Geographical Distribution of Phase II/III Pharmaceutical Randomised Controlled Trials in Adults with Interstitial Lung Disease.

Background: Pharmaceutical randomised controlled trials (RCTs) are critical for advancing treatments in interstitial lung disease (ILD). We evaluated global distribution of pharmaceutical RCTs of ILD and their associations with country-specific features.

Research Question: Do ILD RCT activities relate to disease prevalence, healthcare quality, sociodemographic status, and urban population size?

Study Design And Methods: ClinicalTrials.

Standardized Clinical Terms and Definitions for Interstitial Lung Disease: A Consensus Statement from the Fleischner Society.

Background: Despite advances in diagnosis and management, the interstitial lung disease (ILD) lexicon is plagued by ambiguous and inconsistent terminology that complicates communication and impedes knowledge generation. The objective of this Fleischner Society Consensus Statement was to produce standardized terminology for ILD multidisciplinary diagnoses and major phenotypes.

Methods: Interviews with 10 experts were used to identify ILD clinical diagnoses and major phenotypes.

Global epidemiology and burden of interstitial lung disease.

The global incidence and prevalence of interstitial lung disease (ILD) are difficult to determine due to the rarity of the condition, inconsistent case ascertainment and reporting methods, and differences in risk factors, burden of exposures, and access to health-care systems across geographical areas. With these caveats, several studies have reported an increased incidence and prevalence of ILD over time. Idiopathic pulmonary fibrosis is the most common and extensively studied ILD, whereas connective tissue disease-associated ILD and hypersensitivity pneumonitis display the most geographical variability.

Treatment Toxicity and Outcomes Following Definitive Radiation Therapy for Patients With Early-Stage Non-Small Cell Lung Cancers and Pre-Existing Interstitial Lung Disease-A Systematic Review and Dosimetric Analysis.

Background: Patients with interstitial lung disease (ILD) who develop lung cancer represent a unique challenge, as they are at higher risk for serious toxicity from local and systemic therapies. The aim of this study is to provide an up-to-date analysis on toxicities and outcomes associated with definitive radiation therapy (RT) in patients with ILD and early-stage non-small cell lung cancer (ES-NSCLC).

Methods And Materials: We performed a systematic review in accordance with Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines.

Association of vaccination status and immunosuppression with mortality of SARS-CoV-2 infection in patients with fibrotic interstitial lung disease.

Background: There are limited data exploring the outcome following SARS-CoV-2 infection in fibrotic interstitial lung disease (fILD). Our goal was to determine the association of vaccination for SARS-CoV-2 with both SARS-CoV-2 infection and subsequent 90-day mortality in fILD. Our second objective was to determine the association of immunosuppressive use with both SARS-CoV-2 infection and subsequent 90-day mortality in fILD.

Epigenome-Wide Analysis Identifies Pollution-Sensitive Loci in Fibrotic Interstitial Lung Disease.

Rationale: Particulate matter <=2.5um (PM) adversely impacts patients with fibrotic interstitial lung disease (fILD).

Objective: To determine whether PM-associated epigenetic alterations contribute to the environmental pathogenesis of fILD.

Particulate Matter Associations with Lung Function in Pulmonary Sarcoidosis.

Sarcoidosis is a multi-system disease with pulmonary manifestations in over 90% of patients. Environmental exposures are associated with sarcoidosis incidence, but the impact on clinical outcomes remains understudied. To evaluate the association of ambient particulate matter ≤2.

Performance Characteristics for Physiological Measures of Progressive Pulmonary Fibrosis.

Rationale: Clinical measures of progressive pulmonary fibrosis (PPF) have been proposed, but their clinical utility remains unclear.

Objectives: To determine performance characteristics of lung function-based PPF measures, including new guideline criteria for discriminating clinically relevant outcomes.

Methods: A multicenter retrospective cohort analysis was performed to assess the performance characteristics of eight categorical measures of forced vital capacity (FVC) and diffusion capacity (DLCO) decline, along with PPF guideline criteria (requiring two of the following: worsening respiratory symptoms, absolute decline in FVC ≥5% or DLCO ≥15%, or radiological progression) for discriminating two-year death or lung transplant among fibrotic ILD patients from the United States, United Kingdom and Canada (n=2727).

Outcomes of a Typical Fibrotic Hypersensitivity Pneumonitis Pattern on Chest Computed Tomography.

Background: Guidelines have defined a "typical hypersensitivity pneumonitis (HP)" imaging pattern for fibrotic HP (fHP); however, the frequency, characteristics, and outcomes of different multidisciplinary diagnoses within this pattern are unknown.

