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Fibrosis pulmonary

Matea Cedilak, Mihailo Banjanac, Daniela Belamarić, Andrea Paravić Radičević, Ivan Faraho, Krunoslav Ilić, Snježana Čužić, Ines Glojnarić, Vesna Eraković Haber, Martina Bosnar
Idiopathic pulmonary fibrosis (IPF) is a complex lung disease with incompletely understood pathophysiology. Effectiveness of available medicines is limited and the need for new and improved therapies remains. Due to complexity of the disease, it is difficult to develop predictable in vitro models. In this study we have described precision-cut lung slices (PCLS) prepared from bleomycin treated mice as an in vitro model for testing of novel compounds with antifibrotic activity. We have shown that PCLS during in vitro incubation retain characteristics of bleomycin model with increased expression of fibrosis related genes ACTA2 (α-smooth muscle actin), COL1A1 (collagen 1), FN1 (fibronectin 1), MMP12 (matrix metalloproteinase 12) and TIMP1 (tissue inhibitor of metalloproteinases)...
February 15, 2019: Pulmonary Pharmacology & Therapeutics
Claudia Ravaglia, Marcello Bosi, Athol U Wells, Carlo Gurioli, Christian Gurioli, Alessandra Dubini, Sara Piciucchi, Silvia Puglisi, Susanna Mascetti, Antonella Arcadu, Sara Tomassetti, Venerino Poletti
Background: Prognostic evaluation in idiopathic pulmonary fibrosis (IPF) may be important as it can guide management decisions, but the potential role of honeycomb changes in providing information about outcome and survival of patients with IPF, particularly if diagnosed using cryobiopsy, has not been evaluated. Aim of this study was to determinate whether a relationship exists between honeycombing on cryobiopsy and clinical/radiological picture and outcome in patients with IPF and to assess whether the same pathologic criteria that have been used to define the UIP pattern (usual interstitial pneumonia) for surgical biopsy can also be applied to cryobiopsy...
2019: Multidisciplinary Respiratory Medicine
Manish Bodas, Neeraj Vij
Cystic fibrosis (CF), a fatal genetic disorder predominant in the Caucasian population, is caused by mutations in the cystic fibrosis transmembrane conductance regulator ( Cftr ) gene. The most common mutation is the deletion of phenylalanine from the position-508 (F508del-CFTR), resulting in a misfolded-CFTR protein, which is unable to fold, traffic and retain its plasma membrane (PM) localization. The resulting CFTR dysfunction, dysregulates variety of key cellular mechanisms such as chloride ion transport, airway surface liquid (ASL) homeostasis, mucociliary-clearance, inflammatory-oxidative signaling, and proteostasis that includes ubiquitin-proteasome system (UPS) and autophagy...
2019: Frontiers in Pharmacology
Yaqiong Tian, Hui Li, Yujuan Gao, Chuanmei Liu, Ting Qiu, Hongyan Wu, Mengshu Cao, Yingwei Zhang, Hui Ding, Jingyu Chen, Hourong Cai
Background: Idiopathic pulmonary fibrosis (IPF) is a progressive, eventually fatal disease. IPF is characterized by excessive accumulation of the extracellular matrix (ECM) in the alveolar parenchyma and progressive lung scarring. The pathogenesis of IPF and whether the ECM involved in the process remain unknown. Methods: To identify potential treatment target and ECM associated proteins that may be involved in the development of IPF, we employed isobaric tag for relative and absolute quantitation (iTRAQ) combined liquid chromatography-tandem mass spectrometry (LC-MS/MS) approach to examine protein expression in lung tissues from IPF patients...
2019: Clinical Proteomics
Zahava Vadasz, Alexandra Balbir Gurman, Pierluigi Meroni, Dominique Farge, Yair Levi, Francesca Ingegnoli, Yolada Braun-Moscovici, Itzhak Rosner, Gleb Slobodin, Michael Rozenbaum, Nizar Jiries, Lisa Kaly, Nina Boulman, Karina Zilber, Shira Ginsberg, Abid Awisat, Yair Goldberg, Michael Lurie, Maria-Rosa Ghigna, Christophe Guignabert, Marc Humbert, Doron Rimar
OBJECTIVE: Lysyl oxidase (LOX) is an extracellular enzyme that cross-links collagen fibrils. LOX was found to be increased in serum of SSc patients and was suggested to be related to skin fibrosis, yet a vascular source of LOX has been demonstrated in idiopathic pulmonary arterial hypertension (iPAH). We aimed to validate elevated LOX serum levels in SSc and to study its correlation with clinical characteristics and investigate its main source at the tissue level. METHODS: A total of 86 established SSc patients were compared with 86 patients with very early diagnosis of systemic sclerosis (VEDOSS), 110 patients with primary RP (PRP) and 80 healthy controls...
