Association between the HAL score and the development of progressive pulmonary fibrosis in idiopathic interstitial pneumonia: A prospective observational study.

Background: Progressive pulmonary fibrosis (PPF) is a critical concern in interstitial lung disease (ILD) management. The HAL score, which incorporates honeycombing (H), age >75 years (A), and serum lactate dehydrogenase >222 U/L (L), can predict acute exacerbations in patients with idiopathic interstitial pneumonia (IIP). This study aims to evaluate the predictive utility of the HAL score for PPF development.

Obstructive sleep apnea in patients with fibrotic interstitial lung disease (non-idiopathic pulmonary fibrosis): what should be offered?

Objective: The frequency of obstructive sleep apnea (OSA) in patients with idiopathic pulmonary fibrosis (IPF) is high. The clinical course of non-IPF interstitial lung disease (ILD) can be similar to that of IPF. We sought to assess the frequency and predictors of OSA in patients with non-IPF fibrotic ILD, as well as the impact of positive airway pressure (PAP) therapy on the quality of life of such patients.

Antifibrotic therapy in progressive pulmonary fibrosis: a review of recent advances.

Introduction: Progressive pulmonary fibrosis (PPF) is a manifestation of a heterogenous group of underlying interstitial lung disease (ILD) diagnoses, defined as non-idiopathic pulmonary fibrosis (IPF) progressive fibrotic ILD meeting at least two of the following criteria in the previous 12 months: worsening respiratory symptoms, absolute decline in forced vital capacity (FVC) more than or equal to 5% and/or absolute decline in diffusing capacity for carbon monoxide (DLCO) more than or equal to 10% and/or radiological progression.

Areas Covered: The authors subjectively reviewed a synthesis of literature from PubMed to identify recent advances in the diagnosis and characterisation of PPF, treatment recommendations, and management challenges. This review provides a comprehensive summary of recent advances and highlights future directions for the diagnosis, management, and treatment of PPF.

A study on the prevalence and prognosis of progressive pulmonary fibrosis: A retrospective observational study.

Interstitial lung disease (ILD) encompasses a heterogeneous group of more than 200 diffuse parenchymal lung diseases with various clinical courses. Disease progression is one of the most important prognostic factors, and, the definition of progressive pulmonary fibrosis (PPF) has recently been established. This study aimed to estimate the prevalence, risk factors, and prognosis of PPF among patients with non-idiopathic pulmonary fibrosis (IPF) in real-world practice.

Nintedanib in chronic fibrosing interstitial lung diseases. A case series.

Progressive pulmonary fibrosis (PPF) can be fatal in non-idiopathic interstitial lung diseases. We report a descriptive series of 13 patients with PPF who received treatment with nintedanib, a multitargeted tyrosine kinase inhibitor with antifibrotic effect. Although the reduced number of patients and the observational nature of a case series prevent us from providing strong evidence, our results suggest that nintedanib could be effective in PPF of various etiologies.

Risk factors for progression of pulmonary fibrosis: a single-centered, retrospective study.

Objective: This study aimed to identify clinical characteristics associated with the prevalence of progressive pulmonary fibrosis (PPF) in interstitial lung disease (ILD) and to develop a prognostic nomogram model for clinical use.

Methods: In this single-centered, retrospective study, we enrolled ILD patients with relatively comprehensive clinical data and assessed the incidence of PPF within a year using collected demographics, laboratory data, high-resolution computed tomography (HRCT), and pulmonary function test (PFT) results. We used a training cohort of ILD patients to identify early predictors of PPF and then validated them in an internal validation cohort and subsets of ILD patients using a multivariable logistic regression analysis.

Use of 6-minute walk distance to predict lung transplant-free survival in fibrosing non-IPF interstitial lung diseases.

Background And Objective: The identification of progression in patients with fibrosing non-idiopathic pulmonary fibrosis (IPF) interstitial lung diseases (ILDs) represents an ongoing clinical challenge. Lung function decline alone may have significant limitations in the detection of clinically significant progression. We hypothesized that longitudinal changes of 6-min walk distance (6MWD) from baseline, simultaneously considered with measures of lung function, may independently predict survival and identifying clinically significant progression of disease.

Systemic corticosteroids in fibrotic lung disease: a systematic review and meta-analysis.

Objectives: We aimed to assess the available evidence for corticosteroids in fibrotic interstitial lung disease (fILD) to inform the randomised embedded multifactorial adaptive platform ILD.

Design: Systematic review and meta-analysis.

Data Sources: We searched Embase, Medline, Cochrane CENTRAL and Web of Science databases from inception to April 17 2023.

Survival and acute exacerbation for patients with idiopathic pulmonary fibrosis (IPF) or non-IPF idiopathic interstitial pneumonias: 5-year follow-up analysis of a prospective multi-institutional patient registry.

