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Fibrosis AND Pirfenidone

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https://www.readbyqxmd.com/read/29341047/german-guideline-for-idiopathic-pulmonary-fibrosis-update-on-pharmacological-therapies-2017
#1
Jürgen Behr, Andreas Günther, Francesco Bonella, Klaus Geißler, Dirk Koschel, Michael Kreuter, Antje Prasse, Nicolas Schönfeld, Helmut Sitter, Joachim Müller-Quernheim, Ulrich Costabel
Idiopathic pulmonary fibrosis (IPF) is a severe and often fatal disease with a median survival of 2 - 4 years after diagnosis. Since the publication of the German IPF guideline in 2013 new treatment trials have been published, necessitating an update of the pharmacological therapy of IPF. Different from the previous guideline, the GRADE system was discarded and replaced by the Oxford evidence classification system which allows a more differentiated judgement. The following pharmacological therapies were rated not suitable for the treatment of IPF patients (recommendation A; evidence 1-b): triple therapy with prednisolone, azathioprine and acetyl-cysteine; imatinib; ambrisentan; bosentan; macitentan...
January 16, 2018: Pneumologie
https://www.readbyqxmd.com/read/29327616/nintedanib-for-the-treatment-of-idiopathic-pulmonary-fibrosis
#2
Francesco Varone, Giacomo Sgalla, Bruno Iovene, Teresa Bruni, Luca Richeldi
Idiopathic Pulmonary Fibrosis (IPF) is an interstitial lung disease characterized by the progressive loss of pulmonary function, ultimately leading to respiratory failure and death. Two novel compounds, nintedanib and pirfenidone, have shown efficacy in reducing the rate of decline of lung function in IPF patients. The multiple tyrosine kinase inhibitor nintedanib has extensively being studied as a potential angiogenesis inhibitor in clinical against various neoplastic disorders. Afterwards, this compound was successfully tested in IPF...
January 12, 2018: Expert Opinion on Pharmacotherapy
https://www.readbyqxmd.com/read/29325685/reduced-incidence-of-lung-cancer-in-patients-with-idiopathic-pulmonary-fibrosis-treated-with-pirfenidone
#3
Yukiko Miura, Takefumi Saito, Toru Tanaka, Hiroyuki Takoi, Yohei Yatagai, Minoru Inomata, Takahito Nei, Yoshinobu Saito, Akihiko Gemma, Arata Azuma
BACKGROUND: Idiopathic pulmonary fibrosis (IPF) is a disease with a worse prognosis than some types of cancer. In patients with IPF, lung cancer is critical because of the associated high mortality rate from its progression and fatal complications from anticancer treatments. Therefore, preventing lung cancer in patients with IPF is primordial. Pirfenidone is an anti-fibrotic agent that reduces the decline in forced vital capacity. This study aimed to assess the effect of pirfenidone in the development of lung cancer in patients with IPF...
January 2018: Respiratory Investigation
https://www.readbyqxmd.com/read/29316328/anti-fibrotic-effects-of-pirfenidone-by-interference-with-the-hedgehog-signalling-pathway-in-patients-with-systemic-sclerosis-associated-interstitial-lung-disease
#4
Hua Xiao, Guang-Feng Zhang, Xiang-Ping Liao, Xiao-Jie Li, Jian Zhang, Haobo Lin, Zhe Chen, Xiao Zhang
AIM: To determine whether pirfenidone attenuates lung fibrosis by interfering with the hedgehog (Hh) signalling pathway in patients with systemic sclerosis-associated interstitial lung disease (SSc-ILD). METHODS: Twenty-five SSc-ILD patients (20 first visit, five who underwent pirfenidone treatment for 6 months) and 10 healthy controls were recruited. Lung tissues were obtained by open-chest surgery, and primary lung fibroblasts were isolated, cultured and stimulated with pirfenidone...
January 7, 2018: International Journal of Rheumatic Diseases
https://www.readbyqxmd.com/read/29288933/targeting-the-renin-angiotensin-system-as-novel-therapeutic-strategy-for-pulmonary-diseases
#5
REVIEW
Wan Shun Daniel Tan, Wupeng Liao, Shuo Zhou, Dan Mei, Wai-Shiu Fred Wong
The renin-angiotensin system (RAS) plays a major role in regulating electrolyte balance and blood pressure. RAS has also been implicated in the regulation of inflammation, proliferation and fibrosis in pulmonary diseases such as asthma, acute lung injury (ALI), chronic obstructive pulmonary disease (COPD), idiopathic pulmonary fibrosis (IPF) and pulmonary arterial hypertension (PAH). Current therapeutics suffer from some drawbacks like steroid resistance, limited efficacies and side effects. Novel intervention is definitely needed to offer optimal therapeutic strategy and clinical outcome...
