691 results match your criteria Collagen-Vascular Disease Associated With Interstitial Lung Disease


Increased susceptibility to organic dust exposure-induced inflammatory lung disease with enhanced rheumatoid arthritis-associated autoantigen expression in HLA-DR4 transgenic mice.

Respir Res 2022 Jun 18;23(1):160. Epub 2022 Jun 18.

Department of Medicine, School of Medicine, University of Pittsburgh, Pittsburgh, PA, USA.

Immunogenetic as well as environmental and occupational exposures have been linked to the development of rheumatoid arthritis (RA), RA-associated lung disease, and other primary lung disorders. Importantly, various inhalants can trigger post-translational protein modifications, resulting in lung autoantigen expression capable of stimulating pro-inflammatory and/or pro-fibrotic immune responses. To further elucidate gene-environment interactions contributing to pathologic lung inflammation, we exploited an established model of organic dust extract (ODE) exposure with and without collagen-induced arthritis (CIA) in C57BL/6 wild type (WT) versus HLA-DR4 transgenic mice. Read More

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Systematic analyses identify the anti-fibrotic role of lncRNA TP53TG1 in IPF.

Cell Death Dis 2022 Jun 4;13(6):525. Epub 2022 Jun 4.

Zhuhai People's Hospital, Guangdong Provincial Key Laboratory of Tumor Interventional Diagnosis and Treatment, Zhuhai Hospital Affiliated With Jinan University, Jinan University, Zhuhai, 519000, Guangdong, China.

Long non-coding RNA (lncRNA) was reported to be a critical regulator of cellular homeostasis, but poorly understood in idiopathic pulmonary fibrosis (IPF). Here, we systematically identified a crucial lncRNA, p53-induced long non-coding RNA TP53 target 1 (TP53TG1), which was the dysregulated hub gene in IPF regulatory network and one of the top degree genes and down-regulated in IPF-drived fibroblasts. Functional experiments revealed that overexpression of TP53TG1 attenuated the increased expression of fibronectin 1 (Fn1), Collagen 1α1, Collagen 3α1, ACTA2 mRNA, Fn1, and Collagen I protein level, excessive fibroblasts proliferation, migration and differentiation induced by TGF-β1 in MRC-5 as well as PMLFs. Read More

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A drug-responsive multicellular human spheroid model to recapitulate drug-induced pulmonary fibrosis.

Biomed Mater 2022 06 9;17(4). Epub 2022 Jun 9.

Department of Bioengineering, Faculty of Engineering, Ege University, 35100 Izmir, Turkey.

Associated with a high mortality rate, pulmonary fibrosis (PF) is the end stage of several interstitial lung diseases. Although many factors are linked to PF progression, initiation of the fibrotic process remains to be studied. Current research focused on generating new strategies to gain a better understanding of the underlying disease mechanism as the animal models remain insufficient to reflect human physiology. Read More

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Collagen 3D matrices as a model for the study of cell behavior in pulmonary fibrosis.

Exp Lung Res 2022 04 20;48(3):126-136. Epub 2022 May 20.

National Consortium of Research in Respiratory Diseases, CIBERES Instituto de Salud Carlos III, Madrid, Spain.

Idiopathic pulmonary fibrosis (IPF) is a complex progressive chronic lung disease where epithelial to mesenchymal interaction, extracellular matrix (ECM) contact, and pro-fibrotic cytokines dynamics take part in the development of the disease. The study of IPF in the widespread two-dimensional (2 D) culture fails to explain the interaction of cells with the changing environment that occurs in fibrotic lung tissue. A three-dimensional (3 D) co-culture model might shed light on the pathogenesis of IPF by mimicking the fibrotic environment. Read More

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Pemafibrate attenuates pulmonary fibrosis by inhibiting myofibroblast differentiation.

Int Immunopharmacol 2022 Jul 6;108:108728. Epub 2022 Apr 6.

