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Fibrosis AND Pirfenidone

K P Suraj, Neethu K Kumar, E Jyothi, Kiran Vishnu Narayan, G Biju
BACKGROUND: But so far there is no proven pharmacological treatment for Idiopathic pulmonary fibrosis (IPF). As trials investigating different agents with different mechanisms of actions are going on, encouraging results have led to the licensing of the first IPF-specific drug, Pirfenidone. OBJECTIVE: To assess the proportion of IPF among interstitial lung disease patients and to assess their treatment response to Pirfenidone. MATERIAL AND METHODS: All consecutive patients attending the outpatient department from 1st January 2012 to 30th June 2012 with a proven diagnosis of Interstitial lung Disease (ILD) were included in this longitudinal cohort study...
May 2016: Journal of the Association of Physicians of India
Pinar Solmaz Hasdemir, Mahmud Ozkut, Tevfik Guvenal, Melis Aylin Uner, Esat Calik, Semra Oruc Koltan, Faik Mumtaz Koyuncu, Kemal Ozbilgin
AIM: To study the efficacy of pirfenidone for prevention of postoperative adhesion formation in an adhesion rat model. MATERIALS AND METHODS: Eighteen female Wistar rats were subjected to right-sided parietal peritoneum and right uterine horn adhesion model. Rats were randomized into three groups: group 1 (control) (closure of midline abdominal incision without any agent administration), group 2 (closure of incision after intraperitoneal administration of pirfenidone), and group 3 (closure of incision and only oral administration of pirfenidone for 14 days)...
August 15, 2016: Journal of Investigative Surgery: the Official Journal of the Academy of Surgical Research
Michael Kreuter, Francesco Bonella, Toby M Maher, Ulrich Costabel, Paolo Spagnolo, Derek Weycker, Klaus-Uwe Kirchgaessler, Martin Kolb
BACKGROUND: Data are conflicting regarding the possible effects of statins in patients with idiopathic pulmonary fibrosis (IPF). This post hoc analysis assessed the effects of statin therapy on disease-related outcomes in IPF. METHODS: Patients randomised to placebo (n=624) in three controlled trials of pirfenidone in IPF (CAPACITY 004 and 006, ASCEND) were categorised by baseline statin use. Outcomes assessed during the 1-year follow-up included disease progression, mortality, hospitalisation and composite outcomes of death or ≥10% absolute decline in FVC and death or ≥50 m decline in 6-minute walk distance (6MWD)...
October 5, 2016: Thorax
Bintou A Ahidjo, Mariama C Maiga, Elizabeth A Ihms, Mamoudou Maiga, Alvaro A Ordonez, Laurene S Cheung, Sarah Beck, Bruno B Andrade, Sanjay Jain, William R Bishai
Pirfenidone is a recently approved antifibrotic drug for the treatment of idiopathic pulmonary fibrosis (IPF). Because tuberculosis (TB) is characterized by granulomatous inflammation in conjunction with parenchymal destruction and replacement fibrosis, we sought to determine whether the addition of pirfenidone as an adjunctive, host-directed therapy provides a beneficial effect during antimicrobial treatment of TB. We hypothesized that pirfenidone's antiinflammatory and antifibrotic properties would reduce inflammatory lung damage and increase antimicrobial drug penetration in granulomas to accelerate treatment response...
September 8, 2016: JCI Insight
Goran Nadir Salih, Saher Burhan Shaker, Helle Dall Madsen, Elisabeth Bendstrup
BACKGROUND: Pirfenidone was approved by the European Medicines Agency and introduced in most European countries in 2011 for treatment of idiopathic pulmonary fibrosis (IPF). OBJECTIVE: To describe the national Danish experiences of pirfenidone treatment for IPF during 30 months with respect to target population, safety, adherence to the treatment and effect analysis in a well-characterised IPF population in a real-life setting. METHODS: Retrospective data collection from medical records of all patients in Denmark with IPF from 2011 to 2014...
2016: European Clinical Respiratory Journal
Deborah L Clarke, Lynne A Murray, Bruno Crestani, Matthew A Sleeman
Idiopathic pulmonary fibrosis (IPF) is a chronic fibrosing interstitial pneumonia of unknown cause, characterised by progressive worsening in lung function and dyspnoea with an associated prognosis similar to or worse than many cancers. As a better understanding emerges around the pathogenesis and mechanisms driving disease pathology, a host of novel agents are being tested both pre-clinically and clinically. However even with this deeper understanding and positive pre-clinical supportive data, negative trial outcomes are frequently reported, highlighting the problems faced in treating such a heterogeneous disease with a varied clinical course...
September 13, 2016: Pharmacology & Therapeutics
Gareth Hughes, Hannah Toellner, Helen Morris, Colm Leonard, Nazia Chaudhuri
Idiopathic Pulmonary Fibrosis (IPF) now has two licensed treatments available. Pirfenidone was the first drug to be licensed and approved for use, followed by nintedanib. We set out our real world experience with these agents in terms of their adverse events profile outside the restrictions of a clinical trial. We have demonstrated in the real world setting, that side effects are common and predominantly gastrointestinal with both therapies. Our study shows that the side effects can be effectively managed in the majority of patients with an acceptable discontinuation rate similar to that seen in the clinical trials...
