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idiopathic lung fibrosis

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https://www.readbyqxmd.com/read/27910075/idiopathic-pulmonary-fibrosis-are-any-of-the-morphological-molecular-markers-useful-in-clinical-management
#1
REVIEW
Francesca Lunardi, Elisabetta Balestro, Nazarena Nannini, Stefania Edith Vuljan, Federico Rea, Fiorella Calabrese
Idiopathic pulmonary fibrosis (IPF), the most common form of chronic interstitial lung disease, is a severe progressive fibrotic disorder of unknown aetiology. The disease has a heterogeneous clinical course, with frequent poor prognosis, similar to malignant disease. Correctly diagnosing IPF has become particularly important in view of the availability of more precise therapeutic indications, thus avoiding steroid treatment and allowing new approaches with novel drugs. To date we have limited information about biomarkers predictive of progressive disease and associated complications...
December 2, 2016: Histology and Histopathology
https://www.readbyqxmd.com/read/27909724/hsp27-regulates-tgf-%C3%AE-mediated-lung-fibroblast-differentiation-through-the-smad3-and-erk-pathways
#2
Gang Wang, Hao Jiao, Jun-Nian Zheng, Xia Sun
Idiopathic pulmonary fibrosis (IPF) is a chronic lethal interstitial lung disease with unknown etiology. Recent studies have indicated that heat-shock protein 27 (HSP27) contributes to the pathogenesis of IPF through the regulation of epithelial-mesenchymal transition (EMT). However, the expression and role of HSP27 in fibroblasts during pulmonary fibrogenesis has not been investigated to date, at least to the best of our knowledge. In this study, we examined the expression of HSP27 in fibrotic lung tissue and fibroblasts from bleomycin (BLM)-challenged mice and human lung fibroblasts treated with transforming growth factor-β (TGF-β)...
November 28, 2016: International Journal of Molecular Medicine
https://www.readbyqxmd.com/read/27905295/desensitization-of-idiopathic-pulmonary-fibrosis-fibroblasts-to-alternaria-alternata-extract-mediated-necrotic-cell-death
#3
Jintaek Im, Kyutae Kim, Ji Young Yhee, Scott M O'Grady, Richard S Nho
Alternaria alternata is an allergenic fungus and known to cause an upper respiratory tract infection and asthma in humans with compromised immunity. Although A. alternata's effect on airway epithelial cells has previously been examined, the potential role of A. alternata on lung fibroblast viability is not understood. Since lung fibroblasts derived from patients with idiopathic pulmonary fibrosis (IPF) display a distinct phenotype that is resistant to stress and cell death inducing conditions, the investigation of the role of Alternaria on pathological IPF fibroblasts provides a better understanding of the fibrotic process induced by an allergenic fungus...
November 2016: Physiological Reports
https://www.readbyqxmd.com/read/27899769/the-role-of-club-cell-phenoconversion-and-migration-in-idiopathic-pulmonary-fibrosis
#4
Jutaro Fukumoto, Ramani Soundararajan, Joseph Leung, Ruan Cox, Sanjay Mahendrasah, Neha Muthavarapu, Travis Herrin, Alexander Czachor, Lee C Tan, Nima Hosseinian, Priyanshi Patel, Jayanthraj Gone, Mason T Breitzig, Young Cho, Andrew J Cooke, Lakshmi Galam, Venkata Ramireddy Narala, Yashwant Pathak, Richard F Lockey, Narasaiah Kolliputi
Idiopathic pulmonary fibrosis (IPF) is an age-related multifactorial disease featuring non-uniform lung fibrosis. The decisive cellular events at early stages of IPF are poorly understood. While the involvement of club cells in IPF pathogenesis is unclear, their migration has been associated with lung fibrosis. In this study, we labeled club cells immunohistochemically in IPF lungs using a club cell marker Claudin-10 (Cldn10), a unique protein based on the recent report which demonstrated that the appearance of Cldn10 in developing and repairing lungs precedes other club cell markers including club cell secretory protein (CCSP)...