February 15, 2019: Rheumatology
Susan K Mathai, David A Schwartz
Pulmonary fibrosis refers to the development of diffuse parenchymal abnormalities in the lung that cause dyspnea, cough, hypoxemia, and impair gas exchange, ultimately leading to respiratory failure. Though pulmonary fibrosis can be caused by a variety of underlying etiologies, ranging from genetic defects to autoimmune diseases to environmental exposures, once fibrosis develops it is irreversible and most often progressive, such that fibrosis of the lung is one of the leading indications for lung transplantation...
February 5, 2019: Translational Research: the Journal of Laboratory and Clinical Medicine
Franz Puttur, Lisa G Gregory, Clare M Lloyd
The lungs present a challenging immunological dilemma for the host. Anatomically positioned at the environmental interface, they are constantly exposed to antigens, pollutants and microbes, while simultaneously facilitating vital gas exchange. Remarkably, the lungs maintain a functionally healthy state, ignoring harmless inhaled proteins, adapting to toxic environmental insults and limiting immune responses to allergens and pathogenic microbes. This functional strategy of environmental adaptation maintains immune defense, reduces tissue damage, and promotes and sustains lung immune tolerance...
February 15, 2019: Immunology and Cell Biology
Sung Woo Moon, Ji Soo Choi, Sang Hoon Lee, Kyung Soo Jung, Ji Ye Jung, Young Ae Kang, Moo Suk Park, Young Sam Kim, Joon Chang, Song Yee Kim
BACKGROUND: Sarcopenia can contribute to negative outcomes in patients with various lung diseases. However, whether sarcopenia affects prognosis in patients with idiopathic pulmonary fibrosis (IPF) has not been reported. Simple measures of muscle mass, derived from chest computed tomography (CT), are increasingly being used to identify patients with sarcopenia. We hypothesized that skeletal muscle mass could be a predictor of prognosis in IPF patients. METHODS: We retrospectively evaluated 180 patients diagnosed with IPF between January 2010 and December 2015 at a tertiary care hospital in South Korea...
February 15, 2019: Respiratory Research
Jennifer L Philip, Thomas M Murphy, David A Schreier, Sydney Stevens, Diana Marcela Tabima, Marjorie E Albrecht, Andrea L Frump, Timothy A Hacker, Tim Lahm, Naomi Chesler
MOTIVATION: Left heart failure (LHF) is the most common cause of pulmonary hypertension, which confers an increase in morbidity and mortality in this context. Pulmonary vascular resistance has prognostic value in LHF but otherwise the mechanical consequences of LHF for the pulmonary vasculature and right ventricle remain unknown. We sought to investigate mechanical mechanisms of pulmonary vascular and right ventricular dysfunction in a rodent model of LHF to address the knowledge gaps in understanding disease pathophysiology...
February 15, 2019: American Journal of Physiology. Heart and Circulatory Physiology
David Fulton, Xueyi Li, Zsuzsanna Bordan, Yusi Wang, Keyvan Mahboubi, Radu Daniel Rudic, Stephen Haigh, Feng Chen, Scott A Barman
SIGNIFICANCE: Pulmonary Arterial Hypertension (PAH) is a progressive disease arising from the narrowing of pulmonary arteries (PA) resulting in high pulmonary arterial blood pressure and ultimately right ventricular (RV) failure. A defining characteristic of PAH is the excessive and unrelenting inward remodeling of PA that includes increased proliferation, inflammation and fibrosis. CRITICAL ISSUES: There is no cure for PAH nor interventions that effectively arrest or reverse PA remodeling and intensive research over the past several decades has sought to identify novel molecular mechanisms of therapeutic value...
