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Fibrosis AND Pirfenidone

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https://www.readbyqxmd.com/read/29747274/-real-life-experience-with-pirfenidone-in-idiopathic-pulmonary-fibrosis
#1
Y J Yan, Y L Fan, S W Yu, Q Ye
Objective: To evaluate the efficacy and safety of pirfenidone (PFD) in idiopathic pulmonary fibrosis (IPF) in real-life world. Methods: 47 consecutive patients with IPF taking PFD for at least 12 months (PFD group) were included for analysis, with 47 patients with IPF who did not take PFD as controls. Data were collected from clinical charts to evaluate the lung function parameters and the adverse reactions of PFD. Results: In the PFD group, the percent predicted forced vital capacity (FVC%) and diffusing capacity of carbon monoxide (D(L)CO%) increased by (3...
May 12, 2018: Chinese Journal of Tuberculosis and Respiratory Diseases
https://www.readbyqxmd.com/read/29724385/sildenafil-added-to-pirfenidone-in-patients-with-advanced-idiopathic-pulmonary-fibrosis-and-risk-of-pulmonary-hypertension-a-phase-iib-randomised-double-blind-placebo-controlled-study-rationale-and-study-design
#2
Jürgen Behr, Steven D Nathan, Sergio Harari, Wim Wuyts, Klaus-Uwe Kirchgaessler, Monica Bengus, Frank Gilberg, Athol U Wells
BACKGROUND: Pulmonary hypertension (PH) is commonly observed in patients with advanced idiopathic pulmonary fibrosis (IPF). Despite the availability of therapies for both IPF and PH, none are approved for PH treatment in the context of significant pulmonary disease. This study will investigate the use of sildenafil added to pirfenidone in patients with advanced IPF and risk of PH, who represent a group with a high unmet medical need. METHODS: This Phase IIb, randomised, double-blind, placebo-controlled trial is actively enrolling patients and will study the efficacy, safety and tolerability of sildenafil or placebo in patients with advanced IPF and intermediate or high probability of Group 3 PH who are receiving a stable dose of pirfenidone...
May 2018: Respiratory Medicine
https://www.readbyqxmd.com/read/29718783/an-update-on-emerging-drugs-for-the-treatment-of-idiopathic-pulmonary-fibrosis
#3
Shambhu Aryal, Steven D Nathan
Idiopathic pulmonary fibrosis is a chronic, progressive fibrotic lung disease of unknown etiology associated with a high morbidity and mortality. The hallmark of the disease is impaired healing after alveolar epithelial injury in the setting of a genetic predisposition. Development of two new drugs has changed the landscape of the treatment of IPF but more work is needed to improve outcomes and improve survival. Areas covered: The development of two antifibrotic agents, nintedanib and pirfenidone has been an exciting landmark in the treatment of IPF...
May 8, 2018: Expert Opinion on Emerging Drugs
https://www.readbyqxmd.com/read/29715556/chitosan-alginate-nano-carrier-for-transdermal-delivery-of-pirfenidone-in-idiopathic-pulmonary-fibrosis
#4
Marzieh Abnoos, Mojdeh Mohseni, Seyed Ali Javad Mousavi, Khadijeh Ashtari, Roya Ilka, Bita Mehravi
Pirfenidone (PFD) is one of the pyridine family components with anti-inflammatory, antifibrotic effects and US FDA approved for the treatment of idiopathic pulmonary fibrosis (IPF). Presently, PFD is administered orally and this has setbacks. Hence, it is important to eliminate the pharmacotherapeutic limitations of PFD. This research was carried out to study the possibility of transdermal delivery of PFD using chitosan-sodium alginate nanogel carriers. In order to synthesize chitosan-sodium alginate nanoparticles loaded with PFD, the pre-gelation method was used...
April 28, 2018: International Journal of Biological Macromolecules
https://www.readbyqxmd.com/read/29703175/anti-fibrotic-effects-of-pirfenidone-and-rapamycin-in-primary-ipf-fibroblasts-and-human-alveolar-epithelial-cells
#5
M Molina-Molina, C Machahua-Huamani, V Vicens-Zygmunt, R Llatjós, I Escobar, E Sala-Llinas, P Luburich-Hernaiz, J Dorca, A Montes-Worboys
BACKGROUND: Pirfenidone, a pleiotropic anti-fibrotic treatment, has been shown to slow down disease progression of idiopathic pulmonary fibrosis (IPF), a fatal and devastating lung disease. Rapamycin, an inhibitor of fibroblast proliferation could be a potential anti-fibrotic drug to improve the effects of pirfenidone. METHODS: Primary lung fibroblasts from IPF patients and human alveolar epithelial cells (A549) were treated in vitro with pirfenidone and rapamycin in the presence or absence of transforming growth factor β1 (TGF-β)...
