Mortality Outcomes and ACE Inhibitor Use in Patients with Idiopathic Pulmonary Fibrosis.

Background: ACE inhibitors (ACEi) are widely used antihypertensive agents with proven cardioprotective effects. Previous mechanistic and clinical studies have suggested ACEi therapy may slow disease progression and reduce mortality in idiopathic pulmonary fibrosis (IPF).

Research Question: Does ACEi use associate with reduced all-cause in a real-world population of IPF patients, and if so, is the association also present in COPD patients?

Study Design And Methods: A retrospective analysis was conducted using electronic health records from the Clinical Practice Research Datalink (CPRD) GOLD database (2019), linked with HES Admitted Patient Care and ONS death registration data.

Distinct Morphological Types of Small Airway Obstructions in Smokers with Emphysema and End-Stage COPD.

Rationale: The precise nature of small airway obstructions in COPD remains poorly understood, especially at early disease stages.

Objectives: This study aimed to characterize small airway obstructions and numbers up to the terminal bronchioles (TB) in smokers with limited emphysema and end-stage COPD. We hypothesized that obstruction subtypes would differ in morphology, nature and number from early to end-stage COPD.

A deep generative model for deciphering cellular dynamics and in silico drug discovery in complex diseases.

Human diseases are characterized by intricate cellular dynamics. Single-cell transcriptomics provides critical insights, yet a persistent gap remains in computational tools for detailed disease progression analysis and targeted in silico drug interventions. Here we introduce UNAGI, a deep generative neural network tailored to analyse time-series single-cell transcriptomic data.

Computational fluid dynamics of small airway disease in chronic obstructive pulmonary disease.

Background: Small airways (<2 mm diameter) are major sites of airflow obstruction in chronic obstructive pulmonary disease (COPD). This study aimed to quantify the impact of small airway disease, characterized by narrowing, occlusion, and obliteration, on airflow parameters in smokers and end-stage patients with COPDs.

Methods: We performed computational fluid dynamics (CFD) simulations of inspiratory airflow in three lung groups: control non-used donor lungs (no smoking/emphysema history), non-used donor lungs with a smoking history and emphysema, and explanted end-stage COPD lungs.

Lung ultrasound outperforms symptom-based screening to detect interstitial lung disease associated with rheumatoid arthritis.

Objectives: Interstitial lung disease associated with rheumatoid arthritis (RA-ILD) is linked to high mortality. Currently, effective screening tools are lacking. We assessed the role of symptoms and lung ultrasound (LUS) as potential screening tools.

Clinical predictors for restrictive allograft syndrome: A nested case-control study.

Risk factors for restrictive allograft syndrome (RAS), a severe phenotype of chronic lung allograft dysfunction (CLAD) after lung transplantation, are currently not well known. In this retrospective nested case-control-study, we analyzed 69 patients with RAS and 69 matched non-CLAD controls to identify clinical risk factors for RAS. Patients with RAS demonstrated overall higher blood eosinophils (P = .

ERS International Congress 2023: highlights from the Interstitial Lung Diseases Assembly.

This article summarises a selection of scientific highlights in the field of interstitial lung diseases (ILDs) presented at the International Congress of the European Respiratory Society in 2023. Translational and clinical studies focused on the whole spectrum of ILDs, from (ultra)rare ILDs to sarcoidosis, ILDs associated with connective tissue disease and idiopathic pulmonary fibrosis. The main topics of the 2023 Congress presentations were improving the diagnostic process of ILDs, better prediction of disease course and investigation of novel treatment options.

Unagi: Deep Generative Model for Deciphering Cellular Dynamics and In-Silico Drug Discovery in Complex Diseases.

Human diseases are characterized by intricate cellular dynamics. Single-cell sequencing provides critical insights, yet a persistent gap remains in computational tools for detailed disease progression analysis and targeted in-silico drug interventions. Here, we introduce UNAGI, a deep generative neural network tailored to analyze time-series single-cell transcriptomic data.

Mucosal immune alterations at the early onset of tissue destruction in chronic obstructive pulmonary disease.

Rationale: COPD is characterized by chronic airway inflammation, small airways changes, with disappearance and obstruction, and also distal/alveolar destruction (emphysema). The chronology by which these three features evolve with altered mucosal immunity remains elusive. This study assessed the mucosal immune defense in human control and end-stage COPD lungs, by detailed microCT and RNA transcriptomic analysis of diversely affected zones.

Ultrasonographic Presentation and Anatomic Distribution of Lung Involvement in Patients with Rheumatoid Arthritis.

Background: Rheumatoid arthritis (RA) is a chronic auto-immune disease, typically affecting the joints, which can also present with lung involvement (pleuritis, interstitial lung disease, pulmonary nodules, etc.). Lung ultrasound (LUS) is an upcoming tool in the detection of these pulmonary manifestations.

COVID-19 progression in hospitalized patients using follow-up CT and microCT.

Background: Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) causes coronavirus disease-19 (COVID-19) which can lead to acute respiratory distress syndrome (ARDS) and evolve to pulmonary fibrosis. Computed tomography (CT) is used to study disease progression and describe radiological patterns in COVID-19 patients. This study aimed to assess disease progression regarding lung volume and density over time on follow-up chest CT and give a unique look at parenchymal and morphological airway changes in "end-stage" COVID-19 lungs using microCT.

Delineating associations of progressive pleuroparenchymal fibroelastosis in patients with pulmonary fibrosis.

Background: Computer quantification of baseline computed tomography (CT) radiological pleuroparenchymal fibroelastosis (PPFE) associates with mortality in idiopathic pulmonary fibrosis (IPF). We examined mortality associations of longitudinal change in computer-quantified PPFE-like lesions in IPF and fibrotic hypersensitivity pneumonitis (FHP).

Methods: Two CT scans 6-36 months apart were retrospectively examined in one IPF (n=414) and one FHP population (n=98).

Defining and predicting progression in non-IPF interstitial lung disease.

Randomized placebo-controlled trials demonstrated the efficacy of antifibrotic treatment in non-IPF progressive fibrosing ILD (fILD). Currently, there is no consensus on how progression should be defined and clinical data of non-IPF fILD patients in a real-world setting are scarce. Non-IPF fILD patients presenting at the University Hospitals Leuven between 2012 and 2016 were included.

Progression in the Management of Non-Idiopathic Pulmonary Fibrosis Interstitial Lung Diseases, Where Are We Now and Where We Would Like to Be.

A significant proportion of patients with interstitial lung disease (ILD) may develop a progressive fibrosing phenotype characterized by worsening of symptoms and pulmonary function, progressive fibrosis on chest computed tomography and increased mortality. The clinical course in these patients mimics the relentless progressiveness of idiopathic pulmonary fibrosis (IPF). Common pathophysiological mechanisms such as a shared genetic susceptibility and a common downstream pathway-self-sustaining fibroproliferation-support the concept of a progressive fibrosing phenotype, which is applicable to a broad range of non-IPF ILDs.