February 15, 2019: Antioxidants & Redox Signaling
Liteng Yang, Xin Liu, Ning Zhang, Lifang Chen, Jingyi Xu, Wencheng Tang
Pulmonary fibrosis is a lethal inflammatory disease. In this study, we aimed to explore the potential-related circular RNAs (circRNAs) and genes that are associated with pulmonary fibrosis. Pulmonary fibrosis rat models were constructed and the fibrosis deposition was detected using hematoxylin and eosin and Masson staining. The differentially expressed circRNAs were obtained through RNA sequencing. Gene Ontology (GO) and Kyoto Encyclopedia of Genes and Genomes (KEGG) analyses were further performed to uncover the key function and pathways in pulmonary fibrosis...
February 14, 2019: Journal of Cellular Biochemistry
Walid K Abdelbasset, Gaber S Soliman, Ahmed A Elshehawy, Saud M Alrawaili
Background: Impairment of peripheral skeletal muscle function is a common phenomenon in patients with cystic fibrosis (CF) in addition to great clinical connotations, such as lack of exercise tolerance and decrease of health-related quality of life. There is very limited data on the effects of maximal exercise on muscle fatiguability and exercise capacity in children with cystic fibrosis. Objectives: The aim of this study was to evaluate the effect of progressive maximal exercise training of the lower extremities on exercise capacity and muscle fatiguability in children with cystic fibrosis...
December 2018: African Health Sciences
Pauline T Lukey, Stephen A Harrison, Shuying Yang, Yim Man, Beverley F Holman, Alaleh Rashidnasab, Gabrielle Azzopardi, Michael Grayer, Juliet K Simpson, Philippe Bareille, Lyn Paul, Hannah V Woodcock, Richard Toshner, Peter Saunders, Philip L Molyneaux, Kris Thielemans, Frederick J Wilson, Paul F Mercer, Rachel C Chambers, Ashley M Groves, William A Fahy, Richard P Marshall, Toby M Maher
PI3 Kinases (PI3Ks) and mammalian target of rapamycin (mTOR) play a role in the pathogenesis of idiopathic pulmonary fibrosis (IPF). Omipalisib (GSK2126458) is a potent inhibitor of PI3K/mTOR.A randomised, placebo-controlled, double-blind, repeat dose escalation, experimental medicine study of omipalisib in subjects with IPF was conducted (NCT01725139) to test safety, tolerability, pharmacokinetics (PK) and pharmacodynamics (PD). Omipalisib was dosed, at 0.25 mg, 1 mg and 2 mg twice per day (BID) for approximately eight days in 4 cohorts of 4 subjects randomised 3:1 to receive omipalisib or placebo (two cohorts received 2 mg BID)...
February 14, 2019: European Respiratory Journal: Official Journal of the European Society for Clinical Respiratory Physiology
Valentino Bezzerri, Francesco Piacenza, Nicole Caporelli, Marco Malavolta, Mauro Provinciali, Marco Cipolli
Pulmonary disease is the main cause of the morbidity and mortality of patients affected by cystic fibrosis (CF). The lung pathology is dominated by excessive recruitment of neutrophils followed by an exaggerated inflammatory process that has also been reported to occur in the absence of apparent pathogenic infections. Airway surface dehydration and mucus accumulation are the driving forces of this process. The continuous release of reactive oxygen species and proteases by neutrophils contributes to tissue damage, which eventually leads to respiratory insufficiency...
February 14, 2019: Respiratory Research
Yan Geng, Xue Liu, Jiurong Liang, David M Habiel, Kulur Vrishika, Ana Lucia Coelho, Nan Deng, Ting Xie, Yizhou Wang, Ningshan Liu, Guanling Huang, Adrianne Kurkciyan, Zhenqiu Liu, Jie Tang, Cory M Hogaboam, Dianhua Jiang, Paul W Noble
Idiopathic pulmonary fibrosis (IPF) is a progressive disease with unremitting extracellular matrix deposition, leading to a distortion of pulmonary architecture and impaired gas exchange. Fibroblasts from IPF patients acquire an invasive phenotype that is essential for progressive fibrosis. Here, we performed RNA-seq analysis on invasive and non-invasive fibroblasts and found that the immune checkpoint ligand CD274 (PD-L1) was up-regulated on invasive lung fibroblasts and was required for the invasive phenotype of lung fibroblasts, is regulated by P53 and FAK, and drives lung fibrosis in a humanized IPF model in mice...