Methods: Patients with a typical fHP pattern on chest computed tomography (CT) were identified from a prospective registry. Multidisciplinary diagnoses were established by consensus during a research-dedicated standardized multidisciplinary discussion of all available data.

Approach to the Evaluation and Management of Interstitial Lung Abnormalities: An Official American Thoracic Society Clinical Statement.

There is growing interest in identifying early stages of interstitial lung disease (ILD) to improve patient outcomes. This document reviews updated evidence on interstitial lung abnormalities (ILAs); provides suggestions for screening, evaluation, and management; proposes criteria for distinguishing ILAs from ILD; and identifies research priorities. A committee of clinical and methodology experts met by video conference to define ILAs and ILD by consensus and voted on 11 prespecified questions after reviewing synthesized evidence from a systematic literature search.

The Use of CT Densitometry for the Assessment of Emphysema in Clinical Trials: A Position Paper from the Fleischner Society.

Emphysema's significant morbidity and mortality underscore the need for reliable outcome metrics in clinical trials. However, commonly accepted COPD outcome measures do not adequately capture emphysema severity or progression. Computed tomography (CT) metrics have been validated as accurate indicators of pathological emphysema and predictors of COPD progression, exacerbations, and mortality.

Effects of long-term oxygen therapy on acute exacerbation and hospital burden: the national DISCOVERY study.

Background: Long-term oxygen therapy (LTOT) improves survival in patients with chronic severe resting hypoxaemia, but effects on hospitalisation are unknown. This study evaluated the potential impact of starting LTOT on acute exacerbation and hospital burden in patients with chronic obstructive pulmonary disease (COPD), interstitial lung disease (ILD) and pulmonary hypertension (PH).

Methods: Longitudinal analysis of consecutive patients in the population-based Swedish DISCOVERY cohort who started LTOT between 2000 and 2018 with a follow-up duration≥3 months.

Comorbidities in the idiopathic pulmonary fibrosis and progressive pulmonary fibrosis trial population: a systematic review and meta-analysis.

Background: Comorbidities can affect drug tolerability and health outcomes in patients with fibrotic interstitial lung disease. This systematic review and meta-analysis evaluated the types and prevalence of comorbidities amongst participants in pharmaceutical randomised controlled trials (RCTs) of idiopathic pulmonary fibrosis (IPF) and progressive pulmonary fibrosis (PPF).

Methods: Ovid Medline, Embase and CENTRAL databases were searched to identify phase II and III pharmaceutical RCTs of IPF or PPF.

CT Honeycombing and Traction Bronchiectasis Extent Independently Predict Survival across Fibrotic Interstitial Lung Disease Subtypes.

Background Prognostic value of radiologic features in interstitial lung disease (ILD) has been predominantly studied in idiopathic pulmonary fibrosis, but findings vary. The relative importance of features versus guideline-defined patterns in predicting outcomes is unknown. Purpose To identify radiologic features that are independently associated with transplant-free survival beyond clinical predictive factors across all ILD subtypes, and to identify whether individual features versus patterns are more important for prognostication.

Diagnosis, screening, and follow-up of patients with familial interstitial lung disease: Results from an international survey.

Background: Advances in the field of genetics of interstitial lung diseases (ILDs) have led to the recent consensus statements made by expert groups. International standards for genetic testing in ILD have not yet been established. We aimed to examine current real-world strategies employed by pulmonologists working with familial ILD.

Accelerated epigenetic ageing worsens survival and mediates environmental stressors in fibrotic interstitial lung disease.

Background: The role of epigenetic ageing in the environmental pathogenesis and prognosis of fibrotic interstitial lung disease (fILD) is unclear. We evaluated whether ambient particulate matter with diameter ≤2.5 μm (PM) and neighbourhood disadvantage exposures are associated with accelerated epigenetic ageing, and whether epigenetic age is associated with adverse clinical outcomes in patients with fILD.

Assessment of online YouTube videos as a source of information and instruction for pulmonary rehabilitation.

Background/objectives: Pulmonary rehabilitation (PR) benefits individuals with chronic respiratory conditions beyond COPD; however, the quality of online resources has not been evaluated. The aims of this study were to assess the content, quality, and comprehensibility of YouTube videos that provide PR to individuals with chronic lung diseases other than COPD.

Methods: A search was conducted on YouTube for videos related to PR on non-COPD conditions, with the first 350 videos screened for eligibility (2004-2024).