Effect of genotype on the disease course in idiopathic pulmonary fibrosis despite antifibrotic treatment

Šterclová, Martina; Kishore, Amit; Sikorova, Katerina; Skibová, J; Petřek, Martin; Vašáková, Martina

doi:10.3892/br.2021.1463

Cited by 4 publications

(3 citation statements)

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“…Reduced TOLLIP expression after TLR stimulation leads to increased production of proinflammatory cytokines such as IL-6 and TNF by macrophages [246]. These results suggest that the reduction in the proinflammatory and profibrotic cascade due to TOLLIP expression may have protective effects [243].…”

Section: Toll-interactin Proteinmentioning

confidence: 97%

“…Activation of TLR3 receptors in primary fibroblasts results in decreased TGF synthesis, increased collagen production, and higher metalloproteinase activity, which can also have an antifibrotic effect [242]. A deficiency in TLR3 signaling may lead to an inadequate lung response to viral pathogens, exposing it to chronic cycles of damage and repair believed to underlie IPF pathology [243].…”

Section: Toll-like Receptormentioning

confidence: 99%

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Idiopathic Pulmonary Fibrosis: Where do We Stand and How Far to Go?

Singh,

Ulasov,

Gupta

et al. 2024

Discovery Medicine

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Idiopathic pulmonary fibrosis is a progressive and incurable lung disease characterized by collagen deposition, alveolar inflammation, fibroblast proliferation, and the destruction of lung tissue structures. It is a rare yet severe condition with a high mortality rate, typically leading to death within 3-5 years of diagnosis. The clinical presentation of idiopathic pulmonary fibrosis (IPF) involves a gradual and substantial loss of lung function, ultimately resulting in respiratory failure. Despite more than half a century of intensive research, the origin of IPF remains a mystery. Despite its unknown etiology, several genetic and non-genetic factors have been linked to IPF. Recent significant advancements have been made in the field of IPF diagnosis and treatment. Two oral small-molecule drugs, pirfenidone and nintedanib, have recently gained approval for the treatment of IPF. Pirfenidone exhibits antifibrotic, antioxidant, and anti-inflammatory properties, while nintedanib is a tyrosine kinase inhibitor with selectivity for vascular endothelial growth factor (VEGF) receptors, prostaglandin F (PGF) receptors, and fibroblast growth factor (FGF) receptors. Both of these compounds are capable of slowing down the progression of the disease with an acceptable safety profile. This review provides a brief introduction, historical background, epidemiological insights, and an exploration of various environmental risk factors that may influence the lung microenvironment and contribute to the advancement of IPF. The review also delves into the diagnosis, signaling pathways, and ongoing clinical trials worldwide. A thorough review of the literature was conducted using PubMed and Google Scholar to gather information on various aspects of IPF. Numerous potential drugs are currently under investigation in clinical trials, and the completion of this process is crucial to the ultimate goal of finding a cure for IPF patients. The investigation of the role of genes, surfactant proteins, infectious agents, biomarkers, and epigenetic changes holds the promise of offering earlier and more accurate understanding and diagnosis of IPF. This information could be instrumental in the development of new therapeutic approaches for treating IPF and is expected to be of great interest to researchers.

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Section: Toll-interactin Proteinmentioning

confidence: 97%

Section: Toll-like Receptormentioning

confidence: 99%

Idiopathic Pulmonary Fibrosis: Where do We Stand and How Far to Go?

Singh,

Ulasov,

Gupta

et al. 2024

Discovery Medicine

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“…MUC5B and TOLLIP reside at the same genetic locus. If IPF is also associated with the TOLLIP promoter SNP, the risk of death is higher 160 . The independence of the association signals at 11p15.5 suggests that multiple variants of this locus may be influencing disease susceptibility and course 127 .…”

Section: Introductionmentioning

confidence: 99%

Genomic Fingerprint Associated with Familial Idiopathic Pulmonary Fibrosis: A Review

Ding¹,

Gao²,

Xue³

et al. 2023

Int. J. Med. Sci.

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Idiopathic pulmonary fibrosis (IPF) is a severe interstitial lung disease; although the recent introduction of two anti-fibrosis drugs, pirfenidone and Nidanib, have resulted in a significant reduction in lung function decline, IPF is still not curable. Approximately 2-20% of patients with IPF have a family history of the disease, which is considered the strongest risk factor for idiopathic interstitial pneumonia. However, the genetic predispositions of familial IPF (f-IPF), a particular type of IPF, remain largely unknown. Genetics affect the susceptibility and progression of f-IPF. Genomic markers are increasingly being recognized for their contribution to disease prognosis and drug therapy outcomes. Existing data suggest that genomics may help identify individuals at risk for f-IPF, accurately classify patients, elucidate key pathways involved in disease pathogenesis, and ultimately develop more effective targeted therapies. Since several genetic variants associated with the disease have been found in f-IPF, this review systematically summarizes the latest progress in the gene spectrum of the f-IPF population and the underlying mechanisms of f-IPF. The genetic susceptibility variation related to the disease phenotype is also illustrated. This review aims to improve the understanding of the IPF pathogenesis and facilitate his early detection.

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