نتائج البحث - "Pirfenidone"

1

دورية أكاديمية

Pirfenidone increases transverse tubule length in the infarcted rat myocardium

المؤلفون: Moammer, Hussam, Bai, Jizhong, Jones, Timothy LM, Ward, Marie, Barrett, Caroyln, Crossman, David J

جغرافية الموضوع: England

الوصف: Transverse (t)-tubule remodelling is a prominent feature of heart failure with reduced ejection fraction (HFrEF). In our previous research, we identified an increased amount of collagen within the t-tubules of HFrEF patients, suggesting fibrosis could contribute to the remodelling of t-tubules. In this research, we tested this hypothesis in a rodent model of myocardial infarction induced heart failure that was treated with the anti-fibrotic pirfenidone. Confocal microscopy demonstrated loss of t-tubules within the border zone region of the infarct. This was documented as a reduction in t-tubule frequency, area, length, and transverse elements. Eight weeks of pirfenidone treatment was able to significantly increase the area and length of the t-tubules within the border zone. Echocardiography showed no improvement with pirfenidone treatment. Surprisingly, pirfenidone significantly increased the thickness of the t-tubules in the remote left ventricle of heart failure animals. Dilation of t-tubules is a common feature in heart failure suggesting this may negatively impact function but there was no functional loss associated with pirfenidone treatment. However, due to the relatively short duration of treatment compared to that used clinically, the impact of long-term treatment on t-tubule structure should be investigated in future studies.

وصف الملف: Electronic-eCollection; application/pdf

العلاقة: Interface focus; (2023). Interface Focus, 13(6), 20230047-.; https://hdl.handle.net/2292/67604Test; 38106917 (pubmed); rsfs20230047

الإتاحة: https://doi.org/10.1098/rsfs.2023.0047Test
https://hdl.handle.net/2292/67604Test

View record in BASE

2

Role of pirfenidone in TGF-β pathways and other inflammatory pathways in acute respiratory syndrome coronavirus 2 (SARS-Cov-2) infection: a theoretical perspective

المؤلفون: Sandhya Kadamboor Veethil, Seyed Hootan Hamidi, Seyedeh Harir Hamidi

المصدر: Pharmacological Reports

مصطلحات موضوعية: Epithelial sodium channel, Pyridones, Pathology of COVID-19, Inflammation, Review, Pharmacology, Lung injury, medicine.disease_cause, TGF-β signaling, SARS-CoV-2 mechanism, chemistry.chemical_compound, Transforming Growth Factor beta, Medicine, Humans, Coronavirus, biology, business.industry, SARS-CoV-2, Anti-Inflammatory Agents, Non-Steroidal, Pirfenidone mechanism of action, COVID-19, General Medicine, Pirfenidone, Lung Injury, Cystic fibrosis transmembrane conductance regulator, COVID-19 Drug Treatment, chemistry, Plasminogen activator inhibitor-1, biology.protein, COVID-19 therapy, medicine.symptom, business, Transforming growth factor, medicine.drug, Signal Transduction

الوصف: Background Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) causes pulmonary injury or multiple-organ injury by various pathological pathways. Transforming growth factor-beta (TGF-β) is a key factor that is released during SARS-CoV-2 infection. TGF-β, by internalization of the epithelial sodium channel (ENaC), suppresses the anti-oxidant system, downregulates the cystic fibrosis transmembrane conductance regulator (CFTR), and activates the plasminogen activator inhibitor 1 (PAI-1) and nuclear factor-kappa-light-chain-enhancer of activated B cells (NF-kB). These changes cause inflammation and lung injury along with coagulopathy. Moreover, reactive oxygen species play a significant role in lung injury, which levels up during SARS-CoV-2 infection. Drug Suggestion Pirfenidone is an anti-fibrotic drug with an anti-oxidant activity that can prevent lung injury during SARS-CoV-2 infection by blocking the maturation process of transforming growth factor-beta (TGF-β) and enhancing the protective role of peroxisome proliferator-activated receptors (PPARs). Pirfenidone is a safe drug for patients with hypertension or diabetes and its side effect tolerated well. Conclusion The drug as a theoretical perspective may be an effective and safe choice for suppressing the inflammatory response during COVID-19. The recommendation would be a combination of pirfenidone and N-acetylcysteine to achieve maximum benefit during SARS-CoV-2 treatment.