November 29, 2016: Aging
https://www.readbyqxmd.com/read/27896060/diffuse-bronchiectasis-as-the-primary-manifestation-of-endobronchial-sarcoidosis
#5
Paul D Hiles, Kenneth R Kemp, Jean M Coviello
Sarcoidosis is an idiopathic disease that most commonly involves the lungs and is characterized by granulomatous inflammation. Bronchiectasis is one pulmonary manifestation of sarcoidosis, although it is almost always observed as traction bronchiectasis in the setting of fibrotic lung disease. A 50-year-old woman was evaluated for chronic cough and bronchiectasis with a small amount of peripheral upper lobe honeycombing and no significant pulmonary fibrosis or lymphadenopathy. After an extensive laboratory and imaging evaluation did not identify a cause of her bronchiectasis, bronchoscopy was performed to assess for primary ciliary dyskinesia and revealed a diffuse cobblestone appearance of the airway mucosa...
2017: Respiratory Medicine Case Reports
https://www.readbyqxmd.com/read/27894300/pleural-inhibition-of-the-caspase-1-il-1%C3%AE-pathway-diminishes-profibrotic-lung-toxicity-of-bleomycin
#6
Olivier Burgy, Pierre-Simon Bellaye, Sebastien Causse, Guillaume Beltramo, Guillaume Wettstein, Pierre-Marie Boutanquoi, Françoise Goirand, Carmen Garrido, Philippe Bonniaud
BACKGROUND: Idiopathic and toxic pulmonary fibrosis are severe diseases starting classically in the subpleural area of the lung. It has recently been suggested that pleural mesothelial cells acquire a myofibroblast phenotype under fibrotic conditions induced by TGF-β1 or bleomycin. The importance and role of inflammation in fibrogenesis are still controversial. In this work, we explored the role of IL-1β/caspase-1 signaling in bleomycin lung toxicity and in pleural mesothelial cell transformation...
November 29, 2016: Respiratory Research
https://www.readbyqxmd.com/read/27888985/azathioprine-response-in-patients-with-fibrotic-connective-tissue-disease-associated-interstitial-lung-disease
#7
Justin M Oldham, Cathryn Lee, Eleanor Valenzi, Leah J Witt, Ayodeji Adegunsoye, Scully Hsu, Lena Chen, Steven Montner, Jonathan H Chung, Imre Noth, Rekha Vij, Mary E Strek
BACKGROUND: Azathioprine is a commonly prescribed therapy for connective tissue disease-associated interstitial lung disease (CTD-ILD). Combination therapy that included azathioprine was recently shown to increase the risk of death and hospitalization in patients with idiopathic pulmonary fibrosis. Whether azathioprine increases the risk of adverse outcomes in patients with fibrotic CTD-ILD, including those with CTD-associated usual interstitial pneumonia (UIP), remains unknown. METHODS: A retrospective cohort analysis was performed to determine the combined incidence rate of death, transplant and respiratory hospitalization associated with azathioprine exposure...
December 2016: Respiratory Medicine
https://www.readbyqxmd.com/read/27886850/cellular-senescence-and-autophagy-in-the-pathogenesis-of-chronic-obstructive-pulmonary-disease-copd-and-idiopathic-pulmonary-fibrosis-ipf
#8
REVIEW
Kazuyoshi Kuwano, Jun Araya, Hiromichi Hara, Shunsuke Minagawa, Naoki Takasaka, Saburo Ito, Kenji Kobayashi, Katsutoshi Nakayama
Aging is associated with impairments in homeostasis. Although aging and senescence are not equivalent, the number of senescent cells increases with aging. Cellular senescence plays important roles in tissue repair or remodeling, as well as embryonic development. Autophagy is a process of lysosomal self-degradation that maintains a homeostatic balance between the synthesis, degradation, and recycling of cellular proteins. Autophagy diminishes with aging; additionally, accelerated aging can be attributed to reduced autophagy...