September 2, 2016: Journal of Clinical Medicine
H Coleman Robinson
Restrictive lung diseases are a heterogeneous group of conditions characterized by a restrictive pattern on spirometry and confirmed by a reduction in total lung volume. Patients with more severe symptoms may have a reduced diffusing capacity of the lung for carbon monoxide. Etiologies can be intrinsic with lung parenchymal involvement, as in interstitial lung diseases, or extrinsic to the lung, as in obesity and neuromuscular disorders. Idiopathic pulmonary fibrosis is a chronic progressive interstitial pneumonia with fibrosis for which treatment is primarily supportive with oxygen therapy, pulmonary rehabilitation, and management of comorbid conditions...
September 2016: FP Essentials
Masashi Bando
Pirfenidone is an oral drug that exerts not only anti-fibrotic activity but also pleiotropic effects, such as anti-inflammatory and anti-oxidative effects. Because it suppresses reduction in vital capacity and improves progression-free survival, it was approved in October 2008 in Japan for the first time in the world as an anti-fibrotic agent for treatment of idiopathic pulmonary fibrosis (IPF). In October 2014, the agent was approved in the U.S., based on the results of the ASCEND study. Today, it is commercially available in 38 countries worldwide...
September 2016: Respiratory Investigation
Yan Xie, Haihong Jiang, Qian Zhang, Suneet Mehrotra, Peter W Abel, Myron L Toews, Dennis W Wolff, Stephen Rennard, Reynold A Panettieri, Thomas B Casale, Yaping Tu
BACKGROUND: Pirfenidone was recently approved for treatment of idiopathic pulmonary fibrosis. However, the therapeutic dose of pirfenidone is very high, causing side effects that limit its doses and therapeutic effectiveness. Understanding the molecular mechanisms of action of pirfenidone could improve its safety and efficacy. Because activated fibroblasts are critical effector cells associated with the progression of fibrosis, this study investigated the genes that change expression rapidly in response to pirfenidone treatment of pulmonary fibroblasts and explored their contributions to the anti-fibrotic effects of pirfenidone...
2016: Respiratory Research
Takekazu Iwata, Shigetoshi Yoshida, Taiki Fujiwara, Hironobu Wada, Takahiro Nakajima, Hidemi Suzuki, Ichiro Yoshino
BACKGROUND: Acute exacerbation of idiopathic pulmonary fibrosis (AE-IPF) is a life-threatening complication of lung cancer operation for patients with IPF, and no effective prophylaxis has ever been reported. In this study, we investigate the effect of perioperative treatment with an anti-IPF agent on reduction of the risk of developing AE-IPF. METHODS: A consecutive series of 50 lung cancer patients with IPF who underwent operations at our institution from October 2006 to October 2014 was retrospectively investigated...
August 18, 2016: Annals of Thoracic Surgery
Francesco Bonella, Michael Kreuter, Lars Hagmeyer, Claus Neurohr, Claus Keller, Martin J Kohlhaeufl, Joachim Müller-Quernheim, Katrin Milger, Antje Prasse
BACKGROUND: Nintedanib is approved for the treatment of idiopathic pulmonary fibrosis (IPF) and has been shown to slow disease progression by reducing annual lung function decline. OBJECTIVE: To evaluate the results of a large cohort of IPF patients treated with nintedanib within a compassionate use program (CUP) in Germany (9 centers). METHODS: Patients (≥40 years) were required to have a confirmed diagnosis of IPF, a forced vital capacity (FVC) ≥50% predicted (pred...
2016: Respiration; International Review of Thoracic Diseases
Sophie Fletcher, Mark G Jones, Katherine Spinks, Giacomo Sgalla, Ben G Marshall, Rachel Limbrey, Luca Richeldi
INTRODUCTION: The management of idiopathic pulmonary fibrosis (IPF) has been transformed by the recent approval of two anti-fibrotic drugs, nintedanib and pirfenidone. An increasing number of patients with IPF are receiving treatment with these novel therapies, and the risk of adverse events that may be associated with their use must be carefully evaluated. AREAS COVERED: Safety data about nintedanib and pirfenidone is critically evaluated, including data from randomized clinical trials and post-marketing reports...
August 17, 2016: Expert Opinion on Drug Safety
Glenn W Vicary, Yeidyly Vergne, Alberto Santiago-Cornier, Lisa R Young, Jesse Roman
Hermansky-Pudlak syndrome (HPS) is a rare autosomal recessive genetic disorder characterized by oculocutaneous albinism and a bleeding diathesis due to platelet dysfunction. More than 50% of cases worldwide are diagnosed on the Caribbean island of Puerto Rico. Genetic testing plays a growing role in diagnosis; however, not all patients with HPS have identified genetic mutations. In Puerto Rico, patients with HPS are often identified shortly after birth by their albinism, although the degree of hypopigmentation is highly variable...
