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idiopathic fibrosis pulmonary

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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/27905849/pathways-to-precision-medicine-in-idiopathic-pulmonary-fibrosis-time-to-relax
#3
Nathan Hambly, Martin Kolb
No abstract text is available yet for this article.
December 1, 2016: American Journal of Respiratory and Critical Care Medicine
https://www.readbyqxmd.com/read/27905295/desensitization-of-idiopathic-pulmonary-fibrosis-fibroblasts-to-alternaria-alternata-extract-mediated-necrotic-cell-death
#4
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
#5
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
#6
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
#7
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/27890713/allogeneic-human-mesenchymal-stem-cells-in-patients-with-idiopathic-pulmonary-fibrosis-via-intravenous-delivery-aether-a-phase-i-safety-clinical-trial
#8
Marilyn K Glassberg, Julia Minkiewicz, Rebecca L Toonkel, Emmanuelle S Simonet, Gustavo A Rubio, Darcy Difede, Shirin Shafazand, Aisha Khan, Marietsy V Pujol, Vincent F LaRussa, Lisa H Lancaster, Glenn D Rosen, Joel Fishman, Yolanda N Mageto, Adam Mendizabal, Joshua M Hare
BACKGROUND: Despite recent FDA approval of two new drugs for idiopathic pulmonary fibrosis (IPF), curative therapies remain elusive and mortality remains high. Pre-clinical and clinical data support the safety of human mesenchymal stem cells as a potential novel therapy for this fatal condition. The AETHER trial was the first study designed to evaluate the safety of a single infusion of bone marrow-derived mesenchymal stem cells in patients with idiopathic pulmonary fibrosis. METHODS: Nine patients with mild to moderate IPF were sequentially assigned to one of three cohorts and dosed with a single intravenous infusion of 20, 100, or 200 x 10(6) human bone marrow-derived mesenchymal stem cells per infusion from young, unrelated, male donors...
November 24, 2016: Chest
https://www.readbyqxmd.com/read/27888985/azathioprine-response-in-patients-with-fibrotic-connective-tissue-disease-associated-interstitial-lung-disease
#9
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
#10
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
#11
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/27883184/epigenetics-in-reactive-and-reparative-cardiac-fibrogenesis-the-promise-of-epigenetic-therapy
#12
REVIEW
Asish K Ghosh, Rahul Rai, Panagiotis Flevaris, Douglas E Vaughan
Epigenetic changes play a pivotal role in the development of a wide spectrum of human diseases including cardiovascular diseases, cancer, diabetes, and intellectual disabilities. Cardiac fibrogenesis is a common pathophysiological process seen during chronic and stress-induced accelerated cardiac aging. While adequate production of extracellular matrix (ECM) proteins is necessary for post-injury wound healing, excessive synthesis and accumulation of extracellular matrix protein in the stressed or injured hearts causes decreased or loss of lusitropy that leads to cardiac failure...
November 24, 2016: Journal of Cellular Physiology
https://www.readbyqxmd.com/read/27881157/plasma-micrornas-are-associated-with-acute-exacerbation-in-idiopathic-pulmonary-fibrosis
#13
Haiyan Min, Shanshan Fan, Shiyu Song, Yi Zhuang, Hui Li, Yongzheng Wu, Hourong Cai, Long Yi, Jinghong Dai, Qian Gao
BACKGROUND: Acute exacerbation of idiopathic pulmonary fibrosis (AE-IPF) has high short-term mortality with unknown causes. To predict this malignant condition in clinics is challenging. In this study, we aim to demonstrate whether there are miRNAs that differ between AE-IPF and stable IPF, which may be served as reliable biomarker for AE-IPF prediction. METHODS: Human fibrotic-associated miRNAs arrays were designed to detect miRNAs expression in plasma of 3 AE-IPF patients, 3 Stable-IPF (S-IPF) patients and 3 normal controls (NC)...
November 23, 2016: Diagnostic Pathology
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
#14
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
#15
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/27876248/pirfenidone-and-mortality-in-idiopathic-pulmonary-fibrosis
#16
Paolo Spagnolo
No abstract text is available yet for this article.
November 18, 2016: Lancet Respiratory Medicine
https://www.readbyqxmd.com/read/27876247/effect-of-pirfenidone-on-mortality-pooled-analyses-and-meta-analyses-of-clinical-trials-in-idiopathic-pulmonary-fibrosis
#17
Steven D Nathan, Carlo Albera, Williamson Z Bradford, Ulrich Costabel, Ian Glaspole, Marilyn K Glassberg, David R Kardatzke, Monica Daigl, Klaus-Uwe Kirchgaessler, Lisa H Lancaster, David J Lederer, Carlos A Pereira, Jeffrey J Swigris, Dominique Valeyre, Paul W Noble
BACKGROUND: In clinical trials of idiopathic pulmonary fibrosis, rates of all-cause mortality are low. Thus prospective mortality trials are logistically very challenging, justifying the use of pooled analyses or meta-analyses. We did pooled analyses and meta-analyses of clinical trials of pirfenidone versus placebo to determine the effect of pirfenidone on mortality outcomes over 120 weeks. METHODS: We did a pooled analysis of the combined patient populations of the three global randomised phase 3 trials of pirfenidone versus placebo-Clinical Studies Assessing Pirfenidone in Idiopathic Pulmonary Fibrosis: Research of Efficacy and Safety Outcomes (CAPACITY 004 and 006; trial durations 72-120 weeks) and Assessment of Pirfenidone to Confirm Efficacy and Safety in Idiopathic Pulmonary Fibrosis (ASCEND 016; 52 weeks)-for all-cause mortality, treatment-emergent all-cause mortality, idiopathic-pulmonary-fibrosis-related mortality, and treatment-emergent idiopathic-pulmonary-fibrosis-related mortality at weeks 52, 72, and 120...
November 19, 2016: Lancet Respiratory Medicine
https://www.readbyqxmd.com/read/27871724/the-palliative-care-needs-of-patients-with-idiopathic-pulmonary-fibrosis-a-qualitative-study-of-patients-and-family-caregivers
#18
Kathleen Oare Lindell, Dio Kavalieratos, Kevin F Gibson, Laura Tycon, Margaret Rosenzweig
OBJECTIVES: To explore the perceptions of palliative care (PC) needs in patients with idiopathic pulmonary fibrosis (IPF) and their caregivers. BACKGROUND: IPF carries a poor prognosis with most patients succumbing to their illness at a rate comparable to aggressive cancers. No prior studies have comprehensively explored perceptions of PC needs from those currently living with the disease, caring for someone living with the disease, and who cared for a deceased family member...
November 18, 2016: Heart & Lung: the Journal of Critical Care
https://www.readbyqxmd.com/read/27871152/human-adipose-derived-mesenchymal-stem-cells-attenuate-early-stage-of-bleomycin-induced-pulmonary-fibrosis-comparison-with-pirfenidone
#19
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
#20
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
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