December 27, 2017: Current Opinion in Pharmacology
https://www.readbyqxmd.com/read/29278701/gentiopicroside-ameliorates-bleomycin-induced-pulmonary-fibrosis-in-mice-via-inhibiting-inflammatory-and-fibrotic-process
#6
Cheng Chen, Yong-Yan Wang, Ying-Xia Wang, Meng-Qun Cheng, Jian-Bing Yin, Xuan Zhang, Zhi-Peng Hong
Pulmonary fibrosis (PF) is a chronic and ultimately fatal interstitial lung disease of various causes. The advent of nintedanib and pirfenidone provides treatment options for PF patients for the first time. However, the adverse effects of the two drugs such as gastrointestinal disorders and hepatic dysfunction often lead to treatment discontinuation. Gentiopicroside (GPS) is a natural secoiridoid glycoside from gentian species of medicinal plants, and has a variety of pharmacological activities, including hepatoprotective and cholagogic, anti-inflammatory, antinociceptive, and smooth muscle relaxing activities...
December 23, 2017: Biochemical and Biophysical Research Communications
https://www.readbyqxmd.com/read/29275617/-treatment-of-patients-with-different-degree-of-acute-respiratory-distress-syndrome-caused-by-inhalation-of-white-smoke
#7
F W Yang, H M Xin, J H Zhu, X Y Feng, X C Jiang, Z Y Gong, Y L Tong
Objective: To summarize the treatment experience of patients with different degree of acute respiratory distress syndrome (ARDS) caused by inhalation of white smoke from burning smoke bomb. Methods: A batch of 13 patients with different degree of ARDS caused by inhalation of white smoke from burning smoke bomb, including 2 patients complicated by pulmonary fibrosis at the late stage, were admitted to our unit in February 2016. Patients were divided into mild (9 cases), moderate (2 cases), and serious (2 cases) degree according to the ARDS Berlin diagnostic criteria...
December 20, 2017: Zhonghua Shao Shang za Zhi, Zhonghua Shaoshang Zazhi, Chinese Journal of Burns
https://www.readbyqxmd.com/read/29273418/pirfenidone-protects-against-paraquat-induced-lung-injury-and-fibrosis-in-mice-by-modulation-of-inflammation-oxidative-stress-and-gene-expression
#8
Fateme Pourgholamhosein, Rokhsana Rasooli, Mostafa Pournamdari, Leyla Pourgholi, Mitra Samareh-Fekri, Mahmoud Ghazi-Khansari, Maryam Iranpour, Hamid-Reza Poursalehi, Mahmoud-Reza Heidari, Ali Mandegary
In this study we investigated the protective effects and possible mechanisms of pirfenidone (PF) in paraquat (PQ)-induced lung injury and fibrosis in mice. Lung injury was induced by injection of PQ (20 mg/kg). Thereafter, mice orally received water and PF (100 and 200 mg/kg) for four weeks. After 28 days, the inflammation and fibrosis were determined in the lungs by analysis of histopathology, bronchoalveolar lavage fluid (BALF) cell count, lung wet/dry weight ratio, hydroxyproline content, and oxidative stress biomarkers...
December 19, 2017: Food and Chemical Toxicology
https://www.readbyqxmd.com/read/29258746/nlrp3-inflammasome-inhibition-attenuates-silica-induced-epithelial-to-mesenchymal-transition-emt-in-human-bronchial-epithelial-cells
#9
Xiang Li, Xiaopei Yan, Yanli Wang, Jingjing Wang, Fang Zhou, Hong Wang, Weiping Xie, Hui Kong
Silicosis is an incurable and progressive lung disease characterized by chronic inflammation and fibroblasts accumulation. Studies have indicated a vital role for epithelial-mesenchymal transition (EMT) in fibroblasts accumulation. NLRP3 inflammasome is a critical mediator of inflammation in response to a wide range of stimuli (including silica particles), and plays an important role in many respiratory diseases. However, whether NLRP3 inflammasome regulates silica-induced EMT remains unknown. Our results showed that silica induced EMT in human bronchial epithelial cells (16HBE cells) in a dose- and time-dependent manner...
