In this editorial, we comment on the article by Lei with a specific focus on the timing of the initiation of the antifibrotic agent pirfenidone (PFD) in the management of idiopathic pulmonary fibrosis (IPF) and its impact on lung function of IPF patients. PFD is an antifibrotic agent that is widely used in the management of IPF in both early and advanced stages. It inhibits various pathways and has antifibrotic, anti-inflammatory, and antioxidant properties. Despite dosage lowering, PFD slowed IPF progression and maintained functional capacity. The 6-min walk distance test indicated that patients tolerated adverse events well, and PFD significantly reduced the incidence of progression episodes and death. Even when a single disease-progression event occurred, continuing PFD treatment had benefits.
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http://dx.doi.org/10.12998/wjcc.v12.i32.6538 | DOI Listing |
Proteomes
January 2025
Research & Development, AbbVie Bioresearch Center, Worcester, MA 01605, USA.
Idiopathic pulmonary fibrosis (IPF) is a progressive lung disease characterized by repetitive alveolar injuries with excessive deposition of extracellular matrix (ECM) proteins. A crucial need in understanding IPF pathogenesis is identifying cell types associated with histopathological regions, particularly local fibrosis centers known as fibroblast foci. To address this, we integrated published spatial transcriptomics and single-cell RNA sequencing (scRNA-seq) transcriptomics and adopted the Query method and the Overlap method to determine cell type enrichments in histopathological regions.
View Article and Find Full Text PDFFront Immunol
January 2025
Department of Medical Immunology, Medical University of Gdansk, Gdansk, Poland.
Idiopathic pulmonary fibrosis (IPF) is a progressive and fatal disease, characterized by impaired wound repair, tissue remodeling and fibrosis. Immune system may participate in the development and progression of the disease as indicated by altered activity in IPF sufferers. This study investigates the immune response to the BNT162b2 COVID-19 vaccine in patients with IPF compared to healthy controls, with a particular focus on evaluation of antibody responses, interferon-gamma release, cytokine profiling and a broad panel of immune cell subpopulations.
View Article and Find Full Text PDFRight ventricular heart failure (RV HF) is the leading cause of death in pulmonary arterial hypertension (PAH). Relevance of the low-risk status assessment using available diagnostic tools requires a reliable confirmation. The study aimed to evaluate right ventricular perfusion and glucose metabolism using positron emission tomography (PET)/computed tomography (CT) with [13N]-ammonia and [18F]-fluorodeoxyglucose ([18F]-FDG) in 30 IPAH patients (33.
View Article and Find Full Text PDFRespir Res
January 2025
Division of Allergy, Pulmonary and Critical Care Medicine, Department of Medicine, School of Medicine and Public Health, University of Wisconsin-Madison, 600 Highland Avenue, Madison, WI, 53792, USA.
Progressive forms of interstitial lung diseases, including idiopathic pulmonary fibrosis (IPF), are deadly disorders lacking non-invasive biomarkers for assessment of early disease activity, which presents a major obstacle in disease management. Excessive extracellular matrix (ECM) deposition is a hallmark of these disorders, with fibronectin being an abundant ECM glycoprotein that is highly upregulated in early fibrosis and serves as a scaffold for the deposition of other matrix proteins. Due to its role in active fibrosis, we are targeting fibronectin as a biomarker of early lung fibrosis disease activity via the PEGylated fibronectin-binding polypeptide (PEG-FUD).
View Article and Find Full Text PDFEur Respir Rev
January 2025
Dept of Public Health and Primary Care, Academic Centre for Nursing and Midwifery, KU Leuven - University of Leuven, Leuven, Belgium
Introduction: People with idiopathic pulmonary fibrosis (IPF) and other forms of progressive pulmonary fibrosis (PPF) have a high symptom burden and a poor health-related quality of life (HRQoL). Despite efforts to offer specialised treatment, clinical care for these patients remains suboptimal and several nonmedical needs remain unaddressed. Developing a core outcome set (COS) can help to identify a minimum set of agreed-upon outcomes that should be measured and acted-upon in clinical care.
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