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https://www.readbyqxmd.com/read/28079978/targeting-coagulation-factor-receptors-protease-activated-receptors-in-idiopathic-pulmonary-fibrosis
#1
REVIEW
Cong Lin, Keren Borensztajn, C Arnold Spek
Idiopathic pulmonary fibrosis (IPF) is a lethal lung disease with a 5-year mortality rate above 50% and unknown etiology. Treatment options remain limited and, currently, only two drugs are available, nintedanib and pirfenidone. However, both of these antifibrotic agents only slow down the progression of the disease but do not remarkably prolong the survival of IPF patients. Hence, the discovery of new therapeutic targets for IPF is crucial. Studies exploring the mechanisms that are involved in IPF identified several possible targets for therapeutic interventions...
January 12, 2017: Journal of Thrombosis and Haemostasis: JTH
https://www.readbyqxmd.com/read/28054535/combined-prednisolone-and-pirfenidone-in-bleomycin-induced-lung-disease
#2
Preyas J Vaidya, H S Sandeepa, Tejinder Singh, S K Susheel Kumar, Rajat Bhargava, Gopal Ramakrishnan, Prashant N Chhajed
Bleomycin is a cytostatic drug commonly employed in the treatment of Hodgkin's disease, seminomas, and choriocarcinoma. Bleomycin may induce a chronic pulmonary inflammation that may progress to fibrosis. So far, only corticosteroids have been used in the treatment of bleomycin-induced lung disease with variable results. Pirfenidone is an antifibrotic drug that has been approved for the treatment of idiopathic pulmonary fibrosis. We report two cases of bleomycin-induced lung disease treated successfully with pirfenidone and oral corticosteroids...
July 2016: Journal of Cancer Research and Therapeutics
https://www.readbyqxmd.com/read/28050003/effectiveness-of-combined-therapy-with-pirfenidone-and-erythromycin-for-unclassifiable-interstitial-pneumonia-induced-by-htlv-1-associated-bronchioloalveolar-disorder-haba
#3
Naoko Yokohori, Akitoshi Sato, Mizue Hasegawa, Hideki Katsura, Kenzo Hiroshima, Tamiko Takemura
Human T-cell lymphotropic virus type 1 (HTLV-1) is a retrovirus involved in the pathogenesis of adult T-cell leukemia (ATL) and HTVL-1-associated bronchioloalveolar disorder (HABA). The clinical and pathological findings of HABA have been characterized as either a diffuse panbronchiolitis (DPB) pattern or idiopathic interstitial pneumonia (IIP) pattern. Treatments for HABA include corticosteroids for the IIP pattern and erythromycin for the DPB pattern. We herein report a case of HABA-associated unclassifiable interstitial pneumonia that improved with combined therapy with pirfenidone and erythromycin...
2017: Internal Medicine
https://www.readbyqxmd.com/read/28039616/a-cost-effectiveness-analysis-of-nintedanib-in-idiopathic-pulmonary-fibrosis-in-the-uk
#4
C Rinciog, M Watkins, S Chang, T M Maher, C LeReun, D Esser, A Diamantopoulos
BACKGROUND: International guidelines recommend nintedanib (OFEV(®)) as an option for the treatment of idiopathic pulmonary fibrosis (IPF). OBJECTIVE: The objective of this study was to assess the cost effectiveness of nintedanib versus pirfenidone, N-acetylcysteine and best supportive care (BSC) for the treatment of IPF from a UK payer's perspective. METHODS: A Markov model was designed to capture the changes in the condition of adults with IPF...
December 31, 2016: PharmacoEconomics
https://www.readbyqxmd.com/read/28035951/a-systematic-review-of-the-role-of-dysfunctional-wound-healing-in-the-pathogenesis-and-treatment-of-idiopathic-pulmonary-fibrosis
#5
REVIEW
Alan Betensley, Rabab Sharif, Dimitrios Karamichos
Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive interstitial lung disorder showcasing an interaction between genetic predisposition and environmental risks. This usually involves the coaction of a mixture of cell types associated with abnormal wound healing, leading to structural distortion and loss of gas exchange function. IPF bears fatal prognosis due to respiratory failure, revealing a median survival of approximately 2 to 3 years. This review showcases the ongoing progress in understanding the complex pathophysiology of IPF and it highlights the latest potential clinical treatments...
December 26, 2016: Journal of Clinical Medicine
https://www.readbyqxmd.com/read/28012488/prognostic-value-of-serial-serum-kl-6-measurements-in-patients-with-idiopathic-pulmonary-fibrosis
#6
Kentaro Wakamatsu, Nobuhiko Nagata, Hiroyuki Kumazoe, Keishi Oda, Hiroshi Ishimoto, Michihiro Yoshimi, Shohei Takata, Minako Hamada, Yoshifusa Koreeda, Kouji Takakura, Miwa Ishizu, Makiko Hara, Shinji Ise, Miiru Izumi, Takashi Akasaki, Sanae Maki, Masaharu Kawabata, Hiroshi Mukae, Masayuki Kawasaki
BACKGROUND: The prognostic significance of serial measurements of serum KL-6 levels in patients with idiopathic pulmonary fibrosis (IPF) is unclear; hence, it was assessed in this study. METHODS: Medical records of 66 patients with IPF, who were not treated with pirfenidone prior to enrollment, were retrospectively reviewed for information on clinical progress, forced vital capacity (FVC), survival, and serum KL-6 levels. We assessed initial serum levels of KL-6, serial changes in serum KL-6 levels, yearly decline in FVC (ΔFVC), and the rate of decline (%ΔFVC)...