December 16, 2017: Experimental Cell Research
https://www.readbyqxmd.com/read/29248299/discovery-of-1-4-3-4-methylpiperazin-1-yl-propyl-amino-benzyl-5-trifluoromethyl-pyridin-2-1h-one-an-orally-active-multi-target-agent-for-the-treatment-of-diabetic-nephropathy
#10
Jun Chen, Zhangzhe Peng, Miaomiao Lu, Xuan Xiong, Zhuo Chen, Qianbin Li, Zeneng Cheng, Dejian Jiang, Lijian Tao, Gaoyun Hu
Oxidative stress, inflammation and fibrosis can cause irreversible damage on cell structure and function of kidney and are key pathological factors in Diabetic Nephropathy (DN). Therefore, multi-target agents are urgently need for the clinical treatment of DN. Using Pirfenidone as a lead compound and based on the previous research, two novel series (5-trifluoromethyl)-2(1H)-pyridone analogs were designed and synthesized. SAR of (5-trifluoromethyl)-2(1H)-pyridone derivatives containing nitrogen heterocyclic ring have been established for in vitro potency...
July 4, 2017: Bioorganic & Medicinal Chemistry Letters
https://www.readbyqxmd.com/read/29238708/longitudinal-real-world-outcomes-of-pirfenidone-in-idiopathic-pulmonary-fibrosis-in-greece
#11
Argyrios Tzouvelekis, Theodoros Karampitsakos, Paschalis Ntolios, Vasilios Tzilas, Evangelos Bouros, Evangelos Markozannes, Ioanna Malliou, Aris Anagnostopoulos, Andreas Granitsas, Paschalis Steiropoulos, Katerina Dimakou, Serafeim Chrysikos, Nikolaos Koulouris, Demosthenes Bouros
Background: Pirfenidone is an antifibrotic compound able to slow down disease progression in patients with idiopathic pulmonary fibrosis (IPF). Objective: To investigate the safety and efficacy of pirfenidone in patients with IPF in a real-life setting. Methods: This was a multicenter, retrospective, real-life, observational study for patients with IPF receiving pirfenidone. Results: We identified 92 patients with IPF receiving pirfenidone...
2017: Frontiers in Medicine
https://www.readbyqxmd.com/read/29225786/recent-advances-in-managing-idiopathic-pulmonary-fibrosis
#12
REVIEW
Chiara Scelfo, Antonella Caminati, Sergio Harari
Idiopathic pulmonary fibrosis (IPF) is a rare pulmonary disease with a poor prognosis and severe impact on quality of life. Early diagnosis is still challenging and important delays are registered before final diagnosis can be reached. Available tools fail to predict the variable course of the disease and to evaluate response to antifibrotic drugs. Despite the recent approval of pirfenidone and nintedanib, significant challenges remain to improve prognosis and quality of life. It is hoped that the new insights gained in pathobiology in the last few years will lead to further advances in the diagnosis and management of IPF...
2017: F1000Research
https://www.readbyqxmd.com/read/29212837/pirfenidone-safety-and-adverse-event-management-in-idiopathic-pulmonary-fibrosis
#13
REVIEW
Lisa H Lancaster, Joao A de Andrade, Joseph D Zibrak, Maria L Padilla, Carlo Albera, Steven D Nathan, Marlies S Wijsenbeek, John L Stauffer, Klaus-Uwe Kirchgaessler, Ulrich Costabel
Pirfenidone is one of two approved therapies for the treatment of idiopathic pulmonary fibrosis (IPF). Randomised controlled clinical trials and subsequent post hoc analyses have demonstrated that pirfenidone reduces lung function decline, decreases mortality and improves progression-free survival. Long-term extension trials, registries and real-world studies have also shown similar treatment effects with pirfenidone. However, for patients with IPF to obtain the maximum benefits of pirfenidone treatment, the potential adverse events (AEs) associated with pirfenidone need to be managed...
December 31, 2017: European Respiratory Review: An Official Journal of the European Respiratory Society
https://www.readbyqxmd.com/read/29209910/efficacy-and-safety-of-nintedanib-for-the-treatment-of-idiopathic-pulmonary-fibrosis-an-update
#14
José Antonio Rodríguez-Portal
Idiopathic pulmonary fibrosis is a fatal form of progressive fibrosing interstitial pneumonia with limited treatment options. In recent years, its management has been transformed with the approval of two new antifibrotic drugs: nintedanib and pirfenidone. Nintedanib is a tyrosine kinase inhibitor that efficiently slows idiopathic pulmonary fibrosis progression and has an acceptable tolerability profile. This article reviews new available evidence on the long-term efficacy and safety of nintedanib in patients with idiopathic pulmonary fibrosis...
