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https://www.readbyqxmd.com/read/28427556/pirfenidone-for-acute-exacerbation-of-idiopathic-pulmonary-fibrosis-a%C3%A2-retrospective-study
#1
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
#2
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
#3
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...
April 12, 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
#4
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
#5
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
#6
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
#7
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
#8
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/28304388/pirfenidone-inhibits-post-traumatic-proliferative-vitreoretinopathy
#9
B N M K Khanum, R Guha, V P Sur, S Nandi, S K Basak, A Konar, S Hazra
PurposeThe purpose of the study was to evaluate the efficacy and safety of intravitreal pirfenidone for inhibition of proliferative vitreoretinopathy (PVR) in a model of penetrating ocular injury.Patients and methodsPenetrating trauma was induced on the retina of rabbit and treated either with 0.1 ml of phosphate-buffered saline (PBS) or 0.1 ml of 0.5% pirfenidone, and development of PVR was evaluated clinically and graded after 1 month. Histopathology and immunohistochemistry with transforming growth factor beta (TGFβ), alpha smooth muscle actin (αSMA), and collagen-1 were performed to assess the fibrotic changes...
March 17, 2017: Eye
https://www.readbyqxmd.com/read/28303974/antifibrotic-effect-of-pirfenidone-in-a-mouse-model-of-human-nonalcoholic-steatohepatitis
#10
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
#11
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...
March 11, 2017: Archivos de Bronconeumología
https://www.readbyqxmd.com/read/28287347/predicting-life-expectancy-for-pirfenidone-in-idiopathic-pulmonary-fibrosis
#12
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
https://www.readbyqxmd.com/read/28287346/systematic-review-and-network-meta-analysis-of-idiopathic-pulmonary-fibrosis-treatments
#13
Kelly Fleetwood, Rachael McCool, Julie Glanville, Susan C Edwards, Sandro Gsteiger, Monica Daigl, Mark Fisher
BACKGROUND: The antifibrotics pirfenidone and nintedanib are both approved for the treatment of idiopathic pulmonary fibrosis (IPF) by regulatory agencies and are recommended by health technology assessment bodies. Other treatments such as N-acetylcysteine are used in clinical practice but have not received regulatory approval. No head-to-head trials have been conducted to directly compare the efficacy of these therapies in IPF. OBJECTIVE: To compare the efficacy of treatments for IPF...
March 2017: Journal of Managed Care & Specialty Pharmacy
https://www.readbyqxmd.com/read/28285699/pirfenidone-plays-a-biphasic-role-in-inhibition-of-epithelial-mesenchymal-transition-in-non-small-cell-lung-cancer
#14
Ayako Fujiwara, Yasushi Shintani, Soichiro Funaki, Tomohiro Kawamura, Toru Kimura, Masato Minami, Meinoshin Okumura
INTRODUCTION: Epithelial to mesenchymal transition (EMT) relates to both organ fibrosis and malignant behavior of cancer. Pirfenidone (PFD) is an anti-fibrotic agent for idiopathic pulmonary fibrosis and one of its functions may be to inhibit fibrotic EMT. This study aimed to investigate the possibility that PFD might exert an anti-tumor effect through inhibition of EMT in non-small cell lung cancer (NSCLC) cell lines in vitro and in vivo. METHODS: NSCLC cells (A549, NCI-H358) were used to evaluate PFD effects on TGF-β1 induced phenotypic changes...
April 2017: Lung Cancer: Journal of the International Association for the Study of Lung Cancer
https://www.readbyqxmd.com/read/28284377/treatment-of-renal-fibrosis-turning-challenges-into-opportunities
#15
REVIEW
Barbara M Klinkhammer, Roel Goldschmeding, Jürgen Floege, Peter Boor
Current treatment modalities are not effective in halting the progression of most CKD. Renal fibrosis is a pathological process common to all CKD and thereby represents an excellent treatment target. A large number of molecular pathways involved in renal fibrosis were identified in preclinical studies, some of them being similar among different organs and some with available drugs in various phases of clinical testing. Yet only few clinical trials with antifibrotic drugs are being conducted in CKD patients...