Objective: Few prospective cohort studies with relatively large numbers of patients with non-idiopathic pulmonary fibrosis (non-IPF) of idiopathic interstitial pneumonia (IIP) have been described. We aimed to assess disease progression and cause of death for patients with non-IPF IIPs or IPF under real-life conditions.

Methods: Data were analysed for a prospective multi-institutional cohort of 528 IIP patients enrolled in Japan between September 2013 and April 2016.

Lung Magnetic Resonance Imaging for Prediction of Progression in Patients With Nonidiopathic Pulmonary Fibrosis Interstitial Lung Disease: A Pilot Study.

Purpose: Correlate magnetic resonance imaging (MRI) parameters at baseline with disease progression in nonidiopathic pulmonary fibrosis interstitial lung disease (ILD).

Materials And Methods: Prospective observational cohort study, in which patients with non-idiopathic pulmonary fibrosis ILD underwent MRI at baseline (1.5 T).

Telomere length and immunosuppression in non-idiopathic pulmonary fibrosis interstitial lung disease.

Background: Studies suggest a harmful pharmacogenomic interaction exists between short leukocyte telomere length (LTL) and immunosuppressants in idiopathic pulmonary fibrosis (IPF). It remains unknown if a similar interaction exists in non-IPF interstitial lung disease (ILD).

Methods: A retrospective, multicentre cohort analysis was performed in fibrotic hypersensitivity pneumonitis (fHP), unclassifiable ILD (uILD) and connective tissue disease (CTD)-ILD patients from five centres.

Nintedanib for non-IPF progressive pulmonary fibrosis: 12-month outcome data from a real-world multicentre observational study.

Background: Nintedanib slows lung function decline for patients with non-idiopathic pulmonary fibrosis progressive pulmonary fibrosis (PPF) in clinical trials, but the real-world safety and efficacy are not known.

Methods: In this retrospective cohort study, standardised data were collected from patients in whom nintedanib was initiated for PPF between 2019 and 2020 through an early-access programme across eight centres in the United Kingdom. Rate of lung function change in the 12months pre- and post-nintedanib initiation was the primary analysis.

The current status and future of FDA-approved artificial intelligence tools in chest radiology in the United States.

Artificial intelligence (AI) is becoming more widespread within radiology. Capabilities that AI algorithms currently provide include detection, segmentation, classification, and quantification of pathological findings. Artificial intelligence software have created challenges for the traditional United States Food and Drug Administration (FDA) approval process for medical devices given their abilities to evolve over time with incremental data input.

Validation of Proposed Criteria for Progressive Pulmonary Fibrosis.

Criteria for progressive pulmonary fibrosis (PPF) have been proposed, but their prognostic value beyond categorical decline in FVC remains unclear. To determine whether proposed PPF criteria predict transplant-free survival (TFS) in patients with non-idiopathic pulmonary fibrosis (IPF) forms of interstitial lung disease (ILD). A retrospective, multicenter cohort analysis was performed.

A multi-criteria decision analysis on the value of nintedanib for interstitial lung diseases.

Objectives: Our aim was to assess the value of nintedanib for non-idiopathic progressive fibrosing interstitial lung disease (non-IPF PF-ILD) and systemic sclerosis-associated ILD (SSc-ILD) in the Spanish context, using a multi-criteria decision analysis (MCDA).

Methods: Following an adaptation of the Evidence and Value: Impact on DEcision Making (EVIDEM) MCDA methodology, the estimated value of nintedanib was obtained by means of an additive linear model that combined individual weights (100-points distribution) of criteria with the individual scoring of nintedanib in each criterion for every indication, assigned by a multidisciplinary committee of twelve clinicians, patients, pharmacists, and decision-makers. To assess the reproducibility, an alternative weighting method was applied, as well as a re-test of weights and scores at a different moment of time.

Novel insights in fibrotic pulmonary sarcoidosis.

Purpose Of Review: In chronic pulmonary sarcoidosis, the transition from the inflammatory to the fibrotic stage of the lungs occurs in about 10-20% of cases, eventually causing end-stage fibrotic disease. To date, pathogenetic mechanisms and clinical management remain challenging; thus, we highlight the recent evidence in pulmonary fibrotic processes, clinical signs for an early detection and the potential role of the current investigated antifibrotic agents and promising targeted therapies.

Recent Findings: Recent findings of relevant key cellular pathways can be considered as a glimmer of light in the complexity of sarcoidosis.

Disease pathology in fibrotic interstitial lung disease: is it all about usual interstitial pneumonia?

The interstitial pneumonias comprise a diverse group of diseases that are typically defined by their cause (either idiopathic or non-idiopathic) and their distinct histopathological features, for which radiology, in the form of high-resolution CT, is often used as a surrogate. One trend, fuelled by the failure of conventional therapies in a subset of patients and the broad-spectrum use of antifibrotic therapies, has been the focus on the progressive fibrosing phenotype of interstitial lung disease. The histological pattern, known as usual interstitial pneumonia, is the archetype of progressive fibrosis.