ERJ Open Research

BackgroundQuality of life (QoL), the highest prioritised outcome by children with bronchiectasis and their parents, is a patient-reported outcome measure increasingly considered essential when evaluating health and interventions. Despite this, there are no validated instruments that specifically measure QoL related to bronchiectasis in children. We aimed to develop and validate a new bronchiectasis child-specific parent-proxy QoL instrument (BC-QoL). MethodsWe developed a draft 44-item BC-QoL a...

Mucociliary clearance is a key component of the pathophysiology of bronchiectasis but cilia function is poorly defined. This study aims to characterize nasal ciliary function in bronchiectasis and examine associations with disease severity, infection, inflammation and outcome. Adults with bronchiectasis and healthy volunteers were recruited to the international observational study EMBARC-BRIDGE. Individuals with a known diagnosis of Primary Ciliary Dyskinesia (PCD) were excluded. Nasal respira...

RationaleSevere SARS-CoV-2 infection induces disrupted oropharyngeal and gut microbiota during acute disease which may persist and contribute to the development of post-acute pulmonary sequelae. To date, it is unclear whether dysbiosis following severe disease is linked to long-term pulmonary function impairment. ObjectivesTo determine associations between oropharyngeal and gut microbiota composition with lung function after severe COVID-19. Methods16S and internal transcribed spacer (ITS) rRN...

BackgroundThough a normal forced vital capacity (FVC) is typically thought to imply the absence of restriction, recent data suggest that restriction may in fact be common among patients with normal spirometry. However, the clinical significance of restriction with normal spirometry is unknown. Research QuestionWhat clinical characteristics and outcomes are associated with restriction with normal spirometry? Study Design and MethodsWe interpreted pulmonary function tests (PFTs) with both static...

The ERS/ATS22 interpretative flowchart classifies diffusing capacity (DLco) into 5 scenarios with associated pathophysiology, and has not been tested on large patient groups. We aimed to obtain a more layered DLco interpretation, by interrogating DLco components Kco and VA, and by estimating lung inflation during the DLco test to identify the presence of restriction, which crucially impacts Kco interpretation. By assessing a "low VA" against lung inflation, a novel 9-scenario DLco classification...

BackgroundBronchiectasis is a debilitating respiratory condition characterized by chronic cough with expectoration of thick sputum. It accounts for significant morbidity and mortality, especially when associated with exacerbations. Assessing the health-related quality of life (HR-QoL) of patients with bronchiectasis is important to ascertain the impact of the disease on day-to-day life, as well as to gauge the effect of targeted interventions. Conventionally used methods for assessing HR-QoL suc...

Background and AimsThe prognosis of interstitial lung diseases (ILDs) other than idiopathic pulmonary fibrosis (IPF) has not been studied as extensively as IPF. This study aimed to evaluate baseline factors associated with mortality in non-IPF ILD, including demographic characteristics, respiratory function test (RFT), comorbidities, and ILD subtypes. MethodsThis retrospective cohort study analysed prospectively collected data of patients with non-IPF ILD at a single tertiary centre in Malaysia...

Background: Interstitial lung abnormalities (ILA) are radiologic findings of increased lung density or fibrosis in individuals without clinical interstitial lung disease (ILD) and are associated with increased mortality and progression to ILD. Understanding physiologic trajectories of lung function preceding ILA diagnosis may illuminate early mechanisms of lung injury. Methods: We recruited participants from the Coronary Artery Risk Development in Young Adults (CARDIA) Lung Study, a prospective ...

ObjectiveMyositis-associated interstitial lung disease (myositis-ILD) consists of two predominant radiologic patterns of lung injury--nonspecific interstitial pneumonia (NSIP) and organizing pneumonia (OP)--that oftentimes coexist. However, it remains unclear whether either is associated with clinical outcomes. We aimed to assess the therapeutic response in patients with NSIP-compared to those with OP-predominant myositis-ILD. MethodsThis retrospective, single-center cohort study recruited part...

BackgroundInterstitial lung diseases (ILDs), including idiopathic pulmonary fibrosis (IPF) and connective tissue disease-associated ILD (CTD-ILD), share similar features that complicate diagnosis. Tumor markers are often elevated in ILD, yet their diagnostic utility remains unclear. MethodsThis retrospective study included ILD patients hospitalized between 2018 and 2025. Serum levels of alpha-fetoprotein, carcinoembryonic antigen (CEA), carbohydrate antigen (CA) 199, CA125, CA153, neuron-specif...

