Category Ranking
98%
Total Visits
921
Avg Visit Duration
2 minutes
Citations
20
Article Abstract
Interstitial lung diseases comprise a heterogenous range of diffuse lung disorders, potentially resulting in pulmonary fibrosis. While idiopathic pulmonary fibrosis has been recognized as the paradigm of a progressive fibrosing interstitial lung disease, other conditions with a progressive fibrosing phenotype characterized by a significant deterioration of the lung function may lead to a burden of significant symptoms, a reduced quality of life, and increased mortality, despite treatment. There is now evidence indicating that some common underlying biological mechanisms can be shared among different chronic fibrosing disorders; therefore, different biomarkers for disease-activity monitoring and prognostic assessment are under evaluation. Thus, understanding the common pathways that induce the progression of pulmonary fibrosis, comprehending the diversity of these diseases, and identifying new molecular markers and potential therapeutic targets remain highly crucial assignments. The purpose of this review is to examine the main pathological mechanisms regulating the progression of fibrosis in interstitial lung diseases and to provide an overview of potential biomarker and therapeutic options for patients with progressive pulmonary fibrosis.
Download full-text PDF |
Source |
|---|---|
| http://www.ncbi.nlm.nih.gov/pmc/articles/PMC10890660 | PMC |
| http://dx.doi.org/10.3390/life14020229 | DOI Listing |
Publication Analysis
Top Keywords
Similar Publications
Airway obstruction and gender affect arterial stiffness in children with cystic fibrosis.
Turk J Pediatr
September 2025
Department of Cardiorespiratory Physiotherapy and Rehabilitation, Faculty of Physical Therapy and Rehabilitation, Hacettepe University, Ankara, Türkiye.
Background: Vascular changes are observed in children with cystic fibrosis (cwCF), and gender-specific differences may impact arterial stiffness. We aimed to compare arterial stiffness and clinical parameters based on gender in cwCF and to determine the factors affecting arterial stiffness in cwCF.
Methods: Fifty-eight cwCF were included.
Elexacaftor/Tezacaftor/Ivacaftor Effect on Bone Density and Body Composition: A Retrospective Analysis.
Pediatr Pulmonol
September 2025
Department of Medicine, Division of Diabetes, Endocrinology and Metabolism, University of Minnesota, Minneapolis, Minnesota, USA.
Background: The approval of cystic fibrosis transmembrane conductance regulator modulators elexacaftor/tezacaftor/ivacaftor (ETI), has significantly improved pulmonary function for people with cystic fibrosis (pwCF). However, the effects on CF-related bone disease and body composition remain unclear.
Methods: This retrospective real-world study examined adults with CF who received ETI treatment.
The Role of Gut Microbiota on Idiopathic Pulmonary Fibrosis Mediated by Circulating Inflammatory Proteins: A Two-Step, Two-Sample Mendelian Randomization Study.
Clin Respir J
September 2025
Department of Thoracic Surgery, Taizhou Hospital, Taizhou, Zhejiang, China.
Background: Persistent inflammation is a crucial characteristic of idiopathic pulmonary fibrosis (IPF). Gut microbiota (GM) contribute to the occurrence and development of several pulmonary diseases through the "gut-lung axis." The genetic role of GM in IPF and the mediating effect of circulating inflammatory proteins.
View Article and Find Full Text PDFIntroduction: Interstitial pneumonia with autoimmune features (IPAF) describes a rare condition characterized by interstitial lung disease (ILD) with autoimmune manifestations in the absence of defined autoimmune rheumatic diseases (AIRD). Although the classification was established in 2015, prospective data on disease progression remain limited.
Objectives: To identify predictors of ILD progression in IPAF patients using three criteria: 1) progressive pulmonary fibrosis (PPF), 2) INBUILD criteria, 3) absolute FVC decline ≥10%.
A Case Series: Pericardial Effusion in Patients Treated With Sotatercept.
Pulm Circ
July 2025
Division of Pulmonary, Critical Care, and Sleep Medicine Tufts Medical Center Boston Massachusetts USA.
Pulmonary arterial hypertension (PAH) is characterized by vasoconstriction, proliferation, fibrosis, and microthrombosis of the pulmonary vasculature, which causes elevated pulmonary arterial pressure and vascular resistance leading to right ventricular failure and death. Previous treatments targeted three known pathways involved in the development of PAH: endothelin, nitric oxide, and prostacyclin. Dysfunctional signaling of the transforming growth factor-beta (TGF-β) family, via bone morphogenetic protein (BMP) receptor 2 and activin signaling, has also been implicated in PAH leading to the development of a new class of therapies.
View Article and Find Full Text PDF