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Fibrosis AND Pirfenidone

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https://www.readbyqxmd.com/read/28544857/new-treatment-directions-for-ipf-current-status-of-ongoing-and-upcoming-clinical-trials
#1
Francesco Macagno, Francesco Varone, Paolo Maria Leone, Pier-Valerio Mari, Loredana Panico, Ludovica Berardini, Luca Richeldi
The main objective of this review is to explore the wide and expanding field of new clinical trials in IPF. Recent trials have confirmed the efficacy of the approved drugs pirfenidone and nintedanib; nonetheless, the discovery of new biological pathways has opened new horizons in this field. Areas Covered: New strategies against matrix deposition are under study and so is for the role of immunity and autoimmunity. Recent advances in the use of stem cells are opening new possibilities for the recovery of damaged lung tissues...
May 25, 2017: Expert Review of Respiratory Medicine
https://www.readbyqxmd.com/read/28508572/clinical-characteristics-and-factors-associated-with-mortality-in-idiopathic-pulmonary-fibrosis-an-experience-from-a-tertiary-care-center-in-pakistan
#2
Ali Bin Sarwar Zubairi, Huzaifa Ahmad, Maryam Hassan, Sajjad Sarwar, Aamir Abbas, Talha Shahzad, Muhammad Irfan
INTRODUCTION: Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive interstitial lung disease (ILD) that predominantly affects older adults. IPF has the highest mortality burden of all ILDs. Data on mortality in patients with IPF is limited in developing countries. OBJECTIVES: To identify factors associated with mortality in patients with IPF at a tertiary care center in Pakistan. METHODS: A retrospective chart review was conducted at the Aga Khan University Hospital (AKUH) in Karachi, Pakistan from January 2005 to December 2015...
May 15, 2017: Clinical Respiratory Journal
https://www.readbyqxmd.com/read/28489801/a-care-compliant-case-report-lung-transplantation-for-a-chinese-young-man-with-idiopathic-pleuroparenchymal-fibroelastosis
#3
Hui Huang, Ruie Feng, Shan Li, Bo Wu, Kai Xu, Zuojun Xu, Jingyu Chen
RATIONAL: Pleuroparenchymal fibroelastosis (PPFE) is a rare interstitial lung disease that is characterized radiologically by apical pleural thickening and histologically by elastic fibrosis of the visceral pleura. Although PPFE cases have been reported occasionally since this disease was initially described, most such cases have involved secondary PPFE. Idiopathic PPFE (iPPFE) cases have been less thoroughly studied. There are no effective medications for patients with iPPFE. PATIENT CONCERNS: A 34-year-old man with no asbestos or cigarette exposure was admitted to our ward due to worsening cough and exertional dyspnea for 10 years...
May 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28483105/comprehensive-and-individualized-patient-care-in-idiopathic-pulmonary-fibrosis-refining-approaches-to-diagnosis-prognosis-and-treatment
#4
Fernando J Martinez, Kevin R Flaherty
As seen in this CME online activity (available at http://courses.elseviercme.com/chest16/647e), idiopathic pulmonary fibrosis (IPF) is a specific form of chronic progressive fibrotic lung disease of unknown cause. It is the most common form of idiopathic interstitial pneumonia in adults. Its prevalence among US Medicare beneficiaries has more than doubled in the past 14 years. With the approval of two agents that reduce functional decline and disease progression, and the 2015 update of the American Thoracic Society guidelines on the treatment of IPF, the options for appropriate clinical management of the disease have become well defined...
May 2017: Chest
https://www.readbyqxmd.com/read/28472808/idiopathic-pulmonary-fibrosis-molecular-endotypes-of-fibrosis-stratifying-existing-and-emerging-therapies
#5
Daniele Magnini, Giuliano Montemurro, Bruno Iovene, Linda Tagliaboschi, Rafael Emanuele Gerardi, Erminia Lo Greco, Teresa Bruni, Alessio Fabbrizzi, Francesco Lombardi, Luca Richeldi
Idiopathic pulmonary fibrosis (IPF) is a specific form of chronic, progressive fibrosing interstitial pneumonia of unknown causes. Current diagnostic criteria are based on radiological, clinical, and histopathological features but, unfortunately, still many patients remain undiagnosed. Two currently approved therapies, pirfenidone and nintedanib, slow down disease progression but failed to block or revert it. On the other hand, many of the therapeutic agents tested in several clinical trials have not given satisfactory answers, probably due to the pathological heterogeneity of the disease...
