ERJ Open Research最新文献

Aim: Secondary spontaneous pneumothorax (SSP) most commonly occurs in older patients with known underlying lung disease. Many are frail, but the effect of frailty on outcomes has not been explored previously. This study aims to evaluate the association between frailty and healthcare outcomes in patients with SSP.

Methods: Patients with SSP were identified from the national Secure Anonymised Information Linkage databank. Frailty status was assessed using the electronic frailty index. The primary outcome was time from diagnosis to all-cause mortality. Secondary outcomes included time from diagnosis to disease-specific mortality and admission to hospital. Data were analysed using a multilevel Cox proportional hazards regression model, adjusted for age, sex, Welsh Index of Multiple Deprivation, smoking status and comorbidities.

Results: Our search identified 3535 individuals diagnosed with SSP between 1 January 2005 and 1 March 2023. By the end of the study, 2102 (59.6%) participants had died with a median follow-up of 683 days (interquartile range 159-1650 days). There was an increasing risk of mortality for those with mild (adjusted hazard ratio (aHR) 1.24, 95% CI 1.10-1.39), moderate (aHR 1.46, 95% CI 1.25-1.70) and severe (aHR 1.83, 95% CI 1.43-2.32) frailty compared to fit individuals. There was also an association between frailty and time to first all-cause hospitalisation, but not disease-specific hospitalisation.

Conclusions: Frailty status at diagnosis was an independent predictor of all-cause mortality in patients with SSP. This demonstrates the importance of assessing frailty status to enable clinicians to provide optimised care and make informed decisions about management of patients with SSP.

Background: The New International Chronic Thromboembolic Pulmonary Hypertension (CTEPH) Registry, conducted between 2015 and 2019, revealed the significant impact of pulmonary endarterectomy (PEA) and balloon pulmonary angioplasty (BPA) on the long-term survival of CTEPH patients. The objective of the current pre-specified analysis was to evaluate the effect of medical treatment, including the approved oral guanylate cyclase stimulator (sGC) riociguat, in patients with or without mechanical intervention.

Methods: 1009 newly diagnosed patients were included in the study, with recruitment conducted at 34 centres across 20 countries. At the outset of the registry, sGC was available in 85% of the countries involved.

Results: 52% of all patients included were treated with pulmonary hypertension (PH) drugs. The proportion of patients receiving PH drugs was 38% among the 605 patients who underwent PEA, 78% among the 185 patients who underwent BPA and 76% among the 219 remaining patients who did not undergo PEA or BPA. In the "BPA" and "no PEA/BPA" groups, the 3-year survival was superior in patients who received sGC in comparison with other PH drugs. Cox regression confirmed that sGC treatment was associated with reduced mortality in the global cohort, in the BPA group and in the "no PEA/BPA" group.

Conclusion: This international CTEPH registry suggests that, although an increasing proportion of patients with CTEPH benefited from mechanical treatment, medical treatment with sGC may be associated with a survival advantage in patients undergoing BPA or no intervention. These observations confirm the results of previous randomised controlled trials in a real-world setting.

Introduction: Early cystic fibrosis (CF) lung disease monitoring is crucial for understanding responses to therapy and preventing progressive pulmonary decline. Structural lung disease (SLD) is a major cause of pulmonary decline in CF. We aimed to identify metabolites in CF bronchoalveolar lavage (BAL) associated with and predictive of SLD.

Methods: We applied untargeted metabolomics to 84 BAL samples from a cross-sectional cohort of 67 clinically stable children with CF aged 1-5 years across two sites. We used a linear mixed-effects model to select metabolites associated with SLD, BAL neutrophils, and myeloperoxidase (MPO) and neutrophil elastase (NE) activities. An independent longitudinal cohort of infants who either did or did not exhibit bronchiectasis by age 9 years was analysed to determine whether metabolites associated with SLD could also predict future lung disease.

Results: In the cross-sectional cohort, 10 BAL metabolites, including methionine sulfoxide (MetO), ornithine and N-acetylmethionine (NAcMet), were associated with SLD, neutrophils, MPO and NE. The percentage of methionine oxidation (%OxMet) and the arginine-ornithine ratio were also associated with these outcomes. In the longitudinal cohort, MetO, %OxMet, ornithine and NAcMet predicted bronchiectasis development, and MetO plus ornithine was a more sensitive predictor than either alone. At age 0-2 years, these metabolites outperformed established biomarkers such as NE, MPO and interleukin-8.

Conclusions: We identified BAL metabolites associated with SLD, MPO and NE, detectable in the earliest stages of CF. MetO, %OxMet, NAcMet and ornithine predicted bronchiectasis development, outperforming established biomarkers. These metabolites reflect oxidising, proteolytic and other enzymatic activities of neutrophils, highlighting potential therapeutic avenues.

Background: Transition of adolescents with chronic diseases to adult care is at risk of health complications and loss of medical follow-up. There is currently no official general consensus specific to rare pulmonary diseases. We aimed at setting up a consensus of experts to establish a consensus statement for the transition of patients with rare pulmonary diseases in France.

Methods: We sought consensus using a three-round Delphi method, involving the French rare lung disease network. Statements were submitted to a panel of 38 experts (including nurses, patients, physiotherapists, specialised and general physicians, social workers and psychologists). A statement was validated if 80% of the respondents rated it as 7 or more on a Likert scale.

Results: We received all three completed surveys from 37 respondents. We identified 77 key elements that reached consensus to be included in future guidelines. The main topics discussed correspond to the future guidelines' structure, as follows: Transition overview and main objectives; Subjects to discuss with the patient during transition; Practical aspects of consultations during transition and transfer; and early follow-up in adult care. The main remaining ideas were: 1) to coordinate global care for each patient; 2) to formalise transfer; and 3) to integrate patients' will and needs into their care in order to support their empowerment.

Conclusion: This study has established key elements to a successful transition for patients with rare pulmonary disease by a multidisciplinary panel of experts. We achieved consensus on a formalised transition pathway to guarantee a successful transition for patients and their families, and also for healthcare professionals.

Background: Elexacaftor/tezacaftor/ivacaftor (ETI) has impacted the ability for people with cystic fibrosis (PwCF) to spontaneously expectorate sputum, leading to lower respiratory sampling rates and infection detection challenges. Home sampling may permit a potential strategy for fungal detection in PwCF.

Methods: We conducted a prospective decentralised cohort study of PwCF to test the feasibility of home sputum collection and ambient temperature transport for Aspergillus fumigatus (Af) detection. Participants collected and shipped weekly sputum samples from home to the laboratory for fungal culture and completed electronic questionnaires. Descriptive statistics were calculated for patient factors, sputum characteristics and Af-positive cultures. We used a generalised estimating equations model to determine the association between highly effective modulator therapy (HEMT) and sputum volume.

Results: We enrolled 76 adults with cystic fibrosis (CF) with a median (interquartile range) forced expiratory volume in 1 s (FEV₁) % predicted of 72.5% (53.8-86.3). 60 (79%) were on ETI and 44 (58%) had a history of Aspergillus. 70 (92%) successfully collected and shipped three or more sputum samples. Of 284 samples received, 83% arrived within one day. Sputum collection was reported as easy in 83 (29%) and somewhat easy in 114 (40%) collection events. Sputum volume from PwCF on HEMT was 36% lower than those not on HEMT (36%, 95% CI 3-58; p=0.03), adjusting for covariates. Af was detected in 205 (73%) of home sputum samples.

Conclusion: Home sputum collection is feasible in adults with CF. Af was detected in remotely collected sputum samples. Further work to assess the validity of home sputum samples in PwCF is necessary to determine the value of remote specimens in clinical and research settings.

Background: The extent to which airflow obstruction, a key feature of COPD, can be already present in early adulthood is unclear. We investigated the prevalence of airflow obstruction in young adults across European populations.

Methods: We identified 48 612 individuals aged 20-40 years across eight population-based European cohorts in the Chronic Airway Diseases Early Stratification (CADSET) collaboration and applied two commonly used definitions of airflow obstruction: pre-bronchodilator forced expiratory volume in 1 s (FEV₁)/forced vital capacity (FVC) <0.70 and below the lower limit of normal (LLN). We explored how the prevalence of airflow obstruction according to both criteria was related to age, sex and smoking.

Results: Airflow obstruction prevalence increased with increasing age from 2.3% in those aged 20-24.9 years to 6.3% in those aged 35-39.9 years according to FEV₁/FVC <0.70, and from 7.3% to 8.3% according to FEV₁/FVC versus 7.5% in females, and up to 9.0% in ever-smokers versus 6.9% in never-smokers. Difference in prevalence of airflow obstruction between FEV₁/FVC <0.70 and

Conclusion: Up to 8% of young adults across Europe have airflow obstruction; its cause and its role in prior, concurrent and future airway disease merit further investigation.

The transition to race-neutral reference equations may lead to a loss of accuracy in certain respiratory diseases. Further studies are required to clarify their scope of application. https://bit.ly/3GLOC8W.

Is autoimmune screening impactful in bronchiectasis? Not really. https://bit.ly/3HGVXY6.

Background: Chronic cough (CC) can impact daily life and persist for years. Its prevalence varies globally, but whether quality of life in CC also varies across regions is unknown. This study investigates the association of CC with mental and physical component scores of the 12-item Short Form Health Survey reflecting health-related quality of life in a multinational study.

Methods: We analysed data from 19 642 adults (≥40 years), recruited between 2 January 2003 and 26 December 2016 in 31 sites (25 countries) from the Burden of Obstructive Lung Disease study, who provided information on quality of life and CC. We assessed associations using linear regression, adjusted for confounders, and used random-effects meta-analysis to examine differences by sex and gross national income.

Findings: Overall, lower mental (-1.42, 95% CI -2.11 to -0.73; I²=32.7%) and physical (-2.59, 95% CI -3.22 to -1.96; I²=40.1%) health scores were associated with CC. The association between physical health score and CC did not materially differ between sexes or gross national income. In males, physical health seems to be more affected by CC amongst those living in low- and middle-income countries (LMICs). In females, mental health also seems to be more affected by CC amongst those living in LMICs.

Interpretation: CC impairs health-related quality of life globally. However, it appears that physical health in males and mental health in females living in LMICs may be particularly affected by CC. These findings support the need to consider CC as a target for specific interventions to attenuate its burden on health and the economy.

Background: Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive and ultimately fatal lung disease. Updated global mortality data, especially from underexplored countries, are limited. This study aimed to understand the current global trends in IPF-associated mortality rates.

Methods: This observational study used the World Health Organization Mortality Database to analyse data stratified by sex, age and geographic region, encompassing 64 countries between 2001 and 2022. IPF was defined according to the International Code for Diseases-10 code J84.1. Crude and age-standardised mortality rates per 100 000 individuals were calculated to estimate long-term mortality trends. Mortality rates were calculated by dividing IPF-associated deaths by the corresponding population, with age-specific rates determined for each 5-year age group. Trends in the 2001-2022 period were analysed using a locally weighted regression model, and the average annual percentage change in mortality rates between 2010 and 2022 was estimated using joinpoint regression analysis.

Results: Overall, 874 998 deaths associated with IPF were analysed. The LOESS-smoothed crude mortality rate increased from 2.10 (95% confidence interval (CI) 1.77-2.43) per 100 000 in 2001 to 3.14 (95% CI 2.71-3.57) per 100 000 by 2022. The LOESS-smoothed age-standardised mortality rates increased overall, peaking at 1.59 (95% CI 1.51-1.67) per 100 000 in 2018 and declining slightly to 1.57 (95% CI 1.35-1.79) per 100 000 in 2022. Mortality was higher among males than females; furthermore, 87.5% of deaths occurred in individuals aged ≥65 years. Mortality rates were highest among the American population, with a notable increase in Latin American countries.

Conclusion: IPF-associated mortality rates have increased globally, particularly in males. Significant geographical, age and sex disparities were observed, emphasising the need for targeted public health measures and improved disease management.