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IPF future treatments

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21 papers 100 to 500 followers
By Jason Mann No BS pulmonary critical care fellow
https://www.readbyqxmd.com/read/27581827/cough-in-idiopathic-pulmonary-fibrosis
#1
REVIEW
Mirjam J G van Manen, Surinder S Birring, Carlo Vancheri, Vincent Cottin, Elisabetta A Renzoni, Anne-Marie Russell, Marlies S Wijsenbeek
Many patients with idiopathic pulmonary fibrosis (IPF) complain of chronic refractory cough. Chronic cough is a distressing and disabling symptom with a major impact on quality of life. During recent years, progress has been made in gaining insight into the pathogenesis of cough in IPF, which is most probably "multifactorial" and influenced by mechanical, biochemical and neurosensory changes, with an important role for comorbidities as well. Clinical trials of cough treatment in IPF are emerging, and cough is increasingly included as a secondary end-point in trials assessing new compounds for IPF...
September 2016: European Respiratory Review: An Official Journal of the European Respiratory Society
https://www.readbyqxmd.com/read/27521738/therapeutic-approach-to-adult-fibrotic-lung-diseases
#2
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
https://www.readbyqxmd.com/read/26968970/effect-of-continued-treatment-with-pirfenidone-following-clinically-meaningful-declines-in-forced-vital-capacity-analysis-of-data-from-three-phase-3-trials-in-patients-with-idiopathic-pulmonary-fibrosis
#3
RANDOMIZED CONTROLLED TRIAL
Steven D Nathan, Carlo Albera, Williamson Z Bradford, Ulrich Costabel, Roland M du Bois, Elizabeth A Fagan, Robert S Fishman, Ian Glaspole, Marilyn K Glassberg, Kenneth F Glasscock, Talmadge E King, Lisa Lancaster, David J Lederer, Zhengning Lin, Carlos A Pereira, Jeffrey J Swigris, Dominique Valeyre, Paul W Noble, Athol U Wells
BACKGROUND: The assessment of treatment response in idiopathic pulmonary fibrosis (IPF) is complicated by the variable clinical course. We examined the variability in the rate of disease progression and evaluated the effect of continued treatment with pirfenidone in patients who experienced meaningful progression during treatment. METHODS: The source population included patients enrolled in the ASCEND and CAPACITY trials (N=1247). Pearson's correlation coefficients were used to characterise the relationship between changes in FVC during consecutive 6-month intervals in the placebo population...
May 2016: Thorax
https://www.readbyqxmd.com/read/26965296/fg-3019-anti-connective-tissue-growth-factor-monoclonal-antibody-results-of-an-open-label-clinical-trial-in-idiopathic-pulmonary-fibrosis
#4
Ganesh Raghu, Mary Beth Scholand, João de Andrade, Lisa Lancaster, Yolanda Mageto, Jonathan Goldin, Kevin K Brown, Kevin R Flaherty, Mark Wencel, Jack Wanger, Thomas Neff, Frank Valone, John Stauffer, Seth Porter
FG-3019 is a fully human monoclonal antibody that interferes with the action of connective tissue growth factor, a central mediator in the pathogenesis of fibrosis.This open-label phase 2 trial evaluated the safety and efficacy of two doses of FG-3019 administered by intravenous infusion every 3 weeks for 45 weeks in patients with idiopathic pulmonary fibrosis (IPF). Subjects had a diagnosis of IPF within the prior 5 years defined by either usual interstitial pneumonia (UIP) pattern on a recent high-resolution computed tomography (HRCT) scan, or a possible UIP pattern on HRCT scan and a recent surgical lung biopsy showing UIP pattern...
May 2016: European Respiratory Journal: Official Journal of the European Society for Clinical Respiratory Physiology
https://www.readbyqxmd.com/read/26869678/recombinant-human-pentraxin-2-therapy-in-patients-with-idiopathic-pulmonary-fibrosis-safety-pharmacokinetics-and-exploratory-efficacy
#5
Bernt van den Blink, Marlous R Dillingh, Leo C Ginns, Lake D Morrison, Matthijs Moerland, Marlies Wijsenbeek, Elizabeth G Trehu, Brian J Bartholmai, Jacobus Burggraaf
Abnormal fibrogenic repair response upon alveolar injury is believed to play an important role in the pathogenesis of idiopathic pulmonary fibrosis (IPF). PRM-151 (recombinant human pentraxin-2, also known as serum amyloid P), has been shown to reduce fibrosis in preclinical lung fibrosis models, and was well tolerated with a favourable pharmacokinetic profile in an earlier single-dose phase I study.A randomised, double-blind, placebo-controlled, multiple ascending dose trial was performed to assess the tolerability and pharmacokinetic and pharmacodynamic characteristics of multiple doses of PRM-151 in IPF patients...
March 2016: European Respiratory Journal: Official Journal of the European Society for Clinical Respiratory Physiology
https://www.readbyqxmd.com/read/26593230/cell-based-therapies-for-the-treatment-of-idiopathic-pulmonary-fibrosis-ipf-disease
#6
REVIEW
Maliheh Ghadiri, Paul M Young, Daniela Traini
INTRODUCTION: During the last few decades, cell-based therapies have shown great potential to treat patients with lung diseases. It has been proposed that the administration of cells into an injured lung could be considered as a therapeutic method to repair and replace lost lung tissue. Using this method, transplanted cells with the ability to proliferate and differentiate into alveolar cells, have been suggested as a therapeutic strategy for IