Fibrotic lung phenotype. Medical search. Frequent questions

In this setting, IPF is defined as a chronic progressive fibrotic ILD, with unknown aetiology and a pattern of usual interstitial pneumonia (UIP), which is represented by heterogeneous lesions at different stages of evolution with foci of proliferative fibroblasts. (ersjournals.com)
Lung fibrotic disorders are characterised by accumulation of fibroblasts, myofibroblasts and extracellular matrix (ECM), leading to chronic respiratory failure. (ersjournals.com)
In the lung, fibroblasts exist in ECM-rich interstitial spaces and are key effectors of lung fibrogenesis. (atsjournals.org)
The activation and accumulation of lung fibroblasts resulting in aberrant deposition of extracellular matrix components, is a pathogenic hallmark of Idiopathic Pulmonary Fibrosis, a lethal and incurable disease. (bvsalud.org)
Τhe profibrotic milieu is found to induce TKS5 expression and the formation of prominent podosome rosettes in lung fibroblasts, that are retained ex vivo, culminating in increased extracellular matrix invasion. (bvsalud.org)
As computationally predicted, inhibition of src kinase is shown to potently attenuate podosome formation in lung fibroblasts and extracellular matrix invasion, and bleomycin-induced pulmonary fibrosis, suggesting pharmacological targeting of podosomes as a very promising therapeutic option in pulmonary fibrosis. (bvsalud.org)
Reduced IGFBP\4 levels in SSc lung fibroblasts may contribute to the fibrotic phenotype via loss of IGFBP\4 antifibrotic activity. (museopedrogocial.org)
induced ECM production To assess the effect of IGFBP\4 on ECM production, we 1st tested its effects on untreated main human being adult lung fibroblasts. (museopedrogocial.org)
To further validate the effects of gain of function of IGFBP\4 on ECM reduction, we examined the effect of loss of function of IGFBP\4 in main human being lung fibroblasts. (museopedrogocial.org)
Treatment of principal individual lung fibroblasts with physiological concentrations from the profibrotic elements IGFBP\3 and IGFBP\5 didn't reduce IGFBP\4 appearance (data not really shown). (museopedrogocial.org)
Individual adult lung fibroblasts had been contaminated with replication\deficient adenovirus encoding control or IGFBP\4 adenovirus for 72?hours. (museopedrogocial.org)
B) Endogenous IGFBP\4 decreases the TGF\?Cinduced ECM proteins fibronectin, collagen, and tenascin\C in fetal adult and lung lung fibroblasts. (museopedrogocial.org)
MRC\5 cells and primary human adult lung fibroblasts were infected with replication\deficient adenovirus\expressing control or IGFBP\4 adenovirus for 24?hours and stimulated with 10?ng/mL TGF\?1 for yet another 48?hours. (museopedrogocial.org)
Principal individual adult lung fibroblasts had been treated with 10?ng/mL TGF\?1 as well as the indicated concentrations of rhIGFBP\4 for 72?hours. (museopedrogocial.org)
YT play a role in fibrotic fibroblasts, but their role is not yet known in the fibrotic lung epithelium. (lu.se)
The desmoplastic stroma of lung cancer and other solid tumors is rich in tumor-associated fibroblasts (TAFs) exhibiting an activated/myofibroblast-like phenotype. (ibecbarcelona.eu)
. Conversely, the same fibroblasts are induced to adopt a highly fibrotic phenotype in response to treatment with the pro-fibrotic growth factor TGF-β1. (musculoskeletalresearch.com)
Activated fibroblasts that are located in the lining and sublining of the synovium contribute to pannus formation and bone destruction in rheumatoid arthritis, whereas fibroblasts in the lung tissue react to stimulation by an excessive release of extracellular matrix, resulting in progressive tissue fibrosis. (snmjournals.org)
In contrast to rheumatoid arthritis, which is characterized by a strong inflammatory component, in SSC interstitial lung disease the activation of fibroblasts leads to excessive fibrosis of the lungs. (snmjournals.org)
Because activated fibroblasts in inflammatory conditions have similar characteristics as cancer-associated fibroblasts (CAFs) and CAFs contribute actively to the malignant phenotype, we believe that anti-fibrotic drugs have the potential to be repurposed as anti-cancer drugs. (biomedcentral.com)
CAFs and normal fibroblasts differ with respect to the expression of various markers, such as fibrotic markers, growth factors, chemokines and cytokines ( 9 ). (spandidos-publications.com)
Instead, recent findings suggest that alveolar epithelial type II (ATII) cells undergoing EMT promote a pro-fibrotic microenvironment through paracrine signalling activating local fibroblasts. (lungdiseasesjournal.com)
Provided their pivotal purpose from the generation of the extracel lular matrix, fibroblasts and myofibroblasts are considered the primary effector cells during the evolution of pulmonary fibrosis, selleck chemical and their cellular source is hence a vital query from the pathogenesis of fibrotic lung disorders. (cret-signal.com)
At present, 3 hypotheses tackle the origin of lung fibroblasts, The classical notion is tissue injury induces the activation, proliferation and differentiation of the resident fibroblast during the lung interstitial compartment into a myo fibroblast that migrates in to the alveolar compartment and expresses constituents on the extracellular matrix major to lung fibrosis. (cret-signal.com)
The third mechanism pertains to a cir culating bone marrow derived progenitor cell, the fibro cyte, which will dwelling to web sites of lung damage, differentiate into fibroblasts and myofibroblasts, proliferate, and contri bute on the generation of extracellular matrix. (cret-signal.com)

We used data from the INBUILD and INPULSIS trials to investigate the natural history of progressive fibrosing interstitial lung diseases (ILDs). (ersjournals.com)
They are involved in asthma, chronic obstructive pulmonary diseases (COPD), pulmonary fibrosis, and lung cancer. (wikipedia.org)
The presence of these dirty macrophages has been a characteristic of many smoke-related lung diseases. (wikipedia.org)
The interstitial lung diseases (ILDs) are a group of diffuse parenchymal lung disorders that are classified according to specific clinical, radiological and histopathological features. (ersjournals.com)
SignificanceTissue fibrotic diseases, for example of the liver and lung, represent a huge unmet medical need. (ox.ac.uk)
This article contains a selection of scientific highlights in the field of interstitial lung diseases (ILDs) presented at the hybrid European Respiratory Society International Congress 2022. (bvsalud.org)
Maladaptive epithelial repair from chronic injury is a common feature in fibrotic diseases, which in turn activates a pathogenic fibroblast response that produces excessive matrix deposition. (escholarship.org)
He also has an expertise in interstitial lung disease, occupational lung disease, nontuberculous mycobacterial pneumonia and diseases of the large and small airways. (uchicago.edu)
Through his research, Dr. Chung is studying how imaging can play a more significant role in patients with chronic lung diseases, specifically, interstitial lung disease, pulmonary fibrosis, occupational lung disease and nontuberculous mycobacterial pneumonia. (uchicago.edu)
This review will outline the role and characterization of pro-fibrotic macrophages in fibrotic lung diseases and discuss the possibility of treating lung fibrosis by preventing or reprogramming the polarity of macrophages. (novartis.com)
Numerous research works in Immunology, particularly in pathogenesis and treatment of interstitial lung diseases, particularly idiopathic pulmonary fibrosis and around other Diffuse Parenchymal Lung Diseases. (wasog2022.ro)
He further specialised in interstitial lung diseases at the Royal Brompton Hospital in London, UK. (wasog2022.ro)
Prof Wuyts is an ILD specialist and head of the unit for interstitial lung diseases in university hospitals Leuven, the largest referral centre for ILD in Belgium. (wasog2022.ro)
Furthermore the architectural ultra characterisation using micro CT scanning and transcriptomics in IPF and other fibrotic interstitial lung diseases. (wasog2022.ro)
She is chair of the ERS Group 12.01 (idiopathic interstitial lung diseases ), is a member of the scientific advisory boards of a number of different patient associations including EU-IPFF, and is associate editor of the European Respiratory Journal. (wasog2022.ro)
Despite the overall low incidence of fibrotic diseases, fibrotic tissue responses across different diseases have been estimated to account for up to 45% of deaths in high-income countries, causing socioeconomic costs of tens of billions of U.S. dollars per year ( 2 ). (snmjournals.org)
He is particularly interested in lung diseases leading to fibrosis, including those from exposures to foreign materials. (yalemedicine.org)
My training as a physician scientist motivates me to seek new treatments for chronic lung diseases. (yale.edu)
Interstitial lung diseases (ILDs) comprise an heterogenous group of more than 200 pulmonary disorders characterized by lung architectural distortion, with variable amount of fibrotic and/or inflammatory lesions, and associated with significant morbidity and mortality. (pneumon.org)
Recently, the concept of progressive pulmonary fibrosis (PPF) has been coined to describe fibrotic lung diseases other than IPF that develop, within a period of 1 year, a progressive disease behavior. (pneumon.org)
Interstitial lung diseases (ILDs) comprise an heterogenous group of more than 200 lung disorders characterized by variable amount of fibrotic and/or inflammatory lesions 1 . (pneumon.org)

The present authors contend that highlighting subtle histological differences between UIP and NSIP and using these to define separate clinical entities is incorrect and is hindering attempts to better understand the pathogenesis of fibrotic lung disease. (ersjournals.com)

