Development of a Prediction Model for Acute Exacerbation in Idiopathic Pulmonary Fibrosis: A Study of the Korea IPF Cohort Registry.

Background: Acute exacerbation (AE) of idiopathic pulmonary fibrosis (IPF) has the most disastrous impact on prognosis as a major cause of morbidity and mortality. However, there is no proven treatment, and the occurrence of AE is unpredictable. This study aimed to develop a prediction model for AE in patients with IPF using the nationwide Korea IPF Cohort (KICO) registry.

Phase 3 Trial of Inhaled Molgramostim in Autoimmune Pulmonary Alveolar Proteinosis.

Background: Autoimmune pulmonary alveolar proteinosis (aPAP) is a rare disease characterized by progressive surfactant accumulation and hypoxemia caused by autoantibodies against granulocyte-macrophage colony-stimulating factor (GM-CSF), which alveolar macrophages require to clear surfactant. Molgramostim is a formulation of inhaled recombinant human GM-CSF, but its efficacy and safety in patients with aPAP have not been studied sufficiently.

Methods: In this phase 3, double-blind, placebo-controlled trial, we randomly assigned patients with aPAP to receive molgramostim at a dose of 300 μg or placebo once daily for 48 weeks.

Development and Validation of a Clinical Scoring System Predicting 30-Day Mortality in Acute Exacerbation of Idiopathic Pulmonary Fibrosis.

Background And Objectives: Acute exacerbation (AE) of idiopathic pulmonary fibrosis (IPF) is a frequent and fatal complication of IPF, with highly variable individual outcomes that are challenging to predict.

Methods: This study included patients (≥ 19 years) who met the diagnostic criteria for AE-IPF. The primary outcome was 30-day mortality following hospital admission.

Pharmacoeconomic inequalities in access to antifibrotic treatment for interstitial lung disease in the Asia-Pacific region.

Antifibrotic drugs, available for the best part of the last decade in many parts of the world, has improved outcomes in patients with idiopathic pulmonary fibrosis and progressive pulmonary fibrosis. However, it is unclear whether patients suffering from these devastating conditions have timely and adequate access to antifibrotic therapy in the Asia Pacific region (APAC). In this mixed-methods narrative review of 12 APAC countries, integration of questionnaire-based insights of 31 regional clinical experts in interstitial lung disease (ILD) with publicly available pharmaco-economic information has been used to understand how country-specific challenges impact on antifibrotic accessibility.

Phase 2 study design and analysis approach for BBT-877: an autotaxin inhibitor targeting idiopathic pulmonary fibrosis.

Introduction: Proof-of-concept (POC) studies are vital in determining the feasibility of further drug development, primarily by assessing preliminary efficacy signals with credible endpoints. However, traditional POC studies in idiopathic pulmonary fibrosis (IPF) can suffer from low credibility due to small sample sizes and short durations, leading to non-replicable results in larger phase III trials. To address this, we are conducting a 24-week POC study with 120 patients with IPF, using a statistically supported sample size and incorporating exploratory CT-based imaging biomarkers, to support decision-making in the case of non-significant primary endpoint results.

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.

Polymyxin B-hemoperfusion in patients with acute exacerbation of idiopathic pulmonary fibrosis: a single-center prospective pilot study.

Background/aims: Patients with acute exacerbation of idiopathic pulmonary fibrosis (AE-IPF) typically have a poor prognosis; however, no effective treatment is available. In recent years, several retrospective studies have suggested the clinical benefits of direct hemoperfusion with a polymyxin B-immobilized fiber column (PMX-DHP) in patients with AE-IPF. Herein, we aimed to investigate the efficacy and safety of PMX-DHP treatment in patients with AE-IPF.

Advances in Concept and Imaging of Interstitial Lung Disease.

Although idiopathic pulmonary fibrosis (IPF) is a type of idiopathic interstitial pneumonia (IIP), it is different from other IIPs. IPF also differs from interstitial lung disease (ILD) with known causes, including connective tissue disease, exposure, cysts and/or airspace filling disease, and sarcoidosis. More than 90% of IPFs demonstrate progressive disease.

Evaluating the robustness of deep learning models trained to diagnose idiopathic pulmonary fibrosis using a retrospective study.

Background: Deep learning (DL)-based systems have not yet been broadly implemented in clinical practice, in part due to unknown robustness across multiple imaging protocols.