الوصول الحر: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::88c57b2f218e93b6faa6abde85e9a200Test
http://europepmc.org/articles/PMC8057922Test

3

Pirfenidone modifies hepatic miRNAs expression in a model of MAFLD/NASH

المؤلفون: C. Alejandra Monraz-Méndez, Rebeca Escutia-Gutiérrez, Ana Sandoval-Rodriguez, Juan Armendáriz-Borunda, Arturo Santos-Garcia, J. Samael Rodríguez-Sanabria, Jesús García-Bañuelos

المصدر: Scientific Reports, Vol 11, Iss 1, Pp 1-12 (2021)
Scientific Reports

مصطلحات موضوعية: 0301 basic medicine, Male, medicine.medical_specialty, Pyridones, Science, Inflammation, Biology, Article, Collagen Type I, Transcriptome, 03 medical and health sciences, Mice, 0302 clinical medicine, Insulin resistance, Liver Function Tests, Non-alcoholic Fatty Liver Disease, Internal medicine, microRNA, medicine, Animals, Lipid Metabolic Process, Liver diseases, Non-alcoholic steatohepatitis, Multidisciplinary, Hepatology, Fatty liver, Gastroenterology, Lipid metabolism, Pirfenidone, medicine.disease, Lipid Metabolism, Immunohistochemistry, Fatty Liver, Disease Models, Animal, MicroRNAs, 030104 developmental biology, Endocrinology, Gene Expression Regulation, Cytokines, Medicine, 030211 gastroenterology & hepatology, Disease Susceptibility, medicine.symptom, Inflammation Mediators, Biomarkers, medicine.drug

الوصف: miRNAs are involved in the development of metabolic associated fatty liver disease (MAFLD) and nonalcoholic steatohepatitis (NASH). We aimed to evaluate modifications by prolonged-release pirfenidone (PR-PFD) on key hepatic miRNAs expression in a MAFLD/NASH model. First, male C57BL/6J mice were randomly assigned into groups and fed with conventional diet (CVD) or high fat and carbohydrate diet (HFD) for 16 weeks. At the end of the eighth week, HFD mice were divided in two and only one half was treated with 300 mg/kg/day of PR-PFD mixed with food. Hepatic expression of miRNAs and target genes that participate in inflammation and lipid metabolism was determined by qRT-PCR and transcriptome by microarrays. Increased hepatic expression of miR-21a-5p, miR-34a-5p, miR-122-5p and miR-103-3p in MAFLD/NASH animals was reduced with PR-PFD. Transcriptome analysis showed that 52 genes involved in lipid and collagen biosynthesis and inflammatory response were downregulated in PR-PFD group. The expression of Il1b, Tnfa, Il6, Tgfb1, Col1a1, and Srebf1 were decreased in PR-PFD treated animals. MAFLD/NASH animals compared to CVD group showed modifications in gene metabolic pathways implicated in lipid metabolic process, inflammatory response and insulin resistance; PR-PFD reversed these modifications.

الوصول الحر: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::6f995af61f22c62a8c498cfcc372d922Test
https://doaj.org/article/3d0a5ea6aa3f4130aece7fe0791813bcTest

4

Effect of GLPG1205, a GPR84 Modulator, on CYP2C9, CYP2C19, and CYP1A2 Enzymes: In Vitro and Phase 1 Studies

المؤلفون: Lisa Allamassey, Eric Helmer, Julie Desrivot, Tim Van Kaem

المصدر: Clinical Pharmacology in Drug Development

مصطلحات موضوعية: Adult, Male, cytochrome P450, Metabolite, Pharmaceutical Science, Original Manuscript, CYP2C19, GLPG1205, Pharmacology, 030226 pharmacology & pharmacy, Receptors, G-Protein-Coupled, 03 medical and health sciences, chemistry.chemical_compound, 0302 clinical medicine, Pharmacokinetics, drug‐drug interaction, Double-Blind Method, Cytochrome P-450 CYP1A2, medicine, Humans, Pharmacology (medical), Drug Interactions, Cytochrome P-450 CYP2C9, Cross-Over Studies, biology, business.industry, CYP1A2, Cytochrome P450, Pirfenidone, Articles, Middle Aged, idiopathic pulmonary fibrosis, Isoquinolines, Crossover study, Cytochrome P-450 CYP2C19, Tolerability, chemistry, 030220 oncology & carcinogenesis, biology.protein, business, medicine.drug