Here, we identified miR-323a-3p to be downregulated in the epithelium of lungs with bronchiolitis obliterans syndrome (BOS) after lung transplantation, idiopathic pulmonary fibrosis (IPF), and murine bleomycin-induced fibrosis. (escholarship.org)
Given that transplantation is the only therapy for thousands of patients with end-stage lung disease, the repair and prevention of organ failure is a critical area of research. (rotrf.org)
Lung transplantation provides a realistic hope of improved survival and enhanced quality of life. (ersjournals.com)
As the science of lung transplantation approaches its fifth decade, we are only now reaching a critical mass of clinicians and scientific researchers to enable adequately powered studies to assist in informing our approach to some of these controversies. (ersjournals.com)
Lung transplantation remains an art, combining experience with evidence. (ersjournals.com)
To illustrate the spectrum of controversial areas in lung transplantation including whom to list, which organs can be used and for whom, immune suppression and infection prophylaxis, and causes and phenotypes of chronic lung allograft dysfunction. (ersjournals.com)
One overriding achievement of the International Society for Heart and Lung Transplantation (ISHLT) since its inception over 30 years ago has been a commitment to promoting safe practice leading to the development of a number of consensus position papers. (ersjournals.com)
Despite this, when confronted with real-life situations in lung transplantation (LTX), we may find that two clinicians, both with great experience and wisdom, hold very different and equally strong views. (ersjournals.com)
Furthermore, referral to centers with expertise in pulmonary hypertension on lung transplantation may be required, depending on the individual clinical context. (medscape.com)
Lung transplantation may be an option for those with end-stage lung disease. (medscape.com)
Fibrosing ILDs are heterogeneous group of scarring disorders of lungs (mainly including idiopathic interstitial pneumonias) causing progressive loss of lung function, shortness of breath on minimal exertion, respiratory failure and in the absence of lung transplantation, premature death. (ifpoc.org)
Lung transplantation should be considered for patients with severe fibrotic pulmonary sarcoidosis, as mortality is high in these patients. (livingwithsarcoidosis.org)
CT-based Machine Learning for Donor Lung Screening Prior to Transplantation. (nih.gov)
Computed tomography-based machine learning for donor lung screening before transplantation. (nih.gov)

In this fellowship I will investigate the role of cellular immunity and in particular the macrophage in orchestrating the environment in the lung that determines the fibrotic response to injury. (europa.eu)
I will investigate whether specific macrophage phenotypes contribute to loss of function by driving aberrant epithelial repair and fibrotic remodeling in the lung. (europa.eu)
I hypothesis that manipulation of lung macrophage phenotype by treatment with azithromycin may limit fibrosis in the lung. (europa.eu)
Regardless of active or passive smokers, macrophage accumulation is found in the lungs. (wikipedia.org)
A macrophage can be polarized into the classic M1 or M2 phenotype, and this phenomenon can be seen in cigarette consumption. (wikipedia.org)
The reprogram of macrophage implies a dysregulated inflammation that can damage healthy lung cells. (wikipedia.org)
Although the pathophysiology of fibrotic pulmonary sarcoidosis remains poorly understood, augmented transforming growth factor-β activity, macrophage phenotype switching, and a Th1 to Th2 transition may be important features (11-14). (livingwithsarcoidosis.org)
Moreover, the gene expression profile of inflammatory modulators such as IL10, IL6, and TNF-α was also investigated to verify the macrophage phenotype induced by treatment. (mdpi.com)

Extracellular matrix (ECM) is a dynamic tissue that contributes to organ integrity and function, and its regulation of cell phenotype is a major aspect of cell biology. (atsjournals.org)
Extracellular matrix is important in driving cellular phenotypic responses in fibrotic and other lung disorders. (atsjournals.org)
We define a new method to prepare acellular lung matrix that can be used to study the impact of extracellular matrix on lung cell phenotype. (atsjournals.org)
IPF is characterized by damaged distal lung epithelium with excessive tissue scarring and extracellular matrix remodeling. (lu.se)
Interestingly, we found epithelial YT signaling to be actively involved in extracellular matrix remodeling in the fibrotic lung epithelium through modulation of lysyl oxidase expression, a collagen crosslinking enzyme. (lu.se)
Transcriptional Signatures of IPF Lungs Are Enriched with Developmental Genes Microarray analysis recognized an IPF-specific gene expression signature characterized by the up-regulation of genes indicative of an active tissue remodeling program, including extracellular matrix and a large number of myofibroblast/easy muscle 1009298-09-2 cellCassociated and epithelial cellCrelated genes [3,5]. (thetechnoant.info)
Although the mechanisms underlying such epigenetic reprogramming are incompletely understood, there is increasing evidence that they involve interactions with either cancer cells, pro-fibrotic cytokines such as TGF-β, the stiffening of the surrounding extracellular matrix, smoking cigarette particles and other environmental cues. (ibecbarcelona.eu)
Lungs are composed normally of extracellular collagen which allows the lungs to exert their breathing efforts. (wikidoc.org)
Pulmonary fibrosis (PF) is a chronic and fatal interstitial lung disease involving deposition of collagen and extracellular matrix (ECM) around the fibrotic foci, as well as honeycomb changes of the subpleural and basement membranes. (biomedcentral.com)

