Idiopathic pulmonary fibrosis (IPF) is a rare disorder concerning elderly people, predominantly men, active or former smokers, with a progressive nature and leading to premature mortality. The cause of the disease is unknown. However, there are some risk factors, among which genetic predisposition plays a role. The aim of our single-centered observational study was to assess the correlation between single nucleotide polymorphism (SNP) of the gene (rs35705950) and the disease course, antifibrotic treatment effect, and survival in patients with IPF. A total of 93 patients entered the study, of whom 88 were treated with either nintedanib or pirfenidone. The GG genotype was found in 28 (30.1%) subjects, while the GT or TT genotypes were found in the remaining 65 (63.4%) and 6 (6.5%) patients, respectively. The T allele minor allele frequency (MAF) accounted for 38.2% of the whole group. Patients with different genotypes did not differ significantly regarding age, sex, pulmonary function tests' results, response to the antifibrotic treatment, or survival. However, we found a survival advantage in female patients and patients with higher pre-treatment TL,co. Treatment with antifibrotics significantly decreased the magnitude of FVC and TL,co decline compared to the time before treatment initiation, regardless of status. In conclusion, we found high prevalence of T allele of gene in patients with IPF; however, it showed no influence on disease trajectory, survival, or antifibrotic treatment effect in the presented cohort.
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http://dx.doi.org/10.3390/ijms26052218 | DOI Listing |
Int J Mol Sci
March 2025
Ophthalmology Clinic, Department of Medicine and Aging Sciences, "G. d'Annunzio" University of Chieti-Pescara, 66100 Chieti, Italy.
Filtration bleb (FB) fibrosis represents the primary risk factor for glaucoma filtration surgery (GFS) failure. We reviewed the most recent literature on post-GFS fibrosis in humans, focusing on novel molecular pathways and antifibrotic treatments. Three main literature searches were conducted.
View Article and Find Full Text PDFInt J Mol Sci
February 2025
First Department of Lung Diseases, National Research Institute of Tuberculosis and Lung Diseases, Płocka 26, 01-138 Warsaw, Poland.
Idiopathic pulmonary fibrosis (IPF) is a rare disorder concerning elderly people, predominantly men, active or former smokers, with a progressive nature and leading to premature mortality. The cause of the disease is unknown. However, there are some risk factors, among which genetic predisposition plays a role.
View Article and Find Full Text PDFInt J Mol Sci
February 2025
Institute of Medicinal Biotechnology, Chinese Academy of Medical Science & Peking Union Medical College, Beijing 100050, China.
Liver fibrosis, caused by chronic hepatic injury, is a major threat to human health worldwide, as there are no specific drugs available for its treatment. Natural compounds, such as berberine (BBR) and quercetin (QR), have shown the ability to regulate energy metabolism and protect the liver without significant adverse effects. Additionally, combination therapy (the cocktail therapy approach), using multiple drugs, has shown promise in treating complicated conditions, including liver injury.
View Article and Find Full Text PDFCell Mol Life Sci
March 2025
Univ. Lille, Inserm, CHU Lille, Infinite U1286, Lille, F-59000, France.
Some interstitial lung diseases involve pulmonary fibrosis, which is a process that is characterized by the excessive and abnormal accumulation of extracellular matrix in the pulmonary interalveolar space. Although the current anti-fibrotic therapy aims at slowing down the progression of pulmonary fibrosis, it does not reverse it, and many of the drugs that were identified in basic-research studies failed in clinical phases, mainly because of the lack of a model that can recapitulate the pathophysiological mechanisms of human pulmonary fibrosis. We developed a novel experimental model of pulmonary fibrosis induced by a cocktail of molecules on an air/liquid interface culture of mouse embryonic lung explants.
View Article and Find Full Text PDFJ Pharmacol Exp Ther
February 2025
Idorsia Pharmaceuticals Ltd, Allschwil, Switzerland.
Pulmonary fibrosis encompasses different chronic interstitial lung diseases, and the predominant form, idiopathic pulmonary fibrosis, remains to have a poor prognosis despite 2 approved therapies. Although the exact pathobiological mechanisms are still incompletely understood, epithelial injury and aberrant wound healing responses contribute to the gradual change in lung architecture and functional impairment. Lysophosphatidic acid (LPA)-induced lysophosphatidic receptor 1 (LPA1) signaling was proposed to be a driver of lung fibrosis, and LPA1 antagonists have shown promising antifibrotic profiles in early clinical development.
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