الوصف: GLPG1205 is a novel agent being investigated for the treatment of idiopathic pulmonary fibrosis. GLPG1205 may be concomitantly administered with pirfenidone in future clinical development; therefore, the potential for GLPG1205 to interact with enzymes involved in the metabolism of pirfenidone (cytochrome P450 [CYP] 1A2, CYP2C9, 2C19) was evaluated. In vitro experiments indicated weak inhibition of CYP1A2 and moderate but reversible inhibition of CYP2C9 and CYP2C19 by GLPG1205. A phase 1 randomized, double‐blind crossover study in 14 healthy males (NCT02623296) evaluated the effect of GLPG1205 100 mg or placebo (once daily for 12 days) on the single‐dose pharmacokinetics of a cocktail of CYP1A2, CYP2C9, and CYP2C19 substrates (coadministered on day 13). GLPG1205 had no effect on the exposure of CYP2C9 and CYP1A2 substrates or metabolites; however, a trend toward increased omeprazole (CYP2C19 substrate) exposure was observed. Although considered not clinically relevant, GLPG1205 increased the elimination rate of 5‐hydroxyomeprazole (CYP2C19 metabolite) 1.16‐fold versus placebo. GLPG1205 had no effect on the elimination of all other substrates or metabolites. GLPG1205 had a favorable safety and tolerability profile. In conclusion, GLPG1205 100 mg once daily does not interact with CYP2C9, CYP2C19, or CYP1A2 to a clinically relevant extent and may be administered concomitantly with drugs metabolized by these enzymes.

الوصول الحر: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::83d3469d701a2bae30c9359dadb5a2a0Test
http://europepmc.org/articles/PMC8453848Test

5

Pirfenidone Is an Agonistic Ligand for PPARα and Improves NASH by Activation of SIRT1/LKB1/pAMPK

المؤلفون: Mónica Vázquez-Del Mercado, Hugo Christian Monroy-Ramirez, Jorge Gutiérrez-Cuevas, Jesús García-Bañuelos, José Alfredo Domínguez-Rosales, Jose Navarro-Partida, Jose Vera-Cruz, Arturo Santos-Garcia, Ana Sandoval-Rodriguez, Juan Armendáriz-Borunda, Jorge Silva-Gomez, Alejandra Meza-Rios, Marina Galicia-Moreno, Bart Staels

المصدر: Hepatology Communications, Vol 4, Iss 3, Pp 434-449 (2020)
Hepatology Communications

الوصف: Nonalcoholic steatohepatitis (NASH) is recognized by hepatic lipid accumulation, inflammation, and fibrosis. No studies have evaluated the prolonged‐release pirfenidone (PR‐PFD) properties on NASH features. The aim of this study is to evaluate how PR‐PFD performs on metabolic functions, and provide insight on a mouse model of human NASH. Male C57BL/6J mice were fed with either normo diet or high‐fat/carbohydrate diet for 16 weeks and a subgroup also fed with PR‐PFD (300 mg/kg/day). An insulin tolerance test was performed at the end of treatment. Histological analysis, determination of serum hormones, adipocytokines measurement, and evaluation of proteins by western blot was performed. Molecular docking, in silico site‐directed mutagenesis, and in vitro experiments using HepG2 cultured cells were performed to validate PR‐PFD binding to peroxisome proliferator–activated receptor alpha (PPAR‐α), activation of PPAR‐α promoter, and sirtuin 1 (SIRT1) protein expression. Compared with the high‐fat group, the PR‐PFD‐treated mice displayed less weight gain, cholesterol, very low density lipoprotein and triglycerides, and showed a significant reduction of hepatic macrosteatosis, inflammation, hepatocyte ballooning, fibrosis, epididymal fat, and total adiposity. PR‐PFD restored levels of insulin, glucagon, adiponectin, and resistin along with improved insulin resistance. Noteworthy, SIRT1–liver kinase B1–phospho‐5′ adenosine monophosphate–activated protein kinase signaling and the PPAR‐α/carnitine O‐palmitoyltransferase 1/acyl‐CoA oxidase 1 pathway were clearly induced in high fat + PR‐PFD mice. In HepG2 cells incubated with palmitate, PR‐PFD induced activation and nuclear translocation of both PPARα and SIRT1, which correlated with increased SIRT1 phosphorylated in serine 47, suggesting a positive feedback loop between the two proteins. These results were confirmed with both synthetic PPAR‐α and SIRT1 activators and inhibitors. Finally, we found that PR‐PFD is a true agonist/ligand for PPAR‐α. Conclusions: PR‐PFD provided an anti‐steatogenic effect and protection for inflammation and fibrosis.
Pirfenidone is an agonist/ligand for PPAR‐α and improves inflammation, fibrosis, insulin sensitivity, and fat metabolism in a mouse model of human NASH through the SIRT1‐LKB1‐pAMPK noncanonical pathway.