January 2017: Respiratory Investigation
https://www.readbyqxmd.com/read/27971558/cost-effectiveness-analysis-of-pirfenidone-for-the-treatment-of-mild-to-moderate-idiopathic-pulmonary-fibrosis-ipf-compared-to-best-supportive-care-and-nintedanib-from-the-italian-nhs-perspective
#7
R Ravasio, M Ferrario, M Pata, P Thuresson
No abstract text is available yet for this article.
November 2016: Value in Health: the Journal of the International Society for Pharmacoeconomics and Outcomes Research
https://www.readbyqxmd.com/read/27939076/efficacy-of-simtuzumab-versus-placebo-in-patients-with-idiopathic-pulmonary-fibrosis-a-randomised-double-blind-controlled-phase-2-trial
#8
Ganesh Raghu, Kevin K Brown, Harold R Collard, Vincent Cottin, Kevin F Gibson, Robert J Kaner, David J Lederer, Fernando J Martinez, Paul W Noble, Jin Woo Song, Athol U Wells, Timothy P M Whelan, Wim Wuyts, Emmanuel Moreau, Scott D Patterson, Victoria Smith, Selina Bayly, Jason W Chien, Qi Gong, Jenny J Zhang, Thomas G O'Riordan
BACKGROUND: Lysyl oxidase-like 2 (LOXL2) catalyses collagen cross-linking and is implicated in the pathogenesis of idiopathic pulmonary fibrosis (IPF). The aim of this study was to investigate the efficacy and safety of simtuzumab, a monoclonal antibody against LOXL2, in patients with IPF. METHODS: In this randomised, double-blind, phase 2 trial, we recruited patients aged 45-85 years with definite IPF diagnosed prior to 3 years of screening from 183 hospitals and respiratory clinics in 14 countries...
January 2017: Lancet Respiratory Medicine
https://www.readbyqxmd.com/read/27937011/effects-and-mechanisms-of-pirfenidone-prednisone-and-acetylcysteine-on-pulmonary-fibrosis-in-rat-idiopathic-pulmonary-fibrosis-models
#9
Wencheng Yu, Fang Guo, Xiaoxia Song
CONTEXT: Previous studies have reported that caveolin-1 (Cav-1) is associated with lung fibrosis. However, the role of Cav-1 expression in pirfenidone-treated idiopathic pulmonary fibrosis (IPF) is unknown. OBJECTIVE: This study investigated Cav-1 expression in pirfenidone-treated IPF, and compared the effects of pirfenidone with acetylcysteine and prednisone on IPF. MATERIALS AND METHODS: Rat IPF model was established by endotracheal injection of 5 mg/kg bleomycin A5 into the specific pathogen-free Wistar male rats...
December 2017: Pharmaceutical Biology
https://www.readbyqxmd.com/read/27886639/hyaluronan-derived-swelling-of-solid-tumors-the-contribution-of-collagen-and-cancer-cells-and-implications-for-cancer-therapy
#10
Chrysovalantis Voutouri, Christiana Polydorou, Panagiotis Papageorgis, Vasiliki Gkretsi, Triantafyllos Stylianopoulos
Despite the important role that mechanical forces play in tumor growth and therapy, the contribution of swelling to tumor mechanopathology remains unexplored. Tumors rich in hyaluronan exhibit a highly negative fixed charge density. Repulsive forces among these negative charges as well as swelling of cancer cells due to regulation of intracellular tonicity can cause tumor swelling and development of stress that might compress blood vessels, compromising tumor perfusion and drug delivery. Here, we designed an experimental strategy, using four orthotopic tumor models, to measure swelling stress and related swelling to extracellular matrix components, hyaluronan and collagen, as well as to tumor perfusion...
December 2016: Neoplasia: An International Journal for Oncology Research
https://www.readbyqxmd.com/read/27876248/pirfenidone-and-mortality-in-idiopathic-pulmonary-fibrosis
#11
Paolo Spagnolo
No abstract text is available yet for this article.
January 2017: 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
#12
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...