2017: Respiration; International Review of Thoracic Diseases
https://www.readbyqxmd.com/read/28471697/pirfenidone-reduces-respiratory-related-hospitalizations-in-idiopathic-pulmonary-fibrosis
#6
Brett Ley, Jeffrey Swigris, Bann-Mo Day, John L Stauffer, Karina Raimundo, Willis Chou, Harold R Collard
RATIONALE: Respiratory-related hospitalizations in patients with idiopathic pulmonary fibrosis (IPF) are more frequent than acute IPF exacerbations and are associated with poor outcomes. OBJECTIVES: To compare the risk of non-elective hospitalization by type (all-cause, respiratory-related, non-respiratory related) and death after hospitalization for pirfenidone versus placebo over 52 weeks from three phase 3 IPF clinical trials. METHODS: Individual patient data was pooled from three phase 3 randomized, placebo-controlled studies of pirfenidone for IPF (CAPACITY and ASCEND) including all patients randomized to pirfenidone 2403 mg/day (n=623) or placebo (n=624)...
May 4, 2017: American Journal of Respiratory and Critical Care Medicine
https://www.readbyqxmd.com/read/28445938/pirfenidone-normalizes-the-tumor-microenvironment-to-improve-chemotherapy
#7
Christiana Polydorou, Fotios Mpekris, Panagiotis Papageorgis, Chrysovalantis Voutouri, Triantafyllos Stylianopoulos
Normalization of the tumor microenvironment by selectively targeting components of the tumor extracellular matrix has been recently proposed to have the potential to decompress tumor blood vessels, increase vessel perfusion and thus, improve drug delivery and the efficacy of cancer therapy. Therefore, we now need to identify safe and well tolerated pharmaceutical agents that are able to remodel the microenvironment of solid tumors and enhance chemotherapy. In this study, we repurposed Pirfenidone, a clinically approved anti-fibrotic drug for the treatment of idiopathic pulmonary fibrosis, to investigate its possible role on tumor microenvironment normalization...
April 11, 2017: Oncotarget
https://www.readbyqxmd.com/read/28440554/tolerability-of-treatment-with-pirfenidone-or-nintedanib-for-pulmonary-fibrosis-in-the-real-world
#8
EDITORIAL
Takashi Ogura, Hideya Kitamura
No abstract text is available yet for this article.
April 25, 2017: Respirology: Official Journal of the Asian Pacific Society of Respirology
https://www.readbyqxmd.com/read/28435277/role-of-pirfenidone-in-the-management-of-pulmonary-fibrosis
#9
REVIEW
Keith C Meyer, Catherine A Decker
Pulmonary fibrosis is associated with a number of specific forms of interstitial lung disease (ILD) and can lead to progressive decline in lung function, poor quality of life, and, ultimately, early death. Idiopathic pulmonary fibrosis (IPF), the most common fibrotic ILD, affects up to 1 in 200 elderly individuals and has a median survival that ranges from 3 to 5 years following initial diagnosis. IPF has not been shown to respond to immunomodulatory therapies, but recent trials with novel antifibrotic agents have demonstrated lessening of lung function decline over time...
2017: Therapeutics and Clinical Risk Management
https://www.readbyqxmd.com/read/28427556/pirfenidone-for-acute-exacerbation-of-idiopathic-pulmonary-fibrosis-a%C3%A2-retrospective-study
#10
Kenta Furuya, Susumu Sakamoto, Hiroshige Shimizu, Muneyuki Sekiya, Arisa Kinoshita, Takuma Isshiki, Keishi Sugino, Keiko Matsumoto, Sakae Homma
BACKGROUND: Acute exacerbation of idiopathic pulmonary fibrosis (AE-IPF) is a rapid and ultimately fatal condition, and no effective treatment has been established. Pirfenidone has antifibrotic effects in IPF; however, its efficacy for AE-IPF is unclear. OBJECTIVES: To evaluate the efficacy of pirfenidone for AE-IPF. METHODS: We retrospectively reviewed the medical records of 135 IPF patients treated during the period from April 2008 to April 2015 and identified and extracted 47 AE-IPF patients (42 men, 5 women; mean age, 73...
