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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
Dina S El-Agamy
OBJECTIVES: This study aimed to evaluate the potential protective effects of pirfenidone (PFD) against concanavalin A (Con A)-induced hepatitis in mice. METHODS: Autoimmune model of hepatitis was established using single intravenous injection of Con A. Mice were randomly assigned into four groups as follows: control group; Con A group; and two groups, receiving PFD in two dose levels (200, 300 mg/kg) for 5 days before Con A administration. Extent of hepatitis was studied using biochemical, histopathological and immunohistochemical estimations...
October 13, 2016: Journal of Pharmacy and Pharmacology
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
Dianbo Li, Liping Gong
The aim of this study was to develop and characterize pirfenidone (PF)-loaded chitosan microspheres for lung targeting. The microspheres were prepared using the emulsion-solvent evaporation method and characterized by assessing morphology, particle size, and zeta potential. The microspheres had a spherical nature with highly smooth and integrated surfaces. The particle size of microspheres was 4.6±0.3 µm, and the zeta potential was 20.3±1.4 mV. The in vitro release results indicated that the obtained formulation of PF could reach the state of sustained release with a biphasic drug release pattern...
2016: Drug Design, Development and Therapy
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
Ting Li, Li Guo, Zhiwei Chen, Liyang Gu, Fangfang Sun, Xiaoming Tan, Sheng Chen, Xiaodong Wang, Shuang Ye
To evaluate the efficacy of pirfenidone in patients with rapidly progressive interstitial lung disease (RPILD) related to clinically amyopathic dermatomyositis (CADM), we conducted an open-label, prospective study with matched retrospective controls. Thirty patients diagnosed with CADM-RPILD with a disease duration <6 months at Renji Hospital South Campus from June 2014 to November 2015 were prospectively enrolled and treated with pirfenidone at a target dose of 1800 mg/d in addition to conventional treatment, such as a glucocorticoid and/or other immunosuppressants...
2016: Scientific Reports
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
Mei Yang, Yangfan Yang, Ming Lei, Chengtian Ye, Chunshun Zhao, Jiangang Xu, Kaili Wu, Minbin Yu
CONTEXT: Pirfenidone (PFD) has exhibited therapeutic potential in the treatment of cell proliferative disorders. The previously developed 0.5% water-based PFD eye drops by our team exhibited anti-scarring effectiveness and ocular safety but with a limit of short half-life and poor bioavailability. OBJECTIVE: To increase bioavailability of the water-based PFD eye drops, we prepared a viscous solution by adding hydroxypropyl methylcellulose (HPMC, F4M), which acted as a viscosity-enhancer...
September 5, 2016: Drug Development and Industrial Pharmacy
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
Elizabeth R Volkmann, Donald P Tashkin
Although interstitial lung disease accounts for the majority of deaths in patients with systemic sclerosis, treatment options for this manifestation of systemic sclerosis are limited. Few high quality, randomized controlled trials exist for systemic sclerosis-related interstitial lung disease, and historically, studies have favored the use of cyclophosphamide. However, the benefit of cyclophosphamide for systemic sclerosis-related interstitial lung disease is tempered by its complex adverse event profile. More recent studies have demonstrated the effectiveness of mycophenolate for systemic sclerosis-related interstitial lung disease, including the recently published Scleroderma Lung Study II...
August 25, 2016: Annals of the American Thoracic Society
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
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