Contemporary Concise Review 2018: Interstitial lung disease

Mari, Pier‐Valerio; Jones, Mark G.; Richeldi, Luca

doi:10.1111/resp.13572

Cited by 7 publications

(4 citation statements)

References 100 publications

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“…Interstitial pulmonary fibrosis (IPF), also known as interstitial lung disease (ILD), is a collection of diseases with diffuse exudation, infiltration and fibrosis [1]. Up to now, western treatments for IPF mainly include glucocorticoid immunosuppressant, anti-fibrosis drug, antacid drug, oxygen therapy, mechanical ventilation, pulmonary rehabilitation, and lung transplantation [2,3].…”

Section: Introductionmentioning

confidence: 99%

Necrostatin-1 Alleviates Bleomycin-Induced Pulmonary Fibrosis and Extracellular Matrix Expression in Interstitial Pulmonary Fibrosis

Mou

2020

Med Sci Monit

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Departmental sources Background:Interstitial pulmonary fibrosis (IPF) is harmful for patients' life and health. The effective treatment of IPF is lacking because of unclear pathogenesis. Necrostatin-1 has protective effects on lung injury and can suppress the fibrosis development. I this study we investigated whether necrostatin-1 could decrease the proliferation of pulmonary fibroblasts, pulmonary fibrosis and expression of extracellular matrix (ECM) in IPF. Material/Methods:The IPF mice model was conducted by intra-tracheal injection of bleomycin (BLM) (2 mg/kg) for C57BL/6N mice. Necrostatin-1 treatment was performed with 1 mg/kg necrostatin-1 by an intravenous injection for C57BL/6N mice. Lung tissue structures and collagen deposition were observed by hematoxylin and eosin staining and Masson staining. IPF in vitro model was constructed by MRC-5 cells induced by transforming growth factor beta 1 (TGF-b1). And, 20 μM necrostatin-1 was used to treat the TGF-b1 induced MRC-5 cells. Cell Counting Kit-8 (CCK-8) assay detected the viability of MRC-5 cells. The expression of receptor-interacting protein kinase-1 and -3 (RIPK1 and RIPK3), a smooth muscle actin (a-SMA), collagen IV, collagen I, fibronectin (FN), and transforming growth factor-b (TGF-b) in lung tissues and MRC-5 cells was measured by western blot analysis. The a-SMA expression in lung tissues was also analyzed by immunohistochemistry. Results:The expression of RIPK1 and RIPK3 in lung tissues of BLM induced mice was increased. The degree of pulmonary fibrosis and expression of a-SMA, collagen IV, collagen I, FN, and TGF-b in lung tissues of BLM induced mice was enhanced. The proliferation of MRC-5 cells was increased when MRC-5 cells were induced by TGF-b. The expression of RIPK1, RIPK3, a-SMA, collagen IV, collagen I, and FN was increased in TGF-b induced MRC-5 cells. And, necrostatin-1 could effectively reverse the changes of pulmonary fibrosis, RIPK1, RIPK3, and ECM in vivo and in vitro experiments. Conclusions:Necrostatin-1 attenuated pulmonary fibrosis in lung tissues of BLM induced mice and inhibited the fibroblast proliferation. And, necrostatin-1 also decreased the expression of RIPK1, RIPK3, and ECM in lung tissues of BLM induced mice and TGF-b induced fibroblasts. Necrostatin-1 could be a new effective drug for the treatment of IPF.

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Section: Introductionmentioning

confidence: 99%

Necrostatin-1 Alleviates Bleomycin-Induced Pulmonary Fibrosis and Extracellular Matrix Expression in Interstitial Pulmonary Fibrosis

Mou

2020

Med Sci Monit

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“…IPF is a severe and fatal fibrotic lung disease of unknown cause, leading to aberrant lung tissue remodeling, excessive scarring, loss of tissue compliance and respiratory failure (Mari, Jones, and Richeldi 2019). Here, we used a reference IPF human dataset consisting of microarray gene expression readouts of lungs from control and IPF patients (Y.…”

Section: Resultsmentioning

confidence: 99%

In Silico Treatment: a computational framework for animal model selection and drug assessment