Along with these clinical similarities, progressive fibrosing ILDs appear to share pathobiological mechanisms that may represent a common fibrotic response to tissue injury [ 6 - 10 ]. (ersjournals.com)
In multiple organs, including the lungs, age-related tissue and organ dysfunction interferes with tissue regeneration, which requires functional stem cells. (karger.com)
Stem cells of all organs - including the lung, which harbors distinct stem cells for each separate tissue that makes up the lung as a whole - reside in niches described as a microenvironment that supports and maintains the 'stemness' of cells as a critical reservoir for maintaining tissue homeostasis and responding to injury [ 3 ]. (karger.com)
This trait is involved in anti-inflammation and tissue repair, but this can also be pro-fibrotic. (wikipedia.org)
Connective tissue disease, hypersensitivity pneumonitis, drug-induced lung disease, resolved acute respiratory distress syndrome and some cases of cryptogenic organising pneumonia may all produce a histological picture of NSIP 1 , 3 . (ersjournals.com)
We report that the decellularization process effectively removes cellular and nuclear material while retaining native dimensionality and stiffness of lung tissue. (atsjournals.org)
Factors such as younger age, female gender, and the presence of connective tissue disease lean toward an inflammatory phenotype. (medscape.com)
In this study, using single-cell RNA sequencing, cytometry by time of flight (CyTOF), tissue imaging, and functional assays, we identify a complex vascular niche in Dupuytren's disease (DD), a common localized fibrotic condition of the palm, where early-disease-stage tissue can be accessed readily. (ox.ac.uk)
In this report, increased expression of TKS5, a scaffold protein essential for the formation of podosomes, was detected in the lung tissue of Idiopathic Pulmonary Fibrosis patients and bleomycin-treated mice. (bvsalud.org)
Key research interests include prognostic and outcome biomarkers in lung fibrosis across ILD entities, including those associated with connective tissue disease. (wasog2022.ro)
In patients with suspected connective tissue disease (CTD)-associated interstitial lung disease (ILD) (CTD-ILD), referral to a center with expertise in management of CTD-ILD is recommended. (medscape.com)
As mentioned previously, immunosuppression is the cornerstone of the medical management of connective tissue disease (CTD)-associated interstitial lung disease (ILD) (CTD-ILD). (medscape.com)
However, an exuberant fibrotic response is pathologic and leads to frank tissue destruction evident on imaging or gross pathology. (livingwithsarcoidosis.org)
The observation that one inch of cancerous liver tissue can have more than 100 000 000 mutations and an identical mutation can result in different phenotypes, depending on the environment surrounding that mutation, makes it very unlikely that mutations by themselves are causative of most cancers. (4open-sciences.org)
Normally, collagen is degraded and produced regularly to preserve the normal lung tissue. (wikidoc.org)
However, immunopathogenic responses of lung tissue are quite similar. (wikidoc.org)
Alveolar architecture is destroyed and healthy tissue is replaced by altered extra cellular matrix (ECM), with progressive dyspnoea and impairment of lung function ultimately leading to death 1,2 . (lungdiseasesjournal.com)

Current guidelines recommend nintedanib for PPF treatment for patients who have failed standard management for fibrotic interstitial lung disease (ILD) other than IPF. (medscape.com)
The use of antifibrotics may decelerate PPF worsening, similar to their beneficial effects on IPF, and treatment with nintedanib is suggested in patients with PFF, when standard management for fibrotic ILD has failed. (pneumon.org)

The early phase response was characterized by rapid infiltration of neutrophils and macrophages, elevated levels of proinflammatory cytokines in the BAL lavage fluid and lung parenchyma, and increased deposition of collagen fibers in the interstitial space. (cdc.gov)
In diseased conditions due to long-term smoking, the inactivation of JNK reduces the levels of reactive nitrogen species and pro-inflammatory cytokines with more M2-like phenotype. (wikipedia.org)
We developed a model of the aberrant epithelial reprogramming observed in IPF using alveolar organoids derived from human-induced pluripotent stem cells stimulated with a cocktail of pro-fibrotic and inflammatory cytokines. (lu.se)
We further hypothesize that a unique combination of cytokines and chemokines is responsible for progenitor cell recruitment to damaged lungs. (rotrf.org)
Via chemokine signaling, these lymphocytes are recruited to the initial site of inflammation and, polarized to a Th1 phenotype, release cytokines, including IFN-γ, which drive the activation and organization of macrophages. (livingwithsarcoidosis.org)

ILD includes a spectrum of clinical phenotypes. (springer.com)
Patients will be analyzed according to the clinical phenotypes of cardiac and fibrotic pulmonary sarcoidosis. (figshare.com)

While much work has focused on the failure of epithelial cell populations as a key component of the aging process, additional studies have shown that aging, as a global phenomenon in the lung, also impacts resident endothelial, mesenchymal, and immune cell populations. (karger.com)
Epithelial-mesenchymal transition (EMT) is a process where polarised epithelial cells undergo molecular changes allowing them to gain a mesenchymal phenotype, with a subsequent enhanced ability to produce ECM components and increased migration and/or invasion. (lungdiseasesjournal.com)
The 2nd mechanism involves injury induced changes within the microenvironment of your epithe lium or endothelium, inducing their transition to a mesenchymal phenotype and subsequently contributes to fibroproliferation. (cret-signal.com)