February 14, 2019: JCI Insight
Angela Sepe, Valeria R Villella, Chiara Cimbalo, Alice Castaldo, Francesco Nunziata, Adele Corcione, Gianni Bona, Luigi Maiuri, Valeria Raia
Structural lung disease begins very early in children with Cystic Fibrosis, often in the first three months of life. Inhaled medications represent an attractive therapeutic approach in Cystic Fibrosis that are routinely used as early intervention strategies. Two aerosolized solutions, hypertonic saline and dornase alfa, have significant potential benefits by improving mucociliary clearance, with minimal associated side-effects. In particular, they favor rehydration of airway surface liquid and cleavage of extracellular DNA in the airways, respectively, consequently reducing rate of pulmonary disease exacerbations...
February 13, 2019: Minerva Pediatrica
Marianne Skov, Christine Rønne Hansen, Tacjana Pressler
Cystic fibrosis (CF) is a severe, monogenic, autosomal recessive disease caused by mutations in the CFTR (cystic fibrosis transmembrane regulator) gene, where disturbed chloride and bicarbonate transportation in epithelial cells results in a multiorgan disease with primarily pulmonary infections and pancreatic insufficiency. In 1968, the Copenhagen CF Center was established, and centralized care of CF patients with monthly control was introduced. Close monitoring and treatment of Pseudomonas lung infection as well as segregation of patients with different infection status improved the clinical outcome as well as survival...
February 14, 2019: APMIS: Acta Pathologica, Microbiologica, et Immunologica Scandinavica
Tingting Xie, Qiaoyi Xu, Hanxi Wan, Shunpeng Xing, Chen Shang, Yuan Gao, Zhengyu He
Pulmonary fibrosis is a major cause of death in patients with acute respiratory distress syndrome (ARDS). Our previous study revealed that lipopolysaccharide (LPS) challenge could lead to mouse lung fibroblast proliferation. Additionally, inhibition of autophagy in lung fibroblasts was also reported to be crucial during the process of pulmonary fibrosis. However, the correlation between proliferation and inhibition of autophagy of lung fibroblasts and the underlying mechanism remain unknown. In this study, we report that autophagy was inhibited in mouse lung fibroblasts after LPS challenge, and was accompanied by activation of the PI3K-Akt-mTOR signaling pathway...
February 13, 2019: Laboratory Investigation; a Journal of Technical Methods and Pathology
Laure Ricard, Vincent Jachiet, Florent Malard, Yishan Ye, Nicolas Stocker, Sébastien Rivière, Patricia Senet, Jean-Benoit Monfort, Olivier Fain, Mohamad Mohty, Béatrice Gaugler, Arsène Mekinian
OBJECTIVES: Systemic sclerosis (SSc) is an autoimmune disease characterised by widespread fibrosis, microangiopathy and autoantibodies. Follicular helper T (Tfh) cells CD4+ CXCR5+ PD-1+ cooperate with B lymphocytes to induce the differentiation of plasmocytes secreting immunoglobulins (Ig). Circulating Tfh (cTfh) cells are increased in several autoimmune diseases. However, there are no data about cTfh cells and their interaction with B cells in SSc. The aim of this study was to perform a quantitative and functional analysis of cTfh cells in SSc...
February 13, 2019: Annals of the Rheumatic Diseases
Gitte Berkers, Peter van Mourik, Annelotte M Vonk, Evelien Kruisselbrink, Johanna F Dekkers, Karin M de Winter-de Groot, Hubertus G M Arets, Rozemarijn E P Marck-van der Wilt, Jasper S Dijkema, Maaike M Vanderschuren, Roderick H J Houwen, Harry G M Heijerman, Eduard A van de Graaf, Sjoerd G Elias, Christof J Majoor, Gerard H Koppelman, Jolt Roukema, Marleen Bakker, Hettie M Janssens, Renske van der Meer, Robert G J Vries, Hans C Clevers, Hugo R de Jonge, Jeffrey M Beekman, Cornelis K van der Ent
In vitro drug tests using patient-derived stem cell cultures offer opportunities to individually select efficacious treatments. Here, we provide a study that demonstrates that in vitro drug responses in rectal organoids from individual patients with cystic fibrosis (CF) correlate with changes in two in vivo therapeutic endpoints. We measured individual in vitro efficaciousness using a functional assay in rectum-derived organoids based on forskolin-induced swelling and studied the correlation with in vivo effects...
February 12, 2019: Cell Reports
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