January 2017: Lancet Respiratory Medicine
https://www.readbyqxmd.com/read/27871152/human-adipose-derived-mesenchymal-stem-cells-attenuate-early-stage-of-bleomycin-induced-pulmonary-fibrosis-comparison-with-pirfenidone
#13
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/27863518/safety-and-efficacy-of-bridging-to-lung-transplantation-with-antifibrotic-drugs-in-idiopathic-pulmonary-fibrosis-a-case-series
#14
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/27862475/treatment-of-idiopathic-pulmonary-fibrosis-a-position-paper-from-a-nordic-expert-group
#15
REVIEW
C M Sköld, E Bendstrup, M Myllärniemi, G Gudmundsson, T Sjåheim, O Hilberg, A Altraja, R Kaarteenaho, G Ferrara
Idiopathic pulmonary fibrosis (IPF) is a fatal progressive lung disease occurring in adults. In the last decade, the results of a number of clinical trials based on the updated disease classification have been published. The registration of pirfenidone and nintedanib, the first two pharmacological treatment options approved for IPF, marks a new chapter in the management of patients with this disease. Other nonpharmacological treatments such as lung transplantation, rehabilitation and palliation have also been shown to be beneficial for these patients...
November 13, 2016: Journal of Internal Medicine
https://www.readbyqxmd.com/read/27858160/assessing-the-therapeutic-response-to-pirfenidone-in-idiopathic-pulmonary-fibrosis-can-we-do-better-than-with-forced-vital-capacity-alone
#16
Karishma Hosein, Jamie Le, Marco Mura
New anti-fibrotic agents for idiopathic pulmonary fibrosis (IPF) were approved based on the results of forced vital capacity (FVC) trends, although concerns were raised about the reliability of FVC as the only endpoint parameter. We hypothesized that IPF-specific multi-dimensional scores (Composite Physiologic Index-CPI; gender-age-physiology-GAP; risk stratification score-RISE) would better capture response to therapy. In this pilot study, treated and untreated cohorts of IPF patients, matched for demographic and functional characteristics were prospectively followed for 1 year, at 4-month intervals...
November 17, 2016: Lung
https://www.readbyqxmd.com/read/27835939/anti-fibrotic-action-of-pirfenidone-in-dupuytren-s-disease-derived-fibroblasts
#17
Chaoming Zhou, Fang Liu, Phillip H Gallo, Mark E Baratz, Sandeep Kathju, Latha Satish
BACKGROUND: Dupuytren's disease (DD) is a complex fibro-proliferative disorder of the hand that is often progressive and eventually can cause contractures of the affected fingers. Transforming growth factor beta (TGF-β1) has been implicated as a key stimulator of myofibroblast activity and fascial contraction in DD. Pirfenidone (PFD) is an active small molecule shown to inhibit TGF-β1-mediated action in other fibrotic disorders. This study investigates the efficacy of PFD in vitro in inhibiting TGF-β1-mediated cellular functions leading to Dupuytren's fibrosis...
November 11, 2016: BMC Musculoskeletal Disorders
https://www.readbyqxmd.com/read/27821905/combined-effects-of-drugs-and-plasticizers-on-the-properties-of-drug-delivery-films
#18
Cheryl L Jennings, Thomas D Dziubla, David A Puleo
Formation of scar tissue may be reduced or prevented if wounds were locally treated with a combination of molecules tuned to the different healing phases, guiding tissue regeneration along a scar free path. To this end, drug delivery devices made of cellulose acetate phthalate and Pluronic F-127 were loaded with either quercetin or pirfenidone and plasticized with either triethyl citrate (TEC) or tributyl citrate (TBC). Quercetin inhibits oxidative stress, and pirfenidone has been shown to reduce production of pro-inflammatory and fibrogenic molecules...
July 2016: Journal of Bioactive and Compatible Polymers
https://www.readbyqxmd.com/read/27812432/treatment-of-rapidly-progressive-systemic-sclerosis-current-and-futures-perspectives
#19
Fabian A Mendoza, Maryah Mansoor, Sergio A Jimenez
INTRODUCTION: Systemic Sclerosis (SSc) is a systemic autoimmune disease characterized by severe and often progressive cutaneous, pulmonary, cardiac and gastrointestinal tract fibrosis, cellular and humoral immunologic alterations, and pronounced fibroproliferative vasculopathy. There is no effective SSc disease modifying therapy. Patients with rapidly progressive SSc have poor prognosis with frequent disability and very high mortality. AREAS COVERED: This paper reviews currently available therapeutic approaches for rapidly progressive SSc and discuss novel drugs under study for SSc disease modification...
2016: Expert Opinion on Orphan Drugs
https://www.readbyqxmd.com/read/27788604/pirfenidone-exerts-a-suppressive-effect-on-ccl18-expression-in-u937-derived-macrophages-partly-by-inhibiting-stat6-phosphorylation
#20
Yoshinobu Saito, Arata Azuma, Kuniko Matsuda, Koichiro Kamio, Shinji Abe, Akihiko Gemma
CONTEXT: CC chemokine ligand 18 (CCL18) is suggested to play a role in the development of pulmonary fibrosis. Macrophages are thought to be the main source of CCL18, and the effect of pirfenidone, an anti-fibrotic agent for idiopathic pulmonary fibrosis, on the expression of CCL18 in macrophages warrants investigation. OBJECTIVE: The purpose of this study was to investigate the effect of pirfenidone on the expression of CCL18 in macrophages. MATERIALS AND METHODS: U937 cells were differentiated into macrophages by phorbol myristate acetate and then stimulated with recombinant IL-4 to induce the production of CCL18...
October 27, 2016: Immunopharmacology and Immunotoxicology
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