The down-regulation of let-7d in IPF and the profibrotic effects of this down-regulation in vitro and in vivo suggest a key regulatory role for this microRNA in preventing lung fibrosis. (nih.gov)
Thus, M2-like macrophages in vitro and pro-fibrotic macrophages in vivo are not completely identical cell populations. (novartis.com)
Targeting YT in vivo using an FDA approved drug ameliorated the fibrotic phenotype, indicating that YT targeting may be an option to treat fibrosis. (lu.se)

To better address how ECM influences fibroblast phenotype in a disease-specific manner, we developed a culture system using acellular human normal and fibrotic lungs. (atsjournals.org)

The study demonstrates MWCNT exposed mice offer a unique model for studying lung fibrosis and granuloma formation induced by engineered nanomaterial s. (cdc.gov)

Anti-fibrotic drugs such as pirfenidone have been developed for the treatment of idiopathic pulmonary fibrosis. (biomedcentral.com)

Interstitial lung disease (ILD) encompasses a heterogeneous group of respiratory disorders characterised by inflammation and/or fibrosis of the lung interstitium. (springer.com)
Clinical assessments of disease activity in immune-mediated rheumatologic disorders such as rheumatoid arthritis or systemic sclerosis (SSC)-associated interstitial lung disease usually include physical examinations and evaluation of functional parameters, as well as the patient's self-reporting of disease activity and quality of life ( 5 ). (snmjournals.org)
Interstitial lung disease is a group of disorders that involve pulmonary parenchyma . (wikidoc.org)

Chronic lung disease is a very common cause of morbidity and mortality across Europe. (europa.eu)
We will also examine how regenerative failure may contribute to development of aging-related, chronic lung disease. (karger.com)
Chronic lung allograft dysfunction pathogenesis deserves further detailed study. (ersjournals.com)

The aforementioned lead to lung parenchymal destruction and potentially permanent functional impairment 4 . (pneumon.org)
A chest x-ray showed considerable fibrotic interstitial changes associated with patchy parenchymal shadowing in both lower fields and multiple thin-walled cavitary lesions (0.7-3.5 cm) in both upper lobes. (cdc.gov)

We first developed a method to simultaneously isolate proximal and distal lung progenitor cells from an individual mouse with the aid of a 3D printed surgical guide and found that the precision of dissecting the lung lobes affects the purity of the isolated distal progenitors and how they behave in organoid assays. (lu.se)
Progenitor cells derived from bone marrow have been shown to respond following lung injury in both mice and humans. (rotrf.org)

In addition to IPF, there are a number of other ILDs that may develop a progressive fibrosing phenotype characterised by declining lung function, an increasing extent of fibrosis on high-resolution computed tomography (HRCT), worsening symptoms and quality of life, and early mortality [ 2 - 5 ]. (ersjournals.com)
Additionally, genetics plays a role, with shorter telomeres indicating a more fibrotic phenotype and an increased risk of immunomodulatory treatment side effects in non-IPF ILDs. (medscape.com)

Thereafter, the lesions gradually became a chronic fibrotic phenotype with significant interstitial fibrosis and formation of granulomas. (cdc.gov)

However, available therapies do not specifically target the epithelium and human models of fibrotic epithelial damage with suitability for drug discovery are lacking. (lu.se)

In this review, we examine aging as a process dependent on specific changes in molecular pathways within multiple lung cell populations. (karger.com)
AILDR is an important clinical and research tool providing a platform for epidemiological data that will prove essential in promoting understanding of a rare cohort of lung disease and provide foundations for our aspiration to standardise investigation and treatment pathways of ILD across Australasia. (springer.com)
These studies demonstrate that miR-323a-3p has a central role in lung fibrosis that spans across murine and human disease, and downregulated expression by the lung epithelium releases inhibition of various profibrotic pathways to promote fibroproliferation. (escholarship.org)

In systemic sclerosis (SSc) patients, the change in the trend of scleroderma-related deaths over the past 30 years demonstrates that lung involvement (both pulmonary hypertension and pulmonary fibrosis) is among the primary causes of mortality [ 6 ]. (ersjournals.com)
Complications of fibrotic pulmonary sarcoidosis include pulmonary hypertension from capillary obliteration and chronic aspergillus disease, with hemoptysis a common and potentially life-threatening manifestation. (livingwithsarcoidosis.org)

