BACKGROUND Pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH) cause right ventricular dysfunction with extrapulmonary sequalae including renal dysfunction. We sought to characterize renal dysfunction in PAH and CTEPH and to assess the effect of pulmonary hypertension treatment on renal function. METHODS We performed an individual participant data meta-analysis

RATIONALE Bronchiolitis obliterans syndrome (BOS) limits long-term survival after lung transplantation (LuTx) and may be triggered by infections. As immunoglobulin (Ig)A is crucial to ensure adequate mucosal immunity, we explored whether IgA-related mucosal immunity is impaired in BOS. METHODS Sixty LuTx recipients from the COLT cohort were retrospectively included. All participants were in stable

BACKGROUND The EMBARC registry shows considerable variation in culturable microbes in sputum between different European countries. The additive role of next generation metagenomic sequencing remains unexplored and association with antimicrobial resistomes unknown. METHODS We prospectively assessed sputum from N=349 individuals recruited into the EMBARC-BRIDGE study with next-generation shotgun metagenomic

The amount of walking that people with chronic obstructive pulmonary disease (COPD) do is reduced. However, data on their manner of walking (i.e., gait) is still lacking. We characterised real-world gait in COPD by assessing levels and distributions of gait parameters, and comparing them across COPD severity, and with healthy peers.549 people with COPD from seven European sites and 19 healthy older

RATIONALE : Prolonged exposure to airborne particulate matter (PM) is associated with emphysema and chronic obstructive pulmonary disease (COPD); however, the precise underlying mechanism remains unclear. OBJECTIVES In a previous high-throughput screen, we identified inter-alpha-trypsin inhibitor heavy chain 4 (ITIH4) as a biomarker of long-term PM exposure. We hypothesized that ITIH4 is implicated

Over the past three decades, several drugs have been developed to target three major dysfunctional pathways in PAH, including the prostacyclin, endothelin, and nitric oxide pathways. Despite these advances, PAH remains incurable, necessitating further drug discovery efforts. New therapies focus on previously untargeted pathways, especially the bone morphogenetic protein (BMP)/transforming growth factor-beta

BACKGROUND Obstructive sleep apnoea (OSA) carries an increased risk of motor vehicle accidents (MVA), which resulted in the European Commission introducing a legal directive (2014/85/EU) restricting driving in patients with moderate/severe OSA and sleepiness, unless effectively treated. We assessed the implementation of the directive in European Union (EU) member and non-member states. METHODS National

BACKGROUND Tuberculosis Preventive Treatment (TPT) can reduce TB incidence and mortality in people living with HIV. However, low levels of screening and uptake, poor adherence, and loss to follow-up considerably reduce its effectiveness. We aimed, therefore, to assess the losses within all steps of the screening and treatment cascade. METHODS To enhance data generalizibility we included articles which

BACKGROUND Patients with severe COVID-19 may develop lung fibrosis. Pirfenidone is an anti-fibrotic drug approved for idiopathic pulmonary fibrosis. The efficacy and safety of pirfenidone in patients with fibrotic interstitial lung changes after recovery from severe COVID-19 pneumonia were evaluated. METHODS This was a phase 2, double-blind, placebo-controlled, Spanish multicentre clinical trial. Patients

BACKGROUND Optimising Tuberculosis (TB) disease testing algorithms is fundamental to ensure the effectiveness and cost-effectiveness of migrant screening programmes, including better understanding of the individual and combined screening test properties. The aim of our study was to estimate pooled TB test properties from the literature and combining them in decision analytical modelling with a focus

BACKGROUND Pleural infection is a complex disease with poor clinical outcomes and increasing incidence worldwide, yet its biological endotypes remain unknown. METHODS We analysed 80 pleural fluid samples from the PILOT study, a prospective study on pleural infection, using unlabelled mass spectrometry. A total of 449 proteins were retained after filtering. Unsupervised hierarchical clustering and UMAP

BACKGROUND In people with cystic fibrosis (pwCF), human nasal epithelial cultures (HNECs) can be used to assess response to CFTR modulators. However, thresholds of in vitro responses that predict clinical benefit remain poorly understood. In this study we describe the concordance between in vitro response in HNECs and clinical outcomes in pwCF harbouring the F508del variant, treated with either Lumacaftor/Ivacaftor

AIMS Elexacaftor/tezacaftor/ivacaftor (ELX/TEZ/IVA) was efficacious and safe in children aged 6-11 years with cystic fibrosis (CF) heterozygous for F508del and a minimal function CFTR variant (F/MF genotypes) in a 24-week, placebo-controlled trial. We conducted a 96-week open-label extension study for children who completed the 24-week parent study. METHODS In this phase 3b extension study, dosing

BACKGROUND Current guidelines recommend a four-strata model based on World Health Organization functional class (WHO-FC), 6-min walk distance (6MWD), and serum levels of brain natriuretic peptide (BNP) or N-terminal pro-BNP (NT-proBNP) for risk stratification in patients with pulmonary arterial hypertension (PAH) during follow-up. We explored the relevance of using cardiopulmonary exercise testing

The complex pathogenic relationships between idiopathic pulmonary fibrosis (IPF) and its usually associated comorbidities remain poorly understood. While evidence suggests that some comorbidities may directly influence the development or progression of IPF, or vice versa, whether these associations are causal or arise independently due to shared risk factors, such as ageing, smoking, lifestyle and

BACKGROUND Interleukin-33 may have a role in COPD pathobiology. FRONTIER-4 (NCT04631016) investigated tozorakimab (an anti-IL-33 monoclonal antibody) in moderate-to-severe COPD patients with chronic bronchitis receiving dual or triple inhaled therapy. METHODS FRONTIER-4 was a phase 2a, randomized, double-blind, placebo-controlled study. Patients received tozorakimab 600 mg or placebo subcutaneously