April 27, 2018: BMC Pulmonary Medicine
https://www.readbyqxmd.com/read/29702057/undifferentiated-pleomorphic-sarcoma-after-pirfenidone-use-a-case-report
#6
Christine A Moore, Aaysha Kapila
INTRODUCTION: Pirfenidone was approved in 2014 for the treatment of idiopathic pulmonary fibrosis. Pirfenidone inhibits several factors such as tissue growth factor-β and platelet-derived growth factor, leading to decreased epithelial and fibroblast proliferation and collagen synthesis. The drug improves progression-free survival and is well tolerated, with minimal side effects. However, data on its long-term effects are lacking. CASE PRESENTATION: We present a rare case in which an undifferentiated pleomorphic sarcoma developed in a 59-year-old man with idiopathic pulmonary fibrosis who was treated with pirfenidone for more than a year...
April 4, 2018: Permanente Journal
https://www.readbyqxmd.com/read/29601564/-nintedanib-in-the-treatment-of-fibrosing-interstital-lung-diseases
#7
REVIEW
Katarzyna Lewandowska
Nintedanib is an intracellular tyrosine kinase inhibitor approved in a treatment of idiopathic pulmonary fibrosis. It reduces the annual rate of forced vital capacity decline by approximately 50%, that results in slowing of disease progression. The drug also reduces the incidence of acute exacerbations of idiopathic pulmonary fibrosis. The efficacy of nintedanib was the same in different groups of patients, irrespectively to disease status, age, gender and race. The most frequent side effects were gastrointestinal, i...
March 27, 2018: Polski Merkuriusz Lekarski: Organ Polskiego Towarzystwa Lekarskiego
https://www.readbyqxmd.com/read/29601563/-advances-in-differential-diagnosis-and-treatment-of-patients-with-sarcoidosis
#8
REVIEW
Tadeusz Płusa
The implementation of treatment in patients with sarcoidosis (SA) must be associated with the certainty of diagnosis, which is difficult due to the lack of unambiguous criteria. Finding the presence of noncaseating granulomas in bioptic material is not always indicative of SA. The main point of SA's diagnosis is the level of its activity, because only patients in the active stage should be qualified for treatment. In therapy, glucocorticosteroids or second-line drugs - methotrexate or azathioprine are still recommended...
March 27, 2018: Polski Merkuriusz Lekarski: Organ Polskiego Towarzystwa Lekarskiego
https://www.readbyqxmd.com/read/29587263/a-real-life-multicenter-national-study-on-nintedanib-in-severe-idiopathic-pulmonary-fibrosis
#9
Sergio Harari, Antonella Caminati, Venerino Poletti, Marco Confalonieri, Stefano Gasparini, Donato Lacedonia, Fabrizio Luppi, Alberto Pesci, Alfredo Sebastiani, Paolo Spagnolo, Carlo Vancheri, Elisabetta Balestro, Martina Bonifazi, Stefania Cerri, Federica De Giacomi, Rossana Della Porta, Maria Pia Foschino Barbaro, Annalisa Fui, Patrizio Pasquinelli, Roberta Rosso, Sara Tomassetti, Claudia Specchia, Paola Rottoli
BACKGROUND: Two therapeutic options are currently available for patients with mild-to-moderate idiopathic pulmonary fibrosis (IPF): pirfenidone and nintedanib. To date, there is still insufficient data on the efficacy of these 2 agents in patients with more severe disease. OBJECTIVES: This national, multicenter, retrospective real-life study was intended to determine the impact of nintedanib on the treatment of patients with severe IPF. METHODS: All patients included had severe IPF and had to have at least 6 months of follow-up before and at least 6 months of follow-up after starting nintedanib...