الوصول الحر: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::99af8219088eaf33fb768ca2bebfe3a1Test
https://doi.org/10.1002/hep4.1474Test

6

Pirfenidone attenuates the profibrotic contractile phenotype of differentiated human dermal myofibroblasts

المؤلفون: Adrienne R. Wells, Kai P. Leung

المصدر: Biochemical and Biophysical Research Communications. 521:646-651

الوصف: Dysregulated wound healing after burn injury frequently results in debilitating hypertrophic scarring and contractures. Myofibroblasts, the main effector cells for dermal fibrosis, develop from normal fibroblasts via transforming growth factor beta 1 (TGF-β1). During wound healing, myofibroblasts produce extracellular matrix (ECM) proteins, modulate ECM stability, and contract the ECM using alpha smooth muscle actin (α-SMA) in contractile stress fibers. The antifibrotic pirfenidone has previously been shown to inhibit the initial differentiation of fibroblasts into myofibroblasts in vitro and act as a prophylactic measure against hypertrophic scar development in a mouse burn model. To test whether pirfenidone affects differentiated myofibroblasts, we investigated the in vitro effects of pirfenidone treatment after three to five days of stimulation with TGF-β1. In assays for morphology, protein and gene expression, and contractility, pirfenidone treatment produced significant effects. Profibrotic gene expression returned to near-normal levels, further α-SMA protein expression was prevented, and cell contraction within a stressed collagen matrix was reduced. These in vitro results promote pirfenidone as a promising antifibrotic agent to treat existing scars and healing wounds by mitigating the effects of differentiated myofibroblasts.

الوصول الحر: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::6185a19547373fc1d03131589e22d868Test
https://doi.org/10.1016/j.bbrc.2019.10.177Test

7

Protective role of pirfenidone against experimentally-induced pancreatitis

المؤلفون: Ahmed A. Shaaban, Dalia H. El-Kashef, Dina S. El-Agamy

المصدر: Pharmacological Reports. 71:774-781

الوصف: Background Pirfenidone (PFD) is an orally active antifibrotic agent that has anti-inflammatory activity in diverse animal models. Its effect against acute pancreatitis (AP) has not been elucidated. Hence, the present investigation was carried out to assess the potential protective role of PFD against l -arginine-induced AP in mice. Methods AP was induced in adult male Swiss albino mice via intraperitoneal injections of l -arginine (4 g/kg, twice each 1 h apart). PFD (250 mg/kg, orally) was administered one day before and on the day of l -arginine challenge. Twenty-four hours after l -arginine injection, the severity of AP was evaluated using biochemical and histological analyses. Indices of oxidative stress, inflammation and apoptosis were evaluated using ELISA and immunohistochemistry (IHC). Results PFD suppressed the development of l -arginine-induced AP as revealed by the improvement of histopathological lesions of pancreatic specimen and the significant reduction of serum amylase and lipase levels. Notably, PFD reduced the lipid peroxidation and enhanced the antioxidants such as reduced glutathione (GSH) and superoxide dismutase (SOD) in pancreatic tissue. Importantly, PFD suppressed AP-associated elevation of inflammatory cytokines along with depression of nuclear factor kappa-B (NF-κB) immuno-expression in pancreatic tissue. Lastly, PFD efficiently ameliorated AP-induced elevation of the pro-apoptotic protein (Bax) and increased AP-induced reduction of the anti-apoptotic protein (Bcl2). Conclusions PFD protected against l -arginine-induced AP in mice through anti-oxidative, anti-inflammatory and anti-apoptotic properties.