Idiopathic pulmonary fibrosis (IPF) is, by definition, a progressive fibrosing interstitial lung disease (ILD) [ 1 ]. (ersjournals.com)
Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive, and usually lethal fibrotic lung disease characterized by profound changes in epithelial cell phenotype and fibroblast proliferation. (nih.gov)
Interstitial lung disease (ILD) encompasses a large and diverse group of pathological conditions that share similar clinical, radiological and pathological manifestations, despite potentially having quite different aetiologies and comorbidities. (ersjournals.com)
Idiopathic pulmonary fibrosis (IPF) represents probably the most aggressive form of ILD and systemic sclerosis is a multiorgan fibrotic disease frequently associated with ILD. (ersjournals.com)
This schematic view of the morpho-functional unit of the lung (alveolus) depicts the main differences in cellular composition in idiopathic pulmonary fibrosis (IPF) and systemic sclerosis-associated interstitial lung disease (SSc-ILD) compared with normal physiological cellular components. (ersjournals.com)
By using human lungs and cells, this new system provides a novel way to culture cells in vitro to investigate matrix contributions to lung pathology in a disease-specific manner. (atsjournals.org)
Interstitial Lung Disease (ILD) is a group of respiratory conditions affecting the lung interstitium often associated with progressive respiratory failure. (springer.com)
Pulmonary arterial hypertension (PAH) is a progressive disease characterized by lung endothelial cell dysfunction and vascular remodeling. (surrey.ac.uk)
A vasculature niche orchestrates stromal cell phenotype through PDGF signaling: Importance in human fibrotic disease. (ox.ac.uk)
Assessing the contribution of rare genetic variants to phenotypes of chronic obstructive pulmonary disease using whole-genome sequence data. (escholarship.org)
Genetic variation has a substantial contribution to chronic obstructive pulmonary disease (COPD) and lung function measurements. (escholarship.org)
Mice are also easy to breed with a short generation time and a short life span allowing direct study of disease development and can accurately eludicidate how genetic sequences may vary in different strains and result in susceptibility to fibrotic pathologies. (biomedcentral.com)
Control groups include non-infected patients with sarcoidosis and patients with non-sarcoidosis interstitial lung disease. (figshare.com)
Therapeutic strategies targeting pro-fibrotic macrophages in interstitial lung disease. (novartis.com)
Idiopathic pulmonary fibrosis (IPF) is the representative phenotype of interstitial lung disease where severe scarring develops in the lung interstitium. (novartis.com)
The current study aims to elucidate the validity of progenitor cell numbers and phenotype in predicting and monitoring BOS using the primary outcome of BOS status and further assessing covariate of disease. (rotrf.org)
Recently, we have reanalyzed released datasets [3 previously,5,6] using analytical strategies that enable global and impartial mapping from the useful designs that characterize IPF lungs compared to controls or even to various other interstitial lung disease. (thetechnoant.info)
The aim of study is matching patients with fibrosing interstitial lung disease (ILD) with most effective treatment right from start, improving their survival and quality of life. (ifpoc.org)
Keow J, Cecchini M, Jayawardena N, Zompatori M, Joseph M, Mura M. Digital quantification of p16-positive foci in fibrotic interstitial lung disease identifies a senescent phenotype of idiopathic pulmonary fibrosis with reduced survival. (ifpoc.org)
Although most patients enter remission and have good long-term outcomes, up to 20% develop fibrotic lung disease, whereby granulomatous inflammation evolves to pulmonary fibrosis. (livingwithsarcoidosis.org)
Although these alterations meet the definition for radiographic stage IV disease, for the purpose of this review we use the term fibrotic pulmonary sarcoidosis, as it is descriptive, applies also to CT imaging and histopathology, and avoids the implications of "staging. (livingwithsarcoidosis.org)
Defining and predicting progression in non-IPF interstitial lung disease. (nih.gov)
We ultimately found that because fibrocytes were rare and difficult to detect in the blood, removal or repolarizing of macrophages is a more viable therapeutic strategy for fibrotic lung disease. (yale.edu)
Pulmonary fibrosis share the pathogenesis process of interstitial lung disease which involve the pulmonary parenchyma . (wikidoc.org)
Idiopathic pulmonary fibrosis (IPF) is the prototypic progressive fibrotic interstitial lung disease. (lungdiseasesjournal.com)
IPF is a chronic, progressive and fibrotic lung disease of unknown cause, which typically occurs in older adults. (lungdiseasesjournal.com)
The reported overall prevalence of interstitial lung disease in New Mexico is 80.9 per 100,000 males and 67.2 per 100,000 females, corresponding with annual incidence rates of 31.5 per 100,000 per year in males and 26.1 per 100,000 per year in females. (medscape.com)
Although exposure to NTM frequently causes no symptoms, clinical manifestations may range from hypersensitivity reactions ( 2 ) to destructive, even fatal, lung disease. (cdc.gov)
In the case of lung disease caused by NTM, clinical and radiologic features are sometimes indistinguishable from those seen in lung disease caused by Mycobacterium tuberculosis complex (MTB). (cdc.gov)

To date, therapies to limit/reverse fibrosis in the lung have failed to provide long-term beneficial effects and new therapeutic targets are desperately needed. (europa.eu)
A total of 44 studies were included covering 21 mouse strains and focusing on fibrosis in the lung, liver, kidney, intestine and heart. (biomedcentral.com)