March 27, 2018: Respiration; International Review of Thoracic Diseases
https://www.readbyqxmd.com/read/29552620/activation-of-myofibroblast-trpa1-by-steroids-and-pirfenidone-ameliorates-fibrosis-in-experimental-crohn-s-disease
#10
Lin Hai Kurahara, Keizo Hiraishi, Yaopeng Hu, Kaori Koga, Miki Onitsuka, Mayumi Doi, Kunihiko Aoyagi, Hidetoshi Takedatsu, Daibo Kojima, Yoshitaka Fujihara, Yuwen Jian, Ryuji Inoue
Background & Aims: The transient receptor potential ankyrin 1 (TRPA1) channel is highly expressed in the intestinal lamina propria, but its contribution to gut physiology/pathophysiology is unclear. Here, we evaluated the function of myofibroblast TRPA1 channels in intestinal remodeling. Methods: An intestinal myofibroblast cell line (InMyoFibs) was stimulated by transforming growth factor-β1 to induce in vitro fibrosis. Trpa1 knockout mice were generated using the Clustered regularly interspaced short palindromic repeats (CRISPR)/CRISPR-associated 9 (Cas9) system...
March 2018: Cellular and Molecular Gastroenterology and Hepatology
https://www.readbyqxmd.com/read/29548661/does-pirfenidone-have-anticancer-effects-in-patients-with-idiopathic-pulmonary-fibrosis
#11
EDITORIAL
Yoshinori Tanino
No abstract text is available yet for this article.
March 2018: Respiratory Investigation
https://www.readbyqxmd.com/read/29547661/a-fully-automated-image-analysis-method-to-quantify-lung-fibrosis-in-the-bleomycin-induced-rat-model
#12
Shanon Seger, Manuel Stritt, Enrico Vezzali, Oliver Nayler, Patrick Hess, Peter M A Groenen, Anna K Stalder
Intratracheal administration of bleomycin induces fibrosis in the lung, which is mainly assessed by histopathological grading that is subjective. Current literature highlights the need of reproducible and quantitative pulmonary fibrosis analysis. If some quantitative studies looked at fibrosis parameters separately, none of them quantitatively assessed both aspects: lung tissue remodeling and collagenization. To ensure reliable quantification, support vector machine learning was used on digitalized images to design a fully automated method that analyzes two important aspects of lung fibrosis: (i) areas having substantial tissue remodeling with appearance of dense fibrotic masses and (ii) collagen deposition...
2018: PloS One
https://www.readbyqxmd.com/read/29532550/fibroblast-matrix-interplay-nintedanib-and-pirfenidone-modulate-the-effect-of-ipf-fibroblast-conditioned-matrix-on-normal-fibroblast-phenotype
#13
Gali Epstein Shochet, Lutz Wollin, David Shitrit
BACKGROUND AND OBJECTIVE: Idiopathic pulmonary fibrosis (IPF) is a progressive lung disease with poor prognosis. Activated fibroblasts are the key effector cells in fibrosis, producing excessive amounts of collagen and extracellular matrix (ECM) proteins. Whether the ECM conditioned by IPF fibroblasts determines the phenotype of naïve fibroblasts is difficult to explore. METHODS: IPF-derived primary fibroblasts were cultured on Matrigel and then cleared using ammonium hydroxide, creating an IPF-conditioned matrix (CM)...
March 12, 2018: Respirology: Official Journal of the Asian Pacific Society of Respirology
https://www.readbyqxmd.com/read/29497173/pirfenidone-reduces-profibrotic-responses-in-human-dermal-myofibroblasts-in-vitro
#14
Caroline L Hall, Adrienne R Wells, Kai P Leung
Pirfenidone (PFD) is a synthetic small molecule inhibitor with demonstrated anti-inflammatory and antifibrotic properties in vitro and in vivo. The exact mechanism(s) of PFD action remain unclear, due in part to the broad effects of this drug on the complex processes involved in inflammation and fibrosis. While PFD is FDA-approved for the treatment of idiopathic pulmonary fibrosis, the efficacy of this compound for the treatment of dermal fibrosis has not yet been fully characterized. Dermal fibrosis is the pathological formation of excess fibrous connective tissue of the skin, usually the result of traumatic cutaneous injury...
March 1, 2018: Laboratory Investigation; a Journal of Technical Methods and Pathology
https://www.readbyqxmd.com/read/29490307/real-world-experience-with-nintedanib-in-patients-with-idiopathic-pulmonary-fibrosis
#15
Eva Brunnemer, Julia Wälscher, Svenja Tenenbaum, Julia Hausmanns, Karen Schulze, Marianne Seiter, Claus Peter Heussel, Arne Warth, Felix J F Herth, Michael Kreuter
BACKGROUND: Nintedanib, an oral tyrosine kinase inhibitor, has been shown to slow down the progression of idiopathic pulmonary fibrosis (IPF) in two randomised placebo-controlled trials by reducing the annual decline in forced vital capacity (FVC). However, real-world experience is limited. OBJECTIVE: To assess the efficacy and safety of nintedanib in a large cohort of patients treated at a tertiary referral site for interstitial lung diseases. METHODS: The records of patients with a confirmed diagnosis of IPF were reviewed...