November 2016: Respiratory Investigation
https://www.readbyqxmd.com/read/27884593/idiopathic-interstitial-pneumonia-associated-pulmonary-hypertension-a-target-for-therapy
#9
Steven D Nathan, Jürgen Behr, Vincent Cottin, Harold R Collard, Marius M Hoeper, Fernando J Martinez, Athol U Wells
Advances in the treatment of idiopathic interstitial pneumonia (IIP) represent an urgent, unmet medical need for patients with this category of diffuse parenchymal lung disease. IIPs involve varying combinations of fibrosis and inflammation of unknown cause and may be associated with pulmonary hypertension (PH). When it occurs, PH is associated with higher oxygen needs, greater functional impairment, and increased mortality. However, whether or when PH is a maladaptive versus adaptive phenomenon remains to be determined...
November 5, 2016: Respiratory Medicine
https://www.readbyqxmd.com/read/27878273/pi3k-akt-signaling-is-involved-in-the-pathogenesis-of-bleomycin%C3%A2-induced-pulmonary-fibrosis-via-regulation-of-epithelial%C3%A2-mesenchymal-transition
#10
Xiao-Ling Zhang, Rong-Ge Xing, Liang Chen, Chun-Rong Liu, Zhi-Gang Miao
Idiopathic pulmonary fibrosis (IPF) is a progressive lung disease characterized by chronic inflammation, fibroblast proliferation and extracellular matrix deposition. However, the molecular and cellular mechanisms underlying the pathogenesis of pulmonary fibrosis remain to be fully elucidated. The contribution of the phosphoinositide 3‑kinase (PI3K)/protein kinase B (Akt) pathway in fibrotic processes remains to be investigated. The aim of the present study was to investigate the role of the PI3K/Akt pathway in pulmonary fibrosis...
November 22, 2016: Molecular Medicine Reports
https://www.readbyqxmd.com/read/27878256/melatonin-attenuates-tgf%C3%AE-1-induced-epithelial-mesenchymal-transition-in-lung-alveolar-epithelial-cells
#11
Na Yu, Yi-Tian Sun, Xin-Ming Su, Miao He, Bing Dai, Jian Kang
Idiopathic pulmonary fibrosis (IPF) is the most common interstitial lung disease. However, the pathogenesis remains to be fully elucidated. Melatonin is secreted by the pineal gland, it has a strong antioxidant effect, and exerts an anti-fibrosis effect. Whether melatonin attenuates pulm -onary fibrosis by inhibiting epithelial‑mesenchymal transition (EMT) requires further research. The present study aimed to investigate whether melatonin prevents transforming growth factor‑β1 (TGF‑β1)‑induced EMT and underlying signaling pathways using reverse transcription‑quantitative polymerase chain reaction, western blot analysis and immunofluorescence...
November 16, 2016: Molecular Medicine Reports
https://www.readbyqxmd.com/read/27871152/human-adipose-derived-mesenchymal-stem-cells-attenuate-early-stage-of-bleomycin-induced-pulmonary-fibrosis-comparison-with-pirfenidone
#12
Manoj Reddy, Lyle Fonseca, Shashank Gowda, Basavraj Chougule, Aarya Hari, Satish Totey
Background and Objectives: Idiopathic pulmonary fibrosis (IPF) is a progressive, irreversible, invariably fatal fibrotic lung disease with no lasting option for therapy. Mesenchymal stem cells (MSCs) could be a promising modality for the treatment of IPF. Aim of the study was to investigate improvement in survivability and anti-fibrotic efficacy of human adipose-derived mesenchymal stem cells (AD-MSCs) in comparison with pirfenidone in the bleomycin-induced pulmonary fibrosis model. Methods: Human AD-MSCs were administered intravenously on day 3, 6 and 9 after an intra-tracheal challenge with bleomycin, whereas, pirfenidone was given orally in drinking water at the rate of 100 mg/kg body weight three times a day daily from day 3 onward...