Furthermore, UIP and NSIP are frequently found affecting the same individual when lung biopsy samples are taken from different sites in patients with IPF 4 . (ersjournals.com)
Diagnostic uncertainty even after obtaining lung biopsy and lack of personalized medicine approaches are central problems in the management of these conditions. (ifpoc.org)
According to the 2002 American Thoracic Society (ATS)/European Respiratory Society (ERS) consensus statement, IPF is defined as a distinctive type of chronic fibrosing interstitial pneumonia of unknown cause limited to the lungs and with histologic features of UIP on surgical lung biopsy or certain clinical, radiologic, and pulmonary function test findings in the absence of surgical lung biopsy. (medscape.com)

Although several miRs have been shown to be associated with lung fibrosis, the mechanisms by which miRs modulate epithelial behavior in lung fibrosis are lacking. (escholarship.org)
Specifically, Clara cell secretory protein (CCSP) + epithelial progenitors contribute to repair and lung maintenance, while collagen-1 + fibroblast progenitors ( fibrocytes ) are associated with lung fibrosis, impaired lung function, and reduced survival. (rotrf.org)

Recent developments in transcriptome analysis, including single-cell RNA sequencing, have attempted to depict more detailed phenotypic characteristics of pro-fibrotic macrophages. (novartis.com)

Some of the smoker's macrophages are recruited from the circulating monocytes while some are the original alveolar macrophages residing in the lung. (wikipedia.org)
HMGA2 was increased in alveolar epithelial cells of IPF lungs. (nih.gov)
Deconvolution of RNA-seq data of alveolar organoids indicated that the fibrosis cocktail rapidly increased the proportion of transitional cell types including the KRT5-/KRT17+ aberrant basaloid phenotype recently identified in the lungs of. (lu.se)
Strong Shh appearance has been determined in reactive alveolar epithelial cells in IPF lungs. (thetechnoant.info)

These aberrant interactions elicit a global DNA hypomethylation and a selective transcriptional repression through hypermethylation of the TGF-β transcription factor SMAD3 in lung TAFs. (ibecbarcelona.eu)

Pulmonary inflammatory and fibrotic responses to carbon nanotube exposure in mice. (cdc.gov)

We evaluated the effect of the two clinically approved compounds for IPF, nintedanib and pirfenidone, and found that they reduced the expression of ECM and pro-fibrotic mediators but did not completely reverse epithelial reprogramming. (lu.se)
Historically, macrophages have been classified into two functional subtypes, "M1" and "M2," and it is well described that "M2" or "alternatively activated" macrophages contribute to fibrosis via the production of fibrotic mediators, such as TGF-β, CTGF, and CCL18. (novartis.com)

The ATS/ERS classification defines IPF as "a specific form of chronic fibrosing interstitial pneumonia of unknown aetiology, limited to the lung and associated with the histological entity of usual interstitial pneumonia" 1 . (ersjournals.com)

Repeated injury of the lung epithelium is proposed to be the main driver of idiopathic pulmonary fibrosis (IPF). (lu.se)
The aim of this thesis project is to develop the tools needed to explore the role of Hippo-YT in fibrotic lung epithelium and to identify the TFs that YT interact with to exert their various functions. (lu.se)
We further found the Hippo pathway to be dysregulated in the fibrotic lung epithelium which led to increases in nuclear YT as well as known downstream targets. (lu.se)
We found that YT have different interaction partners in the proximal and distal lung epithelium and further identified specific YT interactions in the human fibrotic lung epithelium. (lu.se)

Unlike the other idiopathic interstitial pneumonias, however, NSIP does not exhibit a clinical phenotype that distinguishes it from UIP/IPF. (ersjournals.com)

Usual interstitial pneumonia (UIP) refers to a morphologic entity defined by a combination of (1) patchy interstitial fibrosis with alternating areas of normal lung, (2) temporal heterogeneity of fibrosis characterized by scattered fibroblastic foci in the background of dense acellular collagen, and (3) architectural alteration due to chronic scarring or honeycomb change. (medscape.com)

Baseline data on demographic features, comorbidities, sarcoidosis characteristics, and pre-enrollment lung function will be captured at study entry. (figshare.com)

Even when the extent of fibrosis is anatomically limited and pulmonary function remains normal, a pathologic fibrotic response results in some degree of permanent alteration of pulmonary architecture. (livingwithsarcoidosis.org)

We analyzed the expression of TIMP-1 and its cell surface receptor CD63 in two major lung cancer subtypes: lung adenocarci-noma (ADC) and squamous cell carcinoma (SCC), and defined the tumor-promoting effects of their interac-tion. (ibecbarcelona.eu)

Relevance of fibrocytes to lung fibrosis in animal models Using the mouse model of purchase GDC-0199 bleomycin administration, CD45 Col1 CXCR4 fibrocytes were found to boost from the peripheral blood of animals chal lenged with intrapulmonary bleomycin as in contrast to saline, and after that return to regular state levels. (cret-signal.com)
In con trast, lung fibrocytes began to seem in the lung 2 days soon after bleomycin administration, peak at eight days following bleo mycin and remained elevated concerning days sixteen and 20, this time program correlated temporally with deposition of col lagen while in the lungs and suggested mobi lization of fibrocytes through the bone marrow to the blood and subsequently to your lungs. (cret-signal.com)
These functions have demon strated that in response to intra pulmonary bleomycin or lung irradiation, fibrocytes website traffic and accumulate within the lungs plus a subset differentiates into myelofibro blasts. (cret-signal.com)