الوصول الحر: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::465b261e2c7cb97806a427b2b0af51e0Test
https://doi.org/10.1016/j.pharep.2019.04.005Test

8

Dual inhibition of αvβ6 and αvβ1 reduces fibrogenesis in lung tissue explants from patients with IPF

المصدر: Respiratory research, vol 22, iss 1
Respiratory Research, Vol 22, Iss 1, Pp 1-14 (2021)

مصطلحات موضوعية: Precision-cut lung slice, Respiratory System, αv integrin, alpha(v) integrin, Cardiorespiratory Medicine and Haematology, Inbred C57BL, Mice, Idiopathic pulmonary fibrosis, chemistry.chemical_compound, Receptors, Pulmonary fibrosis, Phosphorylation, alpha 1 Chain, Lung, biology, Transforming growth factor-β, Pirfenidone, Transforming growth factor-beta, medicine.anatomical_structure, 5.1 Pharmaceuticals, Respiratory, Nintedanib, Antifibrotic Agents, Development of treatments and therapeutic interventions, Cell activation, Type I collagen, Signal Transduction, medicine.drug, Integrin, Clinical Sciences, Collagen Type I, Cell Line, Diseases of the respiratory system, Bleomycin, Rare Diseases, medicine, Animals, Humans, Vitronectin, Smad3 Protein, PLN-74809, RC705-779, Integrin alpha6beta1, Animal, Epithelial Cells, Fibroblasts, medicine.disease, Coculture Techniques, Idiopathic Pulmonary Fibrosis, chemistry, Disease Models, Cancer research, biology.protein, Antifibrotic

الوصف: Rationale αv integrins, key regulators of transforming growth factor-β activation and fibrogenesis in in vivo models of pulmonary fibrosis, are expressed on abnormal epithelial cells (αvβ6) and fibroblasts (αvβ1) in fibrotic lungs. Objectives We evaluated multiple αv integrin inhibition strategies to assess which most effectively reduced fibrogenesis in explanted lung tissue from patients with idiopathic pulmonary fibrosis. Methods Selective αvβ6 and αvβ1, dual αvβ6/αvβ1, and multi-αv integrin inhibitors were characterized for potency, selectivity, and functional activity by ligand binding, cell adhesion, and transforming growth factor-β cell activation assays. Precision-cut lung slices generated from lung explants from patients with idiopathic pulmonary fibrosis or bleomycin-challenged mouse lungs were treated with integrin inhibitors or standard-of-care drugs (nintedanib or pirfenidone) and analyzed for changes in fibrotic gene expression or TGF-β signaling. Bleomycin-challenged mice treated with dual αvβ6/αvβ1 integrin inhibitor, PLN-74809, were assessed for changes in pulmonary collagen deposition and Smad3 phosphorylation. Measurements and main results Inhibition of integrins αvβ6 and αvβ1 was additive in reducing type I collagen gene expression in explanted lung tissue slices from patients with idiopathic pulmonary fibrosis. These data were replicated in fibrotic mouse lung tissue, with no added benefit observed from inhibition of additional αv integrins. Antifibrotic efficacy of dual αvβ6/αvβ1 integrin inhibitor PLN-74809 was confirmed in vivo, where dose-dependent inhibition of pulmonary Smad3 phosphorylation and collagen deposition was observed. PLN-74809 also, more potently, reduced collagen gene expression in fibrotic human and mouse lung slices than clinically relevant concentrations of nintedanib or pirfenidone. Conclusions In the fibrotic lung, dual inhibition of integrins αvβ6 and αvβ1 offers the optimal approach for blocking fibrogenesis resulting from integrin-mediated activation of transforming growth factor-β.

وصف الملف: application/pdf

الوصول الحر: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::d8943da51a8a3553a1e3711bc9a9ed07Test
https://escholarship.org/uc/item/5q12m0vqTest

9

Pirfenidone Reduces Epithelial–Mesenchymal Transition and Spheroid Formation in Breast Carcinoma through Targeting Cancer-Associated Fibroblasts (CAFs)

المؤلفون: Jean Paul Thiery, Ehsan Sarafraz-Yazdi, Thomas R. Cox, Hamidreza Aboulkheyr Es, Majid Ebrahimi Warkiani