March 2017: Advances in Chronic Kidney Disease
https://www.readbyqxmd.com/read/28268982/acoustic-evaluation-of-pirfenidone-on-patients-with-combined-pulmonary-fibrosis-emphysema-syndrome
#16
Sonia Charleston-Villalobos, Norma Castaneda-Villa, Ramon Gonzalez-Camarena, M Mejia-Avila, H Mateos-Toledo, Tomas Aljama-Corrales
The combined pulmonary fibrosis emphysema syndrome (CPFES) overall has a poor prognosis with a 5-year survival of 35-80%. Consequently, to evaluate possible positive effects on patients of novel agents as pirfenidone is relevant. However, the efficacy of pirfenidone in CPFES patients is still not well-known. In this study we propose an alternative to evaluate the effects of pirfenidone treatment on CPFES patients via acoustic information. Quantitative analysis of discontinuous adventitious lung sounds (DLS), known as crackles, has been promising to detect and characterize diverse pulmonary pathologies...
August 2016: Conference Proceedings: Annual International Conference of the IEEE Engineering in Medicine and Biology Society
https://www.readbyqxmd.com/read/28266906/pirfenidone-for-the-treatment-of-idiopathic-pulmonary-fibrosis
#17
Peter M George, Athol U Wells
Idiopathic pulmonary fibrosis (IPF) is a diffuse parenchymal lung disease with no cure. Up until recently, no treatment had been proven to alter its natural history as judged by rate of lung function decline. In 2014 however, the emergence of two novel anti-fibrotic agents, Pirfenidone and Nintedanib revolutionized the management of this condition. Both have demonstrated the ability to deliver a major reduction in the rate of chronic IPF progression. Areas Covered: This review article focuses on Pirfenidone - a pyridone derivative initially designed as an analgesic and anti-pyretic agent...
May 2017: Expert Review of Clinical Pharmacology
https://www.readbyqxmd.com/read/28261880/pirfenidone-combined-with-corticosteroids-in-a-patient-with-systemic-lupus-erythematosus-associated-interstitial-lung-disease
#18
LETTER
B-B Yang, X-Y Man, M Zheng
No abstract text is available yet for this article.
March 6, 2017: Journal of the European Academy of Dermatology and Venereology: JEADV
https://www.readbyqxmd.com/read/28257817/the-earlier-the-better-impact-of-early-diagnosis-on-clinical-outcome-in-idiopathic-pulmonary-fibrosis
#19
REVIEW
Marina Aiello, Giuseppina Bertorelli, Marialuisa Bocchino, Alfredo Chetta, Alfeo Fiore-Donati, Alessandro Fois, Stefano Marinari, Tiberio Oggionni, Biagio Polla, Elisabetta Rosi, Anna Stanziola, Francesco Varone, Alessandro Sanduzzi
BACKGROUND: Idiopathic pulmonary fibrosis (IPF) is a complex disease with a highly variable clinical course and generally poor prognosis. Classified as a rare disease, significant increases in incidence have been recorded worldwide in recent years. Left untreated IPF is extremely debilitating with substantial personal, social and economic implications. OBJECTIVES: To discuss how IPF is diagnosed and managed in real life clinical practice with particular reference to Italy and to determine how new and effective therapies can be incorporated into a patient-centred management approach in order to improve the lives of patients with IPF...
February 28, 2017: Pulmonary Pharmacology & Therapeutics
https://www.readbyqxmd.com/read/28257580/a-novel-antifibrotic-mechanism-of-nintedanib-and-pirfenidone-inhibition-of-collagen-fibril-assembly
#20
Larissa Knüppel, Yoshihiro Ishikawa, Michaela Aichler, Katharina Heinzelmann, Rudolf Hatz, Jürgen Behr, Axel Walch, Hans Peter Bächinger, Oliver Eickelberg, Claudia A Staab-Weijnitz
RATIONALE: Idiopathic pulmonary fibrosis (IPF) is characterized by excessive deposition of extracellular matrix, in particular collagens. Two IPF therapeutics, nintedanib and pirfenidone, decelerate lung function decline, but their underlying mechanisms of action are poorly understood. In this study we sought to analyze their effects on collagen synthesis and maturation at important regulatory levels. METHODS: Primary human fibroblasts from IPF patients and healthy donors were treated with nintedanib (0...
March 3, 2017: American Journal of Respiratory Cell and Molecular Biology
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