific form of chronic, progressive fibrosing interstitial pneumonia of unknown cause. IPF patients have an overall median survival

Extract Improving lung function, preventing pulmonary exacerbations, and reducing symptoms are primary objectives in the development of inhaled therapeutics for cystic fibrosis (CF) lung disease. Hypertonic saline (HS) emerged as a simple and cost-effective treatment that achieved these goals early in the rapidly evolving era of CF drug development. HS addresses an important pathophysiological abnormality

Extract Air pollution may elevate the risk of infection and severity (e.g. intensive care unit (ICU) admission and death) from coronavirus disease 2019 (COVID-19) by modifying host susceptibility to infection and disease severity, and by elevating the risk of comorbidities [1]. An emerging body of toxicological and experimental evidence has provided insights on how acute [2] and chronic [3] air pollution

Extract Increased age is a strong risk factor for mortality in patients with coronavirus disease 2019 (COVID-19) [1, 2]. Risk of death increases 1.5 times for every 10 years [1]. Among the 3.4 million people who died worldwide up to May 2023 from COVID-19, 2.4 million (71%) were people older than 65 years [3].

Extract Chronic thromboembolic pulmonary hypertension (CTEPH) is a severe late complication of acute pulmonary embolism (PE) [1, 2]. The earlier CTEPH is diagnosed, the better the prognosis for CTEPH patients, both in terms of survival and quality of life [2–4]. Even with this knowledge, the diagnostic delay of CTEPH still remains considerably long with a reported median duration of 14.1 months in