November 30, 2016: International Journal of Stem Cells
https://www.readbyqxmd.com/read/27869174/developmental-reprogramming-in-mesenchymal-stromal-cells-of-human-subjects-with-idiopathic-pulmonary-fibrosis
#13
Diptiman Chanda, Ashish Kurundkar, Sunad Rangarajan, Morgan Locy, Karen Bernard, Nirmal S Sharma, Naomi J Logsdon, Hui Liu, David K Crossman, Jeffrey C Horowitz, Stijn De Langhe, Victor J Thannickal
Cellular plasticity and de-differentiation are hallmarks of tissue/organ regenerative capacity in diverse species. Despite a more restricted capacity for regeneration, humans with age-related chronic diseases, such as cancer and fibrosis, show evidence of a recapitulation of developmental gene programs. We have previously identified a resident population of mesenchymal stromal cells (MSCs) in the terminal airways-alveoli by bronchoalveolar lavage (BAL) of human adult lungs. In this study, we characterized MSCs from BAL of patients with stable and progressive idiopathic pulmonary fibrosis (IPF), defined as <5% and ≥10% decline, respectively, in forced vital capacity over the preceding 6-month period...
November 21, 2016: Scientific Reports
https://www.readbyqxmd.com/read/27868303/fat-free-mass-index-predicts-survival-in-patients-with-idiopathic-pulmonary-fibrosis
#14
Osamu Nishiyama, Ryo Yamazaki, Hiroyuki Sano, Takashi Iwanaga, Yuji Higashimoto, Hiroaki Kume, Yuji Tohda
BACKGROUND AND OBJECTIVE: Detailed body composition, such as fat-free mass, has not been examined in idiopathic pulmonary fibrosis (IPF). We investigated whether the fat-free mass index (FFMI), an index of lean body mass, predicted survival. METHODS: Forty-four patients with IPF were enrolled in the study. Their body composition was assessed using direct segmental multi-frequency bioelectrical impedance analysis. The degree of correlation between variables of body composition and other variables such as forced vital capacity (FVC) and survival was examined...
November 21, 2016: Respirology: Official Journal of the Asian Pacific Society of Respirology
https://www.readbyqxmd.com/read/27867035/two-way-conversion-between-lipogenic-and-myogenic-fibroblastic-phenotypes-marks-the-progression-and-resolution-of-lung-fibrosis
#15
Elie El Agha, Alena Moiseenko, Vahid Kheirollahi, Stijn De Langhe, Slaven Crnkovic, Grazyna Kwapiszewska, Djuro Kosanovic, Felix Schwind, Ralph T Schermuly, Ingrid Henneke, BreAnne MacKenzie, Jennifer Quantius, Susanne Herold, Aglaia Ntokou, Katrin Ahlbrecht, Rory E Morty, Andreas Günther, Werner Seeger, Saverio Bellusci
Idiopathic pulmonary fibrosis (IPF) is a form of progressive interstitial lung disease with unknown etiology. Due to a lack of effective treatment, IPF is associated with a high mortality rate. The hallmark feature of this disease is the accumulation of activated myofibroblasts that excessively deposit extracellular matrix proteins, thus compromising lung architecture and function and hindering gas exchange. Here we investigated the origin of activated myofibroblasts and the molecular mechanisms governing fibrosis formation and resolution...
November 14, 2016: Cell Stem Cell
https://www.readbyqxmd.com/read/27866277/identification-of-key-cost-generating-events-for-idiopathic-pulmonary-fibrosis-a-systematic-review
#16
REVIEW
Shalvaree Vaidya, Clare L Hibbert, Elizabeth Kinter, Stefan Boes
BACKGROUND: Idiopathic pulmonary fibrosis (IPF) is an incurable, debilitating disease which impairs lung function and eventually leads to death. Currently, there is a lack of effective modifying therapies and treatments for IPF as the underlying epidemiological mechanism is not clearly understood. This leads to difficulty in diagnosing and managing IPF, which results in a high incurment of disease-associated cost. Even though IPF poses a substantial economic burden, there is a lack of research available on cost triggers and healthcare utilization, which can be a barrier to future economic evaluations of new medicines for IPF...