An increase in transforming growth factor α (TGFα) and EGFR in rats with bleomycin-induced lung injury 16 has been observed. (lungdiseasesjournal.com)

However, how TIMP-1 is dysregulated within the tumor micro-environment and how it drives tumor progression in lung cancer is poorly understood. (ibecbarcelona.eu)

While transcription factors active in morphogenesis and differentiation from the embryonic lung could be transiently expressed during adult 1009298-09-2 lung fix, such as naphthalene injury [8], they are only rarely expressed in the normal lung. (thetechnoant.info)
It has long been assumed that patients with asthma experience intermittent attacks and have relatively normal lung function during intervening periods. (jci.org)

We were able to correlate histologic features to transcriptional signatures, matching peculiar lung microenvironments to expression of certain genes. (ifpoc.org)

Lung-specific overexpression of Shh leads to severe boost of interstitial tissues. (thetechnoant.info)

Idiopathic pulmonary fibrosis (IPF) is a devastating, progressive fibrotic lung condition that carries a median survival of 2.8-4.2 yrs, and for which no effective treatments exist 1 , 2 . (ersjournals.com)
Some forms of fibrosis, such as acute lung injury or cryptogenic organising pneumonia, are at least partially reversible, whereas others, in particular idiopathic pulmonary fibrosis (IPF), are progressive and fatal. (ersjournals.com)
This decision hinges on whether the patient presents with a predominantly inflammatory or a fibrotic progressive phenotype. (medscape.com)
Lastly, if a patient shows a progressive deterioration in lung function or shows no slowing in this decline, discontinuation of the immunosuppressive medications should be considered. (medscape.com)
More recent studies have demonstrated that asthma can cause progressive lung impairment and, in some patients, eventuate in partially reversible or irreversible airway obstruction. (jci.org)

This chest radiograph shows an enlarged cardiac silhouette and edema at the lung bases, signs of acute heart failure. (medscape.com)

Our results identify an excessive stromal TIMP-1 within the tumor microenvironment selectively in lung ADC, and implicate it in a novel tumor-promoting TAF-carcinoma crosstalk, thereby pointing to TIMP-1/CD63 interaction as a novel therapeutic target in lung cancer. (ibecbarcelona.eu)

The examination of surgical lung specimens and an integrated clinico-radiological evaluation allow the start of a complex differential diagnosis and prediction of survival, through the identification of different interstitial patterns [ 2 ]. (ersjournals.com)
According to the American Cancer Society (ACS), the 5-year relative survival rate differs significantly according to cancer type with higher rates ≥90% for prostate cancer, melanoma, skin and female breast cancer compared to lower rates ≤18% in liver, lung, and pancreatic cancer [ 1 ]. (4open-sciences.org)

One approach to understanding its pathophysiology is to know the histopathological damage generated in the lungs of those affected. (bvsalud.org)

Dysregulated microRNAs (miRs) can regulate expression of multiple genes and fundamentally alter cellular phenotypes during fibrosis. (escholarship.org)

MWCNT administered to mice by pharyngeal aspiration elicited significant inflammatory and fibrotic pathology in the lungs. (cdc.gov)

Honeycomb appearance of a fibrotic lung. (wikidoc.org)

IFN-γ Is Protective in Cytokine Release Syndrome-associated Extrapulmonary Acute Lung Injury. (yalemedicine.org)

Anatomy7

Organisms2

Diseases15

Chemicals and Drugs6

Analytical, Diagnostic and Therapeutic Techniques and Equipment7

Phenomena and Processes10

Disciplines and Occupations1

Information Science3

Fibroblasts25

Diseases21

Pathogenesis of fibrotic lung1

Transplantation14

Macrophage8

Extracellular9

Tissue17

Standard management for fibrotic2

Cytokines5

Clinical phenotypes2

Mesenchymal3

Vivo3

Fibroblast phenotype1

Studying lung fibrosis1

Anti-fibrotic1

Disorders3

Chronic lung3

Parenchymal2

Progenitor Cells2

ILDs2

Lesions1

Epithelial damage1

Pathways3

Pulmonary hypertension2

Disease29

Fibrosis in the lung2

Biopsy3

Associated with lung fibrosis2

Phenotypic1

Alveolar4

Aberrant1

Responses1

Mediators2

Unknown aetiology1

Epithelium4

Idiopathic interstitial1

Collagen1

Characteristics1

Extent of fibrosis1

Subtypes1

Fibrocytes3

Bleomycin-induced1

Poorly understood1

Normal lung2

Transcriptional Signatures1

Severe1

Progressive5

Cardiac1

Microenvironment1

Survival2

Pathophysiology1

Cellular1

Pathology1

Honeycomb1

Acute lung i1