December 5, 2017: Drugs in R&D
https://www.readbyqxmd.com/read/29197520/effects-of-pirfenidone-in-acute-and-sub-chronic-liver-fibrosis-and-an-initiation-promotion-cancer-model-in-the-mouse
#15
Oleksii Seniutkin, Shinji Furuya, Yu-Syuan Luo, Joseph A Cichocki, Hisataka Fukushima, Yuki Kato, Hiromi Sugimoto, Tomoko Matsumoto, Takeki Uehara, Ivan Rusyn
Liver fibrosis results from chronic tissue damage and excessive regeneration with accumulation of extracellular matrix proteins; it is a precursor of liver cirrhosis and hepatocellular carcinoma. Liver fibrosis treatments are primarily directed at inflammation, with few options to combat fibrogenesis. Pirfenidone is a drug approved for idiopathic pulmonary fibrosis and this study was focused on anti-fibrotic and anti-cancer potential of pirfenidone in the liver of male B6C3F1/J mice. In a dose-finding study, mice were treated with CCl4 (0...
November 29, 2017: Toxicology and Applied Pharmacology
https://www.readbyqxmd.com/read/29183898/pirfenidone-induced-hyponatraemia-insight-in-mechanism-risk-factor-and-management
#16
Leann Silhan, Panagis Galiatsatos, Justine Corwine, Sonye Danoff
Pirfenidone was approved in October 2014 in the USA for the treatment of idiopathic pulmonary fibrosis. Although not included in the adverse events published in the CAPACITY-1 and CAPACITY-2 or ASCEND trials, hyponatraemia was reported in supplementary data with rate of 3.4% in the active therapy arm versus 0.3% in the placebo arm. We performed a retrospective analysis of patients who were initiated on pirfenidone or nintedanib for the treatment of pulmonary fibrosis at our centre. Of the 52 patients who were started on pirfenidone, three (5...
November 27, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/29171670/potential-treatment-for-vocal-fold-scar-with-pirfenidone
#17
Haruka Kodama, Yoshihiko Kumai, Kohei Nishimoto, Yutaka Toya, Satoru Miyamaru, Shinobu Furushima, Eiji Yumoto
OBJECTIVES/HYPOTHESIS: Pirfenidone (PFD) is a strong antifibrotic agent that has been clinically approved in Japan for idiopathic pulmonary fibrosis. We examined the antifibrotic effects of PFD on fibroblasts isolated from scarred vocal folds (VFs) of ferrets in vitro. STUDY DESIGN: Prospective animal experiments with controls. METHODS: Scar fibroblasts (SFs) were isolated from scarred VFs that had been electrocauterized 2 weeks before harvesting (N = 4)...
November 24, 2017: Laryngoscope
https://www.readbyqxmd.com/read/29143231/correction-to-combination-therapy-with-pirfenidone-plus-prednisolone-ameliorates-paraquat-induced-pulmonary-fibrosis
#18
Rokhsana Rasooli, Fatemeh Pourgholamhosein, Younes Kamali, Fatemeh Nabipour, Ali Mandegary
Unfortunately, the original publication of this article contained mistakes, and the authors would like to correct them. The corrected details are given below.
November 15, 2017: Inflammation
https://www.readbyqxmd.com/read/29101500/early-clinical-experiences-with-nintedanib-in-three-uk-tertiary-interstitial-lung-disease-centres
#19
Hannah Toellner, G Hughes, W Beswick, M G Crooks, C Donaldson, I Forrest, S P Hart, C Leonard, M Major, A J Simpson, N Chaudhuri
BACKGROUND: Nintedanib has been shown to slow disease progression in patients with idiopathic pulmonary fibrosis (IPF). It was approved by the National Institute for Health and Care Excellence (NICE) in January 2016 for IPF patients with a forced vital capacity (FVC) of 50-80% in the United Kingdom (UK). AIM: To report real world data about our early clinical experience using nintedanib in 187 patients with a multi-disciplinary (MDT) diagnosis of IPF in a manufacturer funded patient in need scheme (three UK centres) prior to NICE approval...
November 3, 2017: Clinical and Translational Medicine
https://www.readbyqxmd.com/read/29074545/idiopathic-pulmonary-fibrosis-lessons-from-clinical-trials-over-the-past-25%C3%A2-years
#20
REVIEW
Ganesh Raghu
Idiopathic pulmonary fibrosis (IPF) is a progressive and ultimately fatal disease. A major breakthrough in treatment came when, after decades of clinical trials which failed to identify an efficacious treatment regimen, two therapies were successful in Phase-III trials. The advent of these therapies, nintedanib and pirfenidone, meant that for the first time IPF patients had two treatment options that could reduce disease progression. This review summarises the key lessons to be obtained from the clinical trials that led to the current international clinical practice guidelines for the treatment of IPF and provides insights for the design of future clinical trials that are needed if we are to improve outcomes that are clinically meaningful to IPF patients...
October 2017: European Respiratory Journal: Official Journal of the European Society for Clinical Respiratory Physiology
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