November 19, 2016: Lung
https://www.readbyqxmd.com/read/27865876/interstitial-lung-disease-in-the-elderly
#17
Karen C Patterson, Rupal J Shah, Mary K Porteous, Jason D Christie, Carly D'Errico, Matthew Chadwick, Matthew Triano, Charuhas Deshpande, Milton D Rossman, Leslie A Litzky, Maryl E Kreider, Wallace T Miller
BACKGROUND: Despite the relationship of idiopathic pulmonary fibrosis (IPF) with advancing age, little is known about the epidemiology of interstitial lung diseases (ILD) in the elderly. Here we describe the diagnoses, clinical characteristics, and outcomes of patients who were elderly at the time of ILD diagnosis. METHODS: Among subjects from a prospective cohort study of ILD, elderly was defined as age > 70. Diagnoses were derived from a multi-disciplinary review...
November 16, 2016: Chest
https://www.readbyqxmd.com/read/27864288/the-role-of-nuclear-factor-erythroid-2-related-factor-2-nrf-2-in-the-protection-against-lung-injury
#18
Hailin Zhao, Shiori Eguchi, Azeem Alam, Daqing Ma
Nuclear factor-erythroid 2 related factor 2 (Nrf2) is a ubiquitous master transcription factor that upregulates antioxidant response elements (AREs)-mediated expression of antioxidant enzyme and cytoprotective proteins. Activation of Nrf2 has been shown to be protective against lung injury. In the lung, diverse stimuli including environmental oxidants, medicinal agents and pathogens can activate Nrf2. Nrf2 translocates to the nucleus and binds to an antioxidant response element (ARE). Through transcriptional induction of ARE-bearing genes encoding antioxidant-detoxifying proteins, Nrf2 induces cellular rescue pathways against oxidative pulmonary injury, abnormal inflammatory and immune responses, and apoptosis...
November 18, 2016: American Journal of Physiology. Lung Cellular and Molecular Physiology
https://www.readbyqxmd.com/read/27863518/safety-and-efficacy-of-bridging-to-lung-transplantation-with-antifibrotic-drugs-in-idiopathic-pulmonary-fibrosis-a-case-series
#19
Isabelle Delanote, Wim A Wuyts, Jonas Yserbyt, Eric K Verbeken, Geert M Verleden, Robin Vos
BACKGROUND: Following recent approval of pirfenidone and nintedanib for idiopathic pulmonary fibrosis (IPF), questions arise about the use of these antifibrotics in patients awaiting lung transplantation (LTx). METHODS: Safety and efficacy of antifibrotic drugs in IPF patients undergoing LTx were investigated in a single-centre retrospective cohort analysis. RESULTS: A total of nine patients, receiving antifibrotic therapy for 419 ± 315 days until subsequent LTx, were included...
November 18, 2016: BMC Pulmonary Medicine
https://www.readbyqxmd.com/read/27862639/a-simple-dyspnoea-scale-as-part-of-the-assessment-to-predict-outcome-across-chronic-interstitial-lung-disease
#20
Hadeel Khadawardi, Marco Mura
BACKGROUND AND OBJECTIVE: The Medical Research Council dyspnoea score (MRCDS) is a simple, objective scale to assess dyspnoea, the main complaint in patients with chronic interstitial lung disease (ILD). We sought to investigate whether MRCDS is a predictor of outcome in patients with chronic ILD. METHODS: One hundred and fifteen patients (50 idiopathic pulmonary fibrosis (IPF) and 65 non-IPF ILD) were retrospectively studied. Baseline (time of diagnosis) MRCDS and 3-6-month changes were considered...
November 8, 2016: Respirology: Official Journal of the Asian Pacific Society of Respirology
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