ObjectiveTo demonstrate the association between hospital admissions, health-related quality of life (HRQoL), limitations in daily activities, mortality, and survival in relation to physical activity (PA) and functional capacity (FC), which were used together to classify a cohort of COPD patients. MethodsProspective study. Participants were consecutively recruited from six outpatient respiratory clinics of a single university hospital. Data were recorded on sociodemographic and clinical variable...

Background/ObjectiveBarriers in access to care have prompted development of innovative care delivery models for patients with obstructive sleep apnea (OSA). One such innovation is management of uncomplicated OSA by primary care providers (PCPs), which reserves specialist capacity for more complex patients. We developed a clinical guideline and subsequently, an online clinical pathway to support PCPs in OSA management. We aimed to evaluate the impact of these initiatives on PCP behaviour by asses...

AbstractO_ST_ABSRationaleC_ST_ABSIdiopathic pulmonary fibrosis (IPF) is an age-related disorder with common and rare genetic risk factors. It is unknown if the effects of PF genetic risk factors differ by chronologic age. ObjectivesTo assess age-specific effects of genetic risk factors in PF patients and their relatives. MethodsWe identified common and rare genetic risk factors using a Columbia whole genome sequencing (WGS) cohort (777 IPF, 2905 controls) and replicated findings using Trans-Om...

BackgroundBronchiectasis is an increasingly recognized structural complication among patients with chronic obstructive pulmonary disease (COPD), yet evidence from Bangladesh remains limited. This study aimed to determine the prevalence, clinical characteristics, radiological patterns, and associated factors of bronchiectasis among COPD patients in tertiary-level hospitals. MethodsA cross-sectional study was conducted among 129 COPD patients regardless of age distribution, all are met GOLD crite...

ImportanceChronic obstructive pulmonary disease (COPD) remains a leading cause of morbidity and mortality in the United States (US), largely driven by cigarette smoking and characterized by progressive lung injury. While e-cigarettes are promoted as a less harmful alternative to cigarette smoking, their long-term health effects, including the impact of prolonged use on COPD incidence among adults who have smoked, are not well understood. ObjectiveTo evaluate the prospective association between ...

ObjectivesTo evaluate the efficacy and safety of sitafloxacin-containing regimens versus non-sitafloxacin therapy in patients with nontuberculous mycobacterial (NTM) pulmonary disease, focusing on sputum/BALF conversion rate, time of sputum/BALF culture conversion and radiographic improvement. MethodsThis retrospective cohort study analyzed 149 adults (76 control group vs. 73 sitafloxacin group) with NTM pulmonary disease treated between 2021 to 2024. Inclusion criteria: (1) Sitafloxacin group:...

Chronic obstructive pulmonary disease (COPD) and idiopathic pulmonary fibrosis (IPF) are phenotypically divergent disorders arising from similar exposures (including cigarette smoke). Differences in DNA methylation may drive the exposed lung towards COPD vs. IPF. To characterize differential methylation in COPD and IPF lung tissue relative to controls, we conducted epigenome-wide association studies of COPD and IPF in lung tissue from the Lung Tissue Research Consortium (N=1029), adjusting for a...

Chronic obstructive pulmonary disease (COPD) is a debilitating and progressive lung disease that affects millions of people worldwide. There is a continuing clinical need to characterize COPD at the molecular level to be able to identify the multi-omic biomarkers of its pathogenesis and to enable more accurate diagnoses and more effective treatment. We used Multi-Omics Factor Analysis (MOFA) to jointly analyze genomic, blood transcriptomic, and plasma proteomic data collected from 1,872 particip...

Pulmonary arterial hypertension (PAH) is a rare, life-limiting disease where deficiency of the TGF/BMP pathways have causal roles in hereditary and idiopathic forms. It is an attractive candidate for therapeutic intervention but there is an unmet need for clinically-relevant and practical biomarkers that can measure target engagement. A major challenge has been the inaccessibility of lung tissue in disease for molecular profiling. Here we explore the surrogate capacity of peripheral blood BMP pa...

Respiratory monitoring in daily-life settings is important for health assessment, yet extracting physiologically interpretable information from breathing signals under natural conditions remains challenging, as breathing is inherently dynamic and strongly modulated by behavior. Here, a portable breathing monitoring device based on a flexible lead zirconate titanate sensor is developed to address this challenge. By exploiting polarity-opposed piezoelectric and pyroelectric responses through senso...