October 2016: Annals of the American Thoracic Society
Ayodeji Adegunsoye, Mary E Strek
Among the interstitial lung diseases (ILD), idiopathic pulmonary fibrosis (IPF), chronic hypersensitivity pneumonitis and fibrotic connective tissue disease related ILD are associated with a worse prognosis with death occurring both from respiratory failure and serious associated co-morbidities. The recent development and approval of the antifibrotic agents nintedanib and pirfenidone, both of which reduced the rate of decline in lung function in patients with IPF in clinical trials, offer hope that it may be possible to alter the increased mortality associated with IPF...
August 10, 2016: Chest
Christine A Fiddler, Nicky Simler, Helen Parfrey, Ahmad Miremadi, Edwin R Chilvers
No abstract text is available yet for this article.
August 2016: Annals of the American Thoracic Society
Guanwei Li, Jianan Ren, Qiongyuan Hu, Youming Deng, Guopu Chen, Kun Guo, Ranran Li, Yuan Li, Lei Wu, Gefei Wang, Guosheng Gu, Jieshou Li
Inflammatory bowel disease (IBD), particularly Crohn's disease, frequently causes intestinal fibrosis that ultimately leads to formation of strictures requiring bowel resection. Currently there is no effective antifibrotic therapy available for this disease. Pirfenidone is a small compound that has a broad spectrum of antifibrogenic effect and has been used for the treatment of fibrotic diseases in various organs. The present study aimed to investigate the antifibrogenic effect of pirfenidone in a dextran sulfate sodium (DSS)-induced murine colitis model...
October 1, 2016: Biochemical Pharmacology
Carlo Albera, Ulrich Costabel, Elizabeth A Fagan, Marilyn K Glassberg, Eduard Gorina, Lisa Lancaster, David J Lederer, Steven D Nathan, Dominique Spirig, Jeff J Swigris
This post hoc analysis examined the differences in idiopathic pulmonary fibrosis disease progression and the effects of pirfenidone in patients stratified by more preserved versus less preserved baseline lung function status using forced vital capacity (FVC) or GAP (gender, age and physiology) index stage.Efficacy outcomes, i.e. FVC, 6-min walking distance (6MWD) and dyspnoea (University of California San Diego Shortness of Breath Questionnaire (UCSD SOBQ)), were analysed at 12 months in patients randomised to pirfenidone 2403 mg·day(-1) or placebo in the pooled phase 3 CAPACITY/ASCEND population (n=1247), with subgroups stratified by baseline FVC ≥80% versus <80% or GAP stage I versus II-III...
September 2016: European Respiratory Journal: Official Journal of the European Society for Clinical Respiratory Physiology
Takekazu Iwata, Ichiro Yoshino, Shigetoshi Yoshida, Norihiko Ikeda, Masahiro Tsuboi, Yuji Asato, Nobuyuki Katakami, Kazuhiro Sakamoto, Yoshinori Yamashita, Jiro Okami, Tetsuya Mitsudomi, Motohiro Yamashita, Hiroshi Yokouchi, Kenichi Okubo, Morihito Okada, Mitsuhiro Takenoyama, Masayuki Chida, Keisuke Tomii, Motoki Matsuura, Arata Azuma, Tae Iwasawa, Kazuyoshi Kuwano, Shuji Sakai, Kenzo Hiroshima, Junya Fukuoka, Kenichi Yoshimura, Hirohito Tada, Kazuhiko Nakagawa, Yoichi Nakanishi
BACKGROUND: Idiopathic pulmonary fibrosis (IPF) often accompanies lung cancer, and life-threatening acute exacerbation (AE) of IPF (AE-IPF) is reported to occur in 20 % of IPF patients who undergo lung cancer surgery. Pirfenidone is an anti-fibrotic agent known to reduce disease progression in IPF patients. A phase II study was conducted to evaluate whether perioperative pirfenidone treatment could reduce the incidence of postoperative AE-IPF patients with lung cancer. METHODS: Pirfenidone was orally administered to IPF patients who were candidates for lung cancer surgery; pirfenidone was dosed at 600 mg/day for the first 2 weeks, followed by 1200 mg/day...
2016: Respiratory Research
George A Margaritopoulos, Eirini Vasarmidi, Katerina M Antoniou
The landscape of idiopathic pulmonary fibrosis (IPF) has changed. The significant progress regarding our knowledge on the pathogenesis of the disease together with the experience achieved after a series of negative trials has led to the development of two drugs for the treatment of IPF. Both pirfenidone and nintedanib can slow significantly the rate of disease progression. They are safe with side effects that can be either prevented by close collaboration between health care professionals and patients or treated successfully when they occur, rarely leading to treatment discontinuation...
2016: Core Evidence
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