المصدر: Cancers, Vol 13, Iss 5118, p 5118 (2021)
Cancers
Volume 13
Issue 20

الوصف: Simple Summary Cancer-associated fibroblasts (CAFs) stimulate phenotypic transformation and acquisition of stemness in carcinoma cells. Targeting CAF-derived cytokines may suppress initiation of these events. This study aimed to show the inhibitory effects of pirfenidone on phenotypic transformation and stemness of cancer cells. To this end, we leverage the use of a 3D microfluidic device to analyze carcinoma progression phenotypes. We found that pirfenidone decreased tumor spheroid formation and epithelial–mesenchymal transition (EMT) the inhibition of cytokine production by CAFs. In the microfluidic model, we demonstrate that pirfenidone significantly inhibits the migration of carcinoma cells and CAFs. This study highlights the potential application of pirfenidone in suppressing invasion and potentially metastasis in breast cancer which can be further investigated in vivo. Abstract The aim of this study was to assess the effects of pirfenidone (PFD) on promoting epithelial–mesenchymal-transition (EMT) and stemness features in breast carcinoma cells through targeting cancer-associated-fibroblasts (CAFs). Using The Cancer Genome Atlas (TCGA) database, we analyzed the association between stromal index, EMT, and stemness-related genes across 1084 breast cancer patients, identifying positive correlation between YAP1, EMT, and stemness genes in samples with a high-stromal index. We monitored carcinoma cell invasion and spheroid formation co-cultured with CAFs in a 3D microfluidic device, followed by exposing carcinoma cells, spheroids, and CAFs with PFD. We depicted a positive association between the high-stromal index and the expression of EMT and stemness genes. High YAP1 expression in samples correlated with more advanced EMT status and stromal index. Additionally, we found that CAFs promoted spheroid formation and induced the expression of YAP1, VIM, and CD44 in spheroids. Treatment with PFD reduced carcinoma cell migration and decreased the expression of these genes at the protein level. The cytokine profiling showed significant depletion of various EMT- and stemness-regulated cytokines, particularly IL8, CCL17, and TNF-beta. These data highlight the potential application of PFD on inhibiting EMT and stemness in carcinoma cells through the targeting of critical cytokines.

وصف الملف: Electronic; application/pdf

الوصول الحر: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::5ce3f2614fe10f065424cc6e6c6fca06Test
https://hdl.handle.net/10453/167090Test

10

DSP rs2076295 variants influence nintedanib and pirfenidone outcomes in idiopathic pulmonary fibrosis: a pilot study

المؤلفون: Vladimira Lostakova, Radka Bittenglova, Simona Littnerová, Pavlína Lisá, Eva Kriegova, Tomas Snizek, Hana Suldova, Michael Doubek, Martina Plačková, Martina Sterclova, Pavlina Musilova, Martina Vasakova, Lenka Šišková, Vladimir Bartos, Martina Doubková, Jana Psikalova, Petra Schneiderova, Monika Zurkova

المصدر: Ther Adv Respir Dis
Therapeutic Advances in Respiratory Disease, Vol 15 (2021)

الوصف: Background: The antifibrotic drugs nintedanib and pirfenidone are used for the treatment of idiopathic pulmonary fibrosis (IPF). We analysed the association of common profibrotic polymorphisms in MUC5B (mucin 5B, rs35705950) and DSP (desmoplakin, rs2076295) on antifibrotic treatment outcomes in IPF. Methods: MUC5B rs35705950 and DSP rs2076295 were assessed in IPF patients ( n = 210, 139 men/71 women) from the Czech EMPIRE registry and age- or sex-matched healthy individuals ( n = 205, 125 men/80 women). Genetic data were collated with overall survival (OS), acute exacerbation episodes, worsening lung function and antifibrotic treatment. Results: We confirmed overexpression of the MUC5B rs35705950*T allele (55.2% versus 20.9%, p CO (diffuse lung capacity) at the IPF diagnosis were associated with survival. Conclusion: Our real-world study showed that IPF patients with MUC5B T* allele or DSP G* allele profit from antifibrotic treatment by lower mortability. Moreover, carriers of the DSP rs2076295*G allele benefit from treatment with nintedanib, and TT genotype from treatment with pirfenidone. MUC5B rs35705950 did not impact the outcome of treatment with either nintedanib or pirfenidone. Our single-registry pilot study should be confirmed with an independent patient cohort.

الوصول الحر: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::085ba847fd6bdfb3daff573e1c43b896Test
https://europepmc.org/articles/PMC8442489Test/

تنقيح النتائج