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Idiopathic pulmonary fibrosis (IPF) has traditionally been considered the archetype of progressive fibrotic interstitial lung diseases (f-ILDs), but several other f-ILDs can also manifest a progressive phenotype. Integrating genomic signatures into clinical practice for f-ILD patients may help to identify patients predisposed to a progressive phenotype. In addition to the risk of progressive pulmonary fibrosis, there is a growing body of literature examining how pharmacogenomics influences treatment response, particularly regarding the efficacy and safety profiles of antifibrotic and immunomodulatory agents. In this narrative review, we discuss current studies in IPF and other forms of pulmonary fibrosis, including systemic autoimmune disorders associated ILDs, sarcoidosis and hypersensitivity pneumonitis. We also provide insights into the future direction of research in this complex field.
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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC11274143 | PMC |
http://dx.doi.org/10.3390/biomedicines12071384 | DOI Listing |
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.
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%.
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 PDFCureus
August 2025
Acute Medicine, Southend University Hospital, Mid and South Essex NHS Foundation Trust, Southend-on-Sea, GBR.
Adenocarcinoma of the lung is the most common type of lung cancer and is classified as one of the non-small cell lung cancers. It typically arises in the peripheral regions of the lungs, affecting the dense glandular tissues. Most patients diagnosed with pulmonary adenocarcinoma are current or former smokers and present with nonspecific respiratory symptoms such as a persistent cough and shortness of breath.
View Article and Find Full Text PDF