May 2017: Respiratory Medicine
https://www.readbyqxmd.com/read/28420366/pirfenidone-attenuates-bleomycin-induced-pulmonary-fibrosis-in-mice-by-regulating-nrf2-bach1-equilibrium
#11
Yuan Liu, Fuai Lu, Lirong Kang, Zhihua Wang, Yongfu Wang
BACKGROUND: Oxidative stress is one of the important factors involved in the pathogenesis of idiopathic pulmonary fibrosis (IPF). The equilibrium of Nuclear factor-erythroid-related factor 2 (Nrf2)/[BTB (broad-complex, tramtrack and bric-a-brac) and CNC (cap'n'collar protein) homology 1, Bach1] determines the expression level of antioxidant factors, further regulating the function of oxidation/antioxidation capacity. Pirfenidone (PFD) is one of two currently for IPF therapy approved drugs...
April 18, 2017: BMC Pulmonary Medicine
https://www.readbyqxmd.com/read/28399537/antacid-therapy-and-disease-progression-in-patients-with-idiopathic-pulmonary-fibrosis-who-received-pirfenidone
#12
Michael Kreuter, Paolo Spagnolo, Wim Wuyts, Elisabetta Renzoni, Dirk Koschel, Francesco Bonella, Toby M Maher, Martin Kolb, Derek Weycker, Klaus-Uwe Kirchgässler, Ulrich Costabel
BACKGROUND: Gastroesophageal reflux disease is a potential risk factor for idiopathic pulmonary fibrosis (IPF) progression; however, the impact of antacid therapy (AAT) is under debate. OBJECTIVE: To evaluate the effect of AAT on IPF progression in pirfenidone-treated patients. METHODS: This post hoc analysis included patients with IPF who received pirfenidone in 3 trials (CAPACITY [PIPF-004/PIPF-006] and ASCEND [PIPF-016]). Pulmonary function, exercise tolerance, survival, hospitalizations, and adverse events (AEs) over 52 weeks were analyzed by baseline AAT use...
2017: Respiration; International Review of Thoracic Diseases
https://www.readbyqxmd.com/read/28388260/acute-exacerbation-and-decline-in-forced-vital-capacity-are-associated-with-increased-mortality-in-idiopathic-pulmonary-fibrosis
#13
Miya O Paterniti, Youwei Bi, Dinko Rekić, Yaning Wang, Banu A Karimi-Shah, Badrul A Chowdhury
RATIONALE: Exploration of forced vital capacity (FVC) as it relates to mortality in idiopathic pulmonary fibrosis (IPF), a chronic, progressive, and ultimately fatal parenchymal lung disease, is important both clinically and to the current drug development paradigm. We evaluated the association between FVC decline and mortality in the largest, well-characterized placebo cohort to date. Additionally, we sought to explore the risk of death from acute exacerbations and further validate previously identified baseline predictors of mortality...
April 7, 2017: Annals of the American Thoracic Society
https://www.readbyqxmd.com/read/28357591/development-and-pretesting-of-a-questionnaire-to-assess-patient-experiences-and-satisfaction-with-medications-pesam-questionnaire
#14
Merel L Kimman, Adrienne H Rotteveel, Marlies Wijsenbeek, Rémy Mostard, Nelleke C Tak, Xana van Jaarsveld, Marjolein Storm, Kioa L Wijnsma, Marielle Gelens, Nicole C A J van de Kar, Jack Wetzels, Carmen D Dirksen
BACKGROUND: The aim of this study was to develop, together with the Lung Foundation Netherlands and Dutch Kidney Patients Association, patients and clinicians, a measure to evaluate patient experiences with the orphan drugs pirfenidone (for idiopathic pulmonary fibrosis [IPF]) and eculizumab (for atypical haemolytic uraemic syndrome [aHUS]), as well as a generic measure of patient experiences and satisfaction with medications. METHODS: Development of the Patient Experiences and Satisfaction with Medications (PESaM) questionnaire consisted of four phases: literature review (phase I); focus groups and individual patient interviews (phase II); item generation (phase III); and face and content validity testing (phase IV)...
March 29, 2017: Patient
https://www.readbyqxmd.com/read/28353114/blood-biomarkers-in-idiopathic-pulmonary-fibrosis
#15
REVIEW
Julien Guiot, Catherine Moermans, Monique Henket, Jean-Louis Corhay, Renaud Louis
PURPOSE: Idiopathic pulmonary fibrosis (IPF) is a progressive and lethal lung disease of unknown origin whose incidence has been increasing over the latest decade partly as a consequence of population ageing. New anti-fibrotic therapy including pirfenidone and nintedanib have now proven efficacy in slowing down the disease. Nevertheless, diagnosis and follow-up of IPF remain challenging. METHODS: This review examines the recent literature on potentially useful blood molecular and cellular biomarkers in IPF...