Picart-Armada,

Becker,

Kaestle

et al. 2024

Preprint

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The translation of findings from animal models to human disease is a fundamental part in the field of drug development. However, only a small proportion of promising preclinical results in animals translate to human pathophysiology. This underscores the necessity for novel data analysis strategies to accurately evaluate the most suitable animal model for a specific purpose, ensuring cross-species translatability. To address this need, we presentIn SilicoTreatment (IST), a computational method to assess translation of disease-related molecular expression patterns between animal models and humans. By simulating changes observed in animals onto humans, IST provides a holistic picture of how well animal models recapitulate key aspects of human disease, or how treatments transform pathogenic expression patterns to healthy ones. Furthermore, IST highlights particular genes that influence molecular features of pathogenesis or drug mode of action. We demonstrate the potential of IST with three applications using bulk transcriptomics data. First, we assessed two mouse models for idiopathic pulmonary fibrosis (IPF): one involving injury with intra-tubular Bleomycin exposure, and the other Adeno-associated-virus-induced, TGFβ1-mediated tissue transformation (AAV6.2-TGFβ1). Both models exhibited gene expression patterns resembling extracellular matrix derangement in human IPF, whereas differences in VEGF-driven vascularization were observed. Second, we confirmed known features of non-alcoholic steatohepatitis (NASH) mouse models, including choline-deficient, l-amino acid-defined diet (CDAA), carbon tetrachloride hepatotoxicity injury (CCl4) and bile duct ligation surgery (BDL). Overall, the three mouse models recapitulated expression changes related to fibrosis in human NASH, whereas model-specific differences were found in lipid metabolism, inflammation, and apoptosis. Third, we reproduced the strong anti-fibrotic signature and induction of the PPARα signaling observed in the Elafibranor experimental treatment for NASH in the CDAA model. We validated the contribution of known disease-related genes to the findings made with IST in the IPF and NASH applications. The complete data integration IST framework, including an interactive app to integrate and compare datasets, is made available as an open-source R package.Author summaryPreclinical testing plays a pivotal role in the drug development process, serving as a crucial evaluation phase before a new drug can be tested on humans in clinical trials. The drug must undergo a rigorous evaluation inin vivoandin vitropreclinical studies to assess its safety and efficacy. However, positive outcomes in preclinical animal models do not always translate to positive results in humans, mainly due to biological differences. Therefore, selecting an animal model that closely mirrors human disease traits and detecting and accounting for model limitations is of paramount importance.Over the last decade, the availability of gene expression data in both animals and humans has substantially increased. Gene expression states and perturbations are routinely employed as a proxy to predict and understand changes in disease states. Here, we developed In Silico Treatment, a computational method designed to overlay the gene expression changes observed in animals onto humans, quantifying the change in human disease status. We applied this method to mouse models for idiopathic pulmonary fibrosis and non-alcoholic steatohepatitis, two severe fibrotic diseases. We successfully identified known features of the disease models and provide a granular gene-level rationale behind our predictions. Consequently, our method shows promise as an effective approach to improve animal model selection and thus clinical translation.

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“…Interstitial lung disease (ILD) refers to a group of chronic lung disorders (1) characterized by inflammation and fibrosis of the lung interstitium (2,3) including idiopathic pulmonary fibrosis (IPF), sarcoidosis, hypersensitivity pneumonitis, and connective tissue disease associated ILD (CTD-ILD) (4). Symptoms vary, but commonly include breathlessness, cough, and fatigue (5)(6)(7)(8), and these symptoms can significantly decrease quality of life (QoL) for individuals with ILD.…”

Section: Introductionmentioning

confidence: 99%

The impact of air pollution on interstitial lung disease: a systematic review and meta-analysis

Lan,

Fermoyle,

Troy

et al. 2024

Front. Med.

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IntroductionThere is a growing body of evidence suggesting a causal relationship between interstitial lung disease (ILD) and air pollution, both for the development of the disease, and driving disease progression. We aim to provide a comprehensive literature review of the association between air pollution, and ILD, including idiopathic pulmonary fibrosis (IPF).MethodsWe systematically searched from six online database. Two independent authors (DL and CF) selected studies and critically appraised the risk of bias using the Newcastle-Ottawa Scale (NOS). Findings are presented through a narrative synthesis and meta-analysis. Meta-analyses were performed exclusively when there was a minimum of three studies examining identical pollutant-health outcome pairs, all evaluating equivalent increments in pollutant concentration, using a random effects model.Results24 observational studies conducted in 13 countries or regions were identified. Pollutants under investigation encompassed ozone (O3), nitrogen dioxide (NO2), Particulate matter with diameters of 10 micrometers or less (PM10) and 2.5 micrometers or less (PM2.5), sulfur dioxide (SO2), carbon monoxide (CO), nitric oxide (NO) and nitrogen oxides (NOx). We conducted meta-analyses to assess the estimated Risk Ratios (RRs) for acute exacerbations (AE)-IPF in relation to exposure to every 10 μg/m3 increment in air pollutant concentrations, including O3, NO2, PM10, and PM2.5. The meta-analysis revealed a significant association between the increased risk of AE-IPF in PM2.5, yielding RR 1.94 (95% CI 1.30–2.90; p = 0.001). Findings across all the included studies suggest that increased exposure to air pollutants may be linked to a range of health issues in individuals with ILDs.ConclusionA scarcity of available studies on the air pollutants and ILD relationship underscores the imperative for further comprehensive research in this domain. The available data suggest that reducing levels of PM2.5 in the atmosphere could potentially reduce AE frequency and severity in ILD patients.

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Contemporary Concise Review 2018: Interstitial lung disease

Cited by 7 publications

References 100 publications

Necrostatin-1 Alleviates Bleomycin-Induced Pulmonary Fibrosis and Extracellular Matrix Expression in Interstitial Pulmonary Fibrosis

Necrostatin-1 Alleviates Bleomycin-Induced Pulmonary Fibrosis and Extracellular Matrix Expression in Interstitial Pulmonary Fibrosis

In Silico Treatment: a computational framework for animal model selection and drug assessment

The impact of air pollution on interstitial lung disease: a systematic review and meta-analysis

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