Purpose: To this end, we aim to evaluate the performance of several previously developed DL-based models, which were trained to distinguish idiopathic pulmonary fibrosis (IPF) from non-IPF among interstitial lung disease (ILD) patients, under standardized reference CT imaging protocols. In this study, we utilized CT scans from non-IPF ILD subjects, acquired using various imaging protocols, to assess the model performance.

Current perspectives on interstitial lung abnormalities.

Interstitial lung abnormalities (ILAs) are early indicators of interstitial lung disease, often identified incidentally via computed tomography of the chest. This review explores the diagnostic criteria for ILAs as outlined by the Fleischner Society, highlights associated risk factors, examines their impact on patient outcomes, and discusses management strategies. The prevalence of ILAs varies significantly, ranging from 3% to 17% across populations.

Continued Treatment with Nintedanib in Patients with Progressive Pulmonary Fibrosis: Data from INBUILD-ON.

Purpose: In the INBUILD trial in patients with progressive pulmonary fibrosis (PPF), nintedanib slowed the decline in forced vital capacity (FVC) versus placebo, with a safety profile characterised mainly by gastrointestinal events. INBUILD-ON, the open-label extension of INBUILD, assessed the safety of nintedanib during longer-term treatment. Data on FVC were collected.

Understanding of Patients with Severe COVID-19 Using Lung Ultrasound.

Background: Lung ultrasound (LUS) has proven valuable in the initial assessment of coronavirus disease 2019 (COVID-19), but its role in detecting pulmonary fibrosis following intensive care remains unclear. This study aims to assess the presence of pulmonary sequelae and fibrosis-like changes using LUS in survivors of severe COVID-19 pneumonia one month after discharge.

Methods: We prospectively enrolled patients with severe COVID-19 who required mechanical ventilation in the intensive care unit (ICU) and conducted LUS assessments from admission to the outpatient visit after discharge.

Pirfenidone and risk of lung cancer development in idiopathic pulmonary fibrosis: a nationwide population-based study.

Background: Idiopathic pulmonary fibrosis (IPF) carries a high risk of lung cancer, but the effect of pirfenidone on lung cancer development remains uncertain. We investigated the association between pirfenidone use and lung cancer development in patients with IPF.

Methods: We included 9938 patients with IPF from the Korean national claims database.

Polyhexamethylene guanidine aerosol causes irreversible changes in blood proteins that associated with the severity of lung injury.

Polyhexamethylene guanidine (PHMG) is a positively charged polymer used as a disinfectant that kills microbes but can cause pulmonary fibrosis if inhaled. After the long-term risks were confirmed in South Korea, it became crucial to measure toxicity through diverse surrogate biomarkers, not only proteins, especially after these hazardous chemicals had cleared from the body. These biomarkers, identified by their biological functions rather than simple numerical calculations, effectively explained the imbalance of pulmonary surfactant caused by fibrosis from PHMG exposure.

Circulating biomarkers and progression of idiopathic pulmonary fibrosis: data from the INMARK trial.

Background: We used data from the INMARK trial to investigate associations between circulating biomarkers of extracellular matrix (ECM) turnover, inflammation and epithelial dysfunction and disease progression in subjects with idiopathic pulmonary fibrosis (IPF).

Methods: Subjects with IPF and forced vital capacity (FVC) ≥80% predicted were randomised 1:2 to receive nintedanib 150 mg twice daily or placebo for 12 weeks followed by open-label nintedanib for 40 weeks. Associations between baseline biomarker levels and the proportion of subjects with disease progression (decline in FVC ≥10% predicted or death) over 52 weeks were assessed in subjects randomised to placebo using logistic regression.

Blood lipid profiles as a prognostic biomarker in idiopathic pulmonary fibrosis.

Background: Dysregulation of lipid metabolism is implicated in the pathogenesis of idiopathic pulmonary fibrosis (IPF). However, the association between the blood lipid profiles and the prognosis of IPF is not well defined. We aimed to identify the impacts of lipid profiles on prognosis in patients with IPF.

ECMO is associated with decreased hospital mortality in COVID-19 ARDS.

This study determined whether compared to conventional mechanical ventilation (MV), extracorporeal membrane oxygenation (ECMO) is associated with decreased hospital mortality or fibrotic changes in patients with COVID-19 acute respiratory distress syndrome. A cohort of 72 patients treated with ECMO and 390 with conventional MV were analyzed (February 2020-December 2021). A target trial was emulated comparing the treatment strategies of initiating ECMO vs no ECMO within 7 days of MV in patients with a PaO/FiO < 80 or a PaCO ≥ 60 mmHg.