March 28, 2017: Lung
https://www.readbyqxmd.com/read/28345369/pulmonary-fibrosis-part-ii-state-of-the-art-patient-management
#16
Keith C Meyer
While many pharmacologic therapies for the treatment of idiopathic pulmonary fibrosis (IPF) have been evaluated via randomized, placebo-controlled clinical trials (RCTs) conducted over the past two decades, most therapies have been shown to be ineffective or even potentially harmful. However, a number of recently completed RCTs have shown significant efficacy for pirfenidone and nintedanib for the treatment of IPF. Areas covered: This manuscript reviews recent advances in the management of IPF and other forms of fibrosing interstitial lung disease (ILD) with an emphasis on IPF...
May 2017: Expert Review of Respiratory Medicine
https://www.readbyqxmd.com/read/28317233/pirfenidone-and-nintedanib-for-pulmonary-fibrosis-in-clinical-practice-tolerability-and-adverse-drug-reactions
#17
Jonathan A Galli, Aloknath Pandya, Michelle Vega-Olivo, Chandra Dass, Huaqing Zhao, Gerard J Criner
BACKGROUND AND OBJECTIVE: The real-world tolerability of pirfenidone and nintedanib in non-clinical trial patients is unknown. Many patients with pulmonary fibrosis have significant medical co-morbidities or baseline characteristics that exclude them from clinical trial participation. METHODS: We conducted a retrospective chart review study on subjects prescribed nintedanib or pirfenidone for pulmonary fibrosis treatment (any aetiology) from September 2014 to February 2016...
March 20, 2017: Respirology: Official Journal of the Asian Pacific Society of Respirology
https://www.readbyqxmd.com/read/28303974/antifibrotic-effect-of-pirfenidone-in-a-mouse-model-of-human-nonalcoholic-steatohepatitis
#18
Chikara Komiya, Miyako Tanaka, Kyoichiro Tsuchiya, Noriko Shimazu, Kentaro Mori, Shunsaku Furuke, Yasutaka Miyachi, Kumiko Shiba, Shinobu Yamaguchi, Kenji Ikeda, Kozue Ochi, Kazuhiko Nakabayashi, Ken-Ichiro Hata, Michiko Itoh, Takayoshi Suganami, Yoshihiro Ogawa
Non-alcoholic steatohepatitis (NASH) is characterized by steatosis with lobular inflammation and hepatocyte injury. Pirfenidone (PFD) is an orally bioavailable pyridone derivative that has been clinically used for the treatment of idiopathic pulmonary fibrosis. However, it remains unknown whether PFD improves liver fibrosis in a mouse model with human NASH-like phenotypes. In this study, we employed melanocortin 4 receptor-deficient (MC4R-KO) mice as a mouse model with human NASH-like phenotypes to elucidate the effect and action mechanisms of PFD on the development of NASH...
March 17, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28292522/guidelines-for-the-medical-treatment-of-idiopathic-pulmonary-fibrosis
#19
Antoni Xaubet, María Molina-Molina, Orlando Acosta, Elena Bollo, Diego Castillo, Estrella Fernández-Fabrellas, José Antonio Rodríguez-Portal, Claudia Valenzuela, Julio Ancochea
Idiopathic pulmonary fibrosis is defined as chronic fibrosing interstitial pneumonia limited to the lung, with poor prognosis. The incidence has been rising in recent years probably due to improved diagnostic methods and increased life expectancy. In 2013, the SEPAR guidelines for the diagnosis and treatment for idiopathic pulmonary fibrosis were published. Since then, clinical trials and meta-analyses have shown strong scientific evidence for the use of pirfenidone and nintedanib in the treatment of idiopathic pulmonary fibrosis...
May 2017: Archivos de Bronconeumología
https://www.readbyqxmd.com/read/28287347/predicting-life-expectancy-for-pirfenidone-in-idiopathic-pulmonary-fibrosis
#20
Mark Fisher, Steven D Nathan, Christian Hill, Jade Marshall, Fred Dejonckheere, Per-Olof Thuresson, Toby M Maher
BACKGROUND: Conducting an adequately powered survival study in idiopathic pulmonary fibrosis (IPF) is challenging due to the rare nature of the disease and the need for extended follow-up. Consequently, registration trials of IPF treatments have not been designed to estimate long-term survival. OBJECTIVE: To predict life expectancy for patients with IPF receiving pirfenidone versus best supportive care (BSC) in a population that met the inclusion criteria of patients enrolled in the ASCEND and CAPACITY trials...
March 2017: Journal of Managed Care & Specialty Pharmacy
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