2018: Respiration; International Review of Thoracic Diseases
https://www.readbyqxmd.com/read/29471816/idiopathic-pulmonary-fibrosis-pathogenesis-and-management
#16
REVIEW
Giacomo Sgalla, Bruno Iovene, Mariarosaria Calvello, Margherita Ori, Francesco Varone, Luca Richeldi
BACKGROUND: Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive disease characterized by the aberrant accumulation of fibrotic tissue in the lungs parenchyma, associated with significant morbidity and poor prognosis. This review will present the substantial advances achieved in the understanding of IPF pathogenesis and in the therapeutic options that can be offered to patients, and will address the issues regarding diagnosis and management that are still open. MAIN BODY: Over the last two decades much has been clarified about the pathogenic pathways underlying the development and progression of the lung scarring in IPF...
February 22, 2018: Respiratory Research
https://www.readbyqxmd.com/read/29465476/idiopathic-pulmonary-fibrosis-a-guide-for-nurse-practitioners
#17
Michelle Vega-Olivo, Gerard J Criner
Idiopathic pulmonary fibrosis (IPF) is a rare disease characterized by decline in lung function, dyspnea, and cough. The clinical course of IPF is variable and unpredictable. Early referral to specialists is key to ensure timely and accurate diagnosis. Two antifibrotic drugs (nintedanib and pirfenidone) have been approved for the treatment of IPF.
May 17, 2018: Nurse Practitioner
https://www.readbyqxmd.com/read/29462659/safer-approaches-to-therapeutic-modulation-of-tgf-%C3%AE-signaling-for-respiratory-disease
#18
REVIEW
Philippe Lachapelle, Meina Li, Jo Douglass, Alastair Stewart
The transforming growth factor (TGF)-β cytokines play a central role in development and progression of chronic respiratory diseases. TGF-β overexpression in chronic inflammation, remodeling, fibrotic process and susceptibility to viral infection is established in the most prevalent chronic respiratory diseases including asthma, COPD, lung cancer and idiopathic pulmonary fibrosis. Despite the overwhelming burden of respiratory diseases in the world, new pharmacological therapies have been limited in impact...
February 17, 2018: Pharmacology & Therapeutics
https://www.readbyqxmd.com/read/29408757/therapeutic-administration-of-inhaled-ins1009-a-treprostinil-prodrug-formulation-inhibits-bleomycin-induced-pulmonary-fibrosis-in-rats
#19
Michel R Corboz, Jimin Zhang, Daniel LaSala, Keith DiPetrillo, Zhili Li, Vladimir Malinin, Jeremy Brower, Philip J Kuehl, Ted E Barrett, Walter R Perkins, Richard W Chapman
Idiopathic pulmonary fibrosis is a progressive and lethal disease and while there are now two approved drugs (Esbriet® and Ofev® ) additional effective treatments are still needed. Recently, prostacyclin analogs such as iloprost and treprostinil (TRE) have been shown to exert some protection against bleomycin-induced pulmonary fibrosis in mice when administered in a prophylactic regimen. In this study, we evaluated the effect of the inhaled treprostinil prodrug hexadecyl-treprostinil (C16TR) formulated in a lipid nanoparticle (INS1009) administered therapeutically in a fibrotic rat model...
April 2018: Pulmonary Pharmacology & Therapeutics
https://www.readbyqxmd.com/read/29404521/drug-idiosyncrasy-due-to-pirfenidone-presenting-as-acute-liver-failure-case-report-and-mini-review-of-the-literature
#20
REVIEW
Nipun Verma, Pramod Kumar, Suvradeep Mitra, Sunil Taneja, Sahajal Dhooria, Ashim Das, Ajay Duseja, Radha Krishan Dhiman, Yogesh Chawla
Idiosyncratic drug-induced liver injury (DILI) is ranked among the top most common etiologies of acute liver failure (ALF). It carries poor transplant-free survival. Pirfenidone is an anti-inflammatory and antifibrotic drug that is commonly used for the treatment of idiopathic pulmonary fibrosis (IPF). Hepatotoxicity due to pirfenidone is rare and generally manifests as a mild rise in serum aminotransferases. In this mini-review, we report an unusual case of idiosyncratic DILI due to pirfenidone presenting as ALF, with emphasis on the definition, classification, diagnostic criteria, histopathology, molecular markers, and treatment options for DILI and related ALF...
February 2018: Hepatology communications
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