Department of Pulmonary and Critical Care Medicine, Shenzhen Institute of Respiratory Diseases, The Second Clinical Medical College, Jinan University (Shenzhen People's Hospital), The First Affiliated Hospital of Southern University of Science and Technology (Shenzhen People's Hospital), Shenzhen, China. Electronic address:

Background And Objective: Idiopathic pulmonary fibrosis is a chronic progressive disease associated with substantial morbidity and mortality despite advances in medical therapy. Increasing evidence suggests that peroxisome proliferator-activated receptors (PPARs) play important roles in the fibrosis-related diseases and their agonists may become effective therapeutic targets. Pemafibrate is a selective PPARα agonist, but the efficacy against pulmonary fibrosis and mechanisms involved have not been systematically evaluated. Read More

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Current Treatment of Systemic Sclerosis-Associated Interstitial Lung Disease

Tuberk Toraks 2022 03;70(1):85-92

University of Health Sciences, Yedikule Chest Disease and Chest Surgery Training and Research Hospital, Department of Chest Disease, İstanbul, Turkey.

Systemic sclerosis (SSc) is a connective tissue disease characterized by immune irregularity, vasculopathy and excessive collagen production, which causes skin and internal organ fibrosis. Although SSc often affects multiple organs and/or systems, lung involvement and especially interstitial lung disease (ILD) are the leading cause of death in this disease. The fact that different treatment options have been raised in recent years has given hope to clinicians in the management of this disease. Read More

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Pirfenidone exacerbates Th2-driven vasculopathy in a mouse model of SSc-ILD.

Eur Respir J 2022 Mar 24. Epub 2022 Mar 24.

Ludwig Boltzmann Institute for Lung Vascular Research Graz, Graz, Austria

Systemic sclerosis (SSc) is an autoimmune disease characterised by severe vasculopathy and fibrosis of various organs including the lung. Targeted treatment options for SSc-associated interstitial lung disease (SSc-ILD) are scarce. Here we assessed the effects of pirfenidone in a mouse model of SSc-ILD. Read More

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Arsenic trioxide inhibits the functions of lung fibroblasts derived from patients with idiopathic pulmonary fibrosis.

Toxicol Appl Pharmacol 2022 04 9;441:115972. Epub 2022 Mar 9.

Univ Rennes, CHU Rennes, Inserm, EHESP, Irset (Institut de Recherche en Santé, Environnement et Travail), UMR_S 1085, F-35000 Rennes, France.

Idiopathic pulmonary fibrosis (IPF) is a chronic and fatal interstitial lung disease. Currently, no treatment can block or reverse the development of lung fibrosis in patients suffering from IPF. Recent studies indicate that arsenic trioxide (ATO), a safe, effective anti-cancer pro-oxidant drug, prevents the differentiation of normal human lung fibroblasts (NHLFs) in vitro and reduces experimental pulmonary fibrosis in vivo. Read More

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Transient Agarose Spot (TAS) Assay: A New Method to Investigate Cell Migration.

Int J Mol Sci 2022 Feb 14;23(4). Epub 2022 Feb 14.

1st Department of Pediatrics, Semmelweis University, 1083 Budapest, Hungary.

Fibroblasts play a central role in diseases associated with excessive deposition of extracellular matrix (ECM), including idiopathic pulmonary fibrosis. Investigation of different properties of fibroblasts, such as migration, proliferation, and collagen-rich ECM production is unavoidable both in basic research and in the development of antifibrotic drugs. In the present study we developed a cost-effective, 96-well plate-based method to examine the migration of fibroblasts, as an alternative approach to the gold standard scratch assay, which has numerous limitations. Read More

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February 2022

Possible implication of intermolecular epitope spreading in the production of anti-glomerular basement membrane antibody in anti-neutrophil cytoplasmic antibody-associated vasculitis.

Clin Exp Rheumatol 2022 May 4;40(4):691-704. Epub 2022 Feb 4.

Department of Medical Laboratory Science, Faculty of Health Sciences, Hokkaido University, Sapporo, Japan.

Objectives: Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is sometimes complicated by anti-glomerular basement membrane (GBM) disease. Proteases, including elastase, released from neutrophils activated by ANCA are implicated in the pathogenesis of AAV. Epitopes of anti-GBM antibody exist in the α3-subunit non-collagenous (NC1) domain of collagen type IV [Col (IV)]. Read More

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Inhibition of CHIT1 as a novel therapeutic approach in idiopathic pulmonary fibrosis.

Eur J Pharmacol 2022 Mar 2;919:174792. Epub 2022 Feb 2.

OncoArendi Therapeutics SA, 02-089, Warsaw, Poland.

Idiopathic pulmonary fibrosis (IPF) is a progressive and eventually fatal lung disease with a complex etiology. Approved drugs, nintedanib and pirfenidone, modify disease progression, but IPF remains incurable and there is an urgent need for new therapies. We identified chitotriosidase (CHIT1) as new driver of fibrosis in IPF and a novel therapeutic target. Read More

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Mechanism of SMND-309 against lung injury induced by chronic intermittent hypoxia.

Int Immunopharmacol 2022 Apr 1;105:108576. Epub 2022 Feb 1.

Binzhou Medical University, 346 Guanhai Road, YanTai 264003, China. Electronic address:

Introduction: Obstructive sleep apnea-hypopnea syndrome (OSAHS) is a common sleep disorder that causes severe physiological disturbance. Evidence showed that OSAHS is an important associated comorbidity that can affect the survival of patients with pulmonary fibrosis. Until now, the potential mechanisms by which OSAHS accelerates the progression of lung fibrosis remain unclear. Read More

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ZC3H4 promotes pulmonary fibrosis via an ER stress-related positive feedback loop.

Toxicol Appl Pharmacol 2022 01 1;435:115856. Epub 2022 Jan 1.

Department of Physiology, School of Medicine, Southeast University, Nanjing, Jiangsu 2100096, China; Jiangsu Provincial Key Laboratory of Critical Care Medicine, Zhongda Hospital, School of Medicine, Southeast University, Nanjing, Jiangsu 210009, China; Key Laboratory of Development Genes and Human Disease, Southeast University, Nanjing, Jiangsu 2100096, China; Key Laboratory of Environmental Medicine Engineering, Ministry of Education, School of Public Health, Southeast University, Nanjing, Jiangsu 210009, China; School of Medicine, Xizang Minzu University, Xianyang, Shanxi 712082, China. Electronic address:

Background: Pulmonary fibrosis is a sequela of many pulmonary diseases, such as pneumoconiosis and idiopathic pulmonary fibrosis. The principal characteristics of pulmonary fibrosis comprise myofibroblast proliferation, alveolar damage and deposition of extracellular matrix components, which cause abnormal lung structure remodeling and an irreversible decline in lung function; however, the detailed mechanisms remain unclear. The current study focused on the role of ZC3H4, a new member of the zinc finger protein family, in SiO-induced pulmonary fibrosis. Read More

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January 2022

Modulation of H4K16Ac levels reduces pro-fibrotic gene expression and mitigates lung fibrosis in aged mice.

Theranostics 2022 1;12(2):530-541. Epub 2022 Jan 1.

Division of Pulmonary, Allergy, and Critical Care Medicine, Department of Medicine, University of Alabama at Birmingham, Birmingham, AL 35294, USA.

Histone H4 lysine16 acetylation (H4K16Ac) modulates chromatin structure by serving as a switch from a repressive to a transcriptionally active state. This euchromatin mark is associated with active transcription. In this study, we investigated the effects of H4K16Ac on the expression of pro-fibrotic genes in lung fibroblasts from patients with idiopathic pulmonary fibrosis (IPF) and in an aging murine model of lung fibrosis. Read More

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5‑Aminosalicylic acid attenuates paraquat‑induced lung fibroblast activation and pulmonary fibrosis of rats.

Mol Med Rep 2022 02 16;25(2). Epub 2021 Dec 16.

Department of Pathology, The Second Hospital, Hebei Medical University, Shijiazhuang, Hebei 050000, P.R. China.

Pulmonary fibrosis is one of the most important pathological processes associated with paraquat (PQ) poisoning. 5‑Aminosalicylic acid (5‑ASA) has been shown to be a promising agent against fibrotic diseases. In the present study, the alleviating role of 5‑ASA was evaluated in a rat model of pulmonary fibrosis induced by PQ intragastric poisoning (80 mg/kg). Read More

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February 2022

Chest computed tomography findings of adult patients with antimelanoma differentiation-associated protein 5 antibody-positive interstitial lung disease.

Mod Rheumatol 2022 Feb;32(2):365-372

Third Department of Internal Medicine, Faculty of Medical Sciences, University of Fukui, Fukui, Japan.

Objectives: Interstitial lung disease (ILD) associated with the antimelanoma differentiation-associated protein 5 (anti-MDA5) antibody is a rapidly progressive disease that requires timely, aggressive treatment. However, prompt diagnosis is difficult due to the longer time required for antibody detection. This study described the computed tomography (CT) findings of anti-MDA5 antibody-positive ILD (anti-MDA5-ILD). Read More

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February 2022

Collagen 1a1 Expression by Airway Macrophages Increases In Fibrotic ILDs and Is Associated With FVC Decline and Increased Mortality.

Front Immunol 2021 17;12:645548. Epub 2021 Nov 17.

Laboratory of Molecular and Cellular Pneumonology, Department of Respiratory Medicine, School of Medicine, University of Crete, Heraklion, Greece.

Within the Interstitial Lung Diseases (ILD), patients with idiopathic pulmonary fibrosis (IPF) and a subset of those with non-IPF fibrotic ILD have a distinct clinical phenotype of progression despite management. This group of patients has been collectively termed the progressive fibrotic phenotype (PFP). Their early recognition may facilitate access to antifibrotic therapies to prevent or slow progression. Read More

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December 2021

The efficacy and safety of pinocembrin in a sheep model of bleomycin-induced pulmonary fibrosis.

PLoS One 2021 2;16(12):e0260719. Epub 2021 Dec 2.

Melbourne Veterinary School, Faculty of Veterinary and Agricultural Sciences, University of Melbourne, Parkville, Victoria, Australia.

The primary flavonoid, pinocembrin, is thought to have a variety of medical uses which relate to its reported anti-oxidant, anti-inflammatory, anti-microbial and anti-cancer properties. Some studies have reported that this flavonoid has anti-fibrotic activities. In this study, we investigated whether pinocembrin would impede fibrosis, dampen inflammation and improve lung function in a large animal model of pulmonary fibrosis. Read More

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January 2022

Longitudinal serological assessment of type VI collagen turnover is related to progression in a real-world cohort of idiopathic pulmonary fibrosis.

BMC Pulm Med 2021 Nov 23;21(1):382. Epub 2021 Nov 23.

Department of Respiratory Medicine, Herlev and Gentofte University Hospital, Copenhagen, Denmark.

Background: Remodeling of the extracellular matrix (ECM) is a central mechanism in the progression of idiopathic pulmonary fibrosis (IPF), and remodeling of type VI collagen has been suggested to be associated with disease progression. Biomarkers that reflect and predict the progression of IPF would provide valuable information for clinicians when treating IPF patients.

Methods: Two serological biomarkers reflecting formation (PRO-C6) and degradation (C6M) of type VI collagen were evaluated in a real-world cohort of 178 newly diagnoses IPF patients. Read More

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November 2021

Pulmonary fibrosis from molecular mechanisms to therapeutic interventions: lessons from post-COVID-19 patients.

Biochem Pharmacol 2021 11 21;193:114812. Epub 2021 Oct 21.

Department of Pharmacy, University of Pisa, Via Bonanno 6, Pisa 56126, Italy. Electronic address:

Pulmonary fibrosis (PF) is characterised by several grades of chronic inflammation and collagen deposition in the interalveolar space and is a hallmark of interstitial lung diseases (ILDs). Recently, infectious agents have emerged as driving causes for PF development; however, the role of viral/bacterial infections in the initiation and propagation of PF is still debated. In this context, the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), the virus responsible for the current coronavirus disease 2019 (COVID-19) pandemic, has been associated with acute respiratory distress syndrome (ARDS) and PF development. Read More

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November 2021

Deficiency of CARMA3 attenuates the development of bleomycin induced pulmonary fibrosis.

Biochem Biophys Res Commun 2021 12 11;581:81-88. Epub 2021 Oct 11.

Department of Thoracic and Cardiovascular Surgery, Nanjing First Hospital, Nanjing Medical University, Nanjing, China. Electronic address:

Background: Pulmonary fibrosis (PF) has attracted more and more attention due to its irreversibility and high mortality rate. Currently, there is no effective treatment option is available to reverse the disease. Caspase recruitment domain-containing membrane-associated guanylate kinase protein (CARMA3) has been recognized as a proinflammatory molecule involved in many lung diseases, such as Allergic airway inflammation and lung cancer. Read More

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December 2021

IL-23 amplifies the epithelial-mesenchymal transition of mechanically conditioned alveolar epithelial cells in rheumatoid arthritis-associated interstitial lung disease through mTOR/S6 signaling.

Am J Physiol Lung Cell Mol Physiol 2021 12 29;321(6):L1006-L1022. Epub 2021 Sep 29.

Division of Pulmonary and Critical Care Medicine, Mayo Clinic College of Medicine and Science, Rochester, Minnesota.

Epithelial-mesenchymal transition (EMT) creates an environment facilitating fibrosis following alveolar epithelial cell injury. IL-23 has important roles in chronic autoimmune conditions like rheumatoid arthritis (RA), but its role in the interstitial lung disease that affects patients with RA is unclear. This study aimed to determine the profibrogenic role of IL-23 on somatic alveolar type I (ATI) epithelial cells. Read More

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December 2021

SSC-ILD mouse model induced by osmotic minipump delivered bleomycin: effect of Nintedanib.

Sci Rep 2021 09 16;11(1):18513. Epub 2021 Sep 16.

Pharmacology & Toxicology Department, Chiesi Farmaceutici S.p.A., Corporate Pre-Clinical R&D, Largo Belloli, 11/A, 43122, Parma, Italy.

Systemic sclerosis (SSc) is an autoimmune disease characterized by an excessive production and accumulation of collagen in the skin and internal organs often associated with interstitial lung disease (ILD). Its pathogenetic mechanisms are unknown and the lack of animal models mimicking the features of the human disease is creating a gap between the selection of anti-fibrotic drug candidates and effective therapies. In this work, we intended to pharmacologically validate a SSc-ILD model based on 1 week infusion of bleomycin (BLM) by osmotic minipumps in C57/BL6 mice, since it will serve as a tool for secondary drug screening. Read More

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September 2021

Use of Systemic Corticosteroids for Reasons Other than Asthma in Subjects with Asthma.

Respiration 2022 1;101(2):109-115. Epub 2021 Sep 1.

Department of Pulmonology, Yokohama City University Graduate School of Medicine, Yokohama, Japan.

Backgrounds: Recent studies have reported increased risks of adverse events from systemic corticosteroids even with only low-dose or short-term use. Some patients with asthma experience complications requiring systemic corticosteroids. However, few studies have examined issues associated with administration of systemic corticosteroids for reasons other than asthma among subjects with asthma. Read More

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Identification and Integrated Analysis of circRNA and miRNA of Radiation-Induced Lung Injury in a Mouse Model.

J Inflamm Res 2021 2;14:4421-4431. Epub 2021 Sep 2.

Department of Radiation Oncology, Fudan University Shanghai Cancer Center, Shanghai, People's Republic of China.

Background: Radiation-induced lung injury (RILI) is a main threat to patients who received thoracic radiotherapy. Thus, understanding the molecular mechanism of RILI is of great importance. Circular RNAs (circRNAs) have been found to act as a regulator of multiple biological processes, and the circRNA-microRNA (miRNA)-mRNA axis could play an important role in the signaling pathway of many human diseases including radiation injury. Read More

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September 2021

The impact of airborne endotoxin exposure on rheumatoid arthritis-related joint damage, autoantigen expression, autoimmunity, and lung disease.

Int Immunopharmacol 2021 Nov 27;100:108069. Epub 2021 Aug 27.

Department of Internal Medicine, College of Medicine, University of Nebraska Medical Center, Omaha, NE, USA. Electronic address:

Airborne biohazards are risk factors in the development and severity of rheumatoid arthritis (RA) and RA-associated lung disease, yet the mechanisms explaining this relationship remain unclear. Lipopolysaccharide (LPS, endotoxin) is a ubiquitous inflammatory agent in numerous environmental and occupational air pollutant settings recognized to induce airway inflammation. Combining repetitive LPS inhalation exposures with the collagen induced arthritis (CIA) model, DBA1/J mice were assigned to either: sham (saline injection/saline inhalation), CIA (CIA/saline), LPS (saline/LPS 100 ng inhalation), or CIA + LPS for 5 weeks. Read More

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November 2021

Evaluation on epithelial-mesenchymal state and microRNAs focusing on isolated alveolar epithelial cells from bleomycin injured rat lung.

Toxicology 2021 09 20;461:152903. Epub 2021 Aug 20.

Department of Pharmaceutics and Therapeutics, Graduate School of Biomedical and Health Sciences, Hiroshima University, 1-2-3 Kasumi, Minami-ku, Hiroshima 734-8553, Japan. Electronic address:

Several studies using bleomycin (BLM)-induced lung injury rat model revealed that epithelial-mesenchymal transition (EMT) contributes to pulmonary fibrosis. Conversely, microRNAs (miRNAs) are considered as useful markers of various diseases. In the present study, we aimed to characterize the EMT state through focusing on alveolar epithelial cells and identify the miRNAs that can be used as markers to predict pulmonary fibrosis using a BLM-induced lung injury rat model. Read More

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September 2021

Thymosin β4 Suppresses LPS-Induced Murine Lung Fibrosis by Attenuating Oxidative Injury and Alleviating Inflammation.

Inflammation 2022 Feb 19;45(1):59-73. Epub 2021 Aug 19.

Department of Ultrasound, The First Affiliated Hospital of Xi'an Jiaotong University, Xi'an, Shaanxi Province, China.

Inflammation plays a critical role in the progression of pulmonary fibrosis. Thymosin β4 (Tβ4) has antioxidant, anti-inflammatory, and antifibrotic effects. Although the potent protective role of Tβ4 in bleomycin-induced pulmonary fibrosis has been validated, the underlying mechanism is not clear; moreover, the influence of Tβ4 on lipopolysaccharide (LPS)-induced lung injury/fibrosis has not been reported. Read More

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February 2022

Percolation of collagen stress in a random network model of the alveolar wall.

Sci Rep 2021 08 17;11(1):16654. Epub 2021 Aug 17.

Depatment of Medicine, University of Vermont Larner College of Medicine, 149 Beaumont Ave, Burlington, VT, 05405, USA.

Fibrotic diseases are characterized by progressive and often irreversible scarring of connective tissue in various organs, leading to substantial changes in tissue mechanics largely as a result of alterations in collagen structure. This is particularly important in the lung because its bulk modulus is so critical to the volume changes that take place during breathing. Nevertheless, it remains unclear how fibrotic abnormalities in the mechanical properties of pulmonary connective tissue can be linked to the stiffening of its individual collagen fibers. Read More

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A novel humanized model of rheumatoid arthritis associated lung disease.

Clin Immunol 2021 09 29;230:108813. Epub 2021 Jul 29.

Department of Immunology, Department of Physiology and Biomedical Engineering, Mayo Clinic, Rochester, MN, United States of America; Division of Rheumatology, Department of Physiology and Biomedical Engineering, Mayo Clinic, Rochester, MN, United States of America. Electronic address:

Cigarette smoking has been implicated in the pathogenesis of seropositive rheumatoid arthritis (RA), as well as RA-associated lung disease. Fibrotic interstitial lung disease as well as emphysema occur in RA and cause substantial morbidity. We used arthritis-susceptible HLA-DQ8 transgenic mice to generate RA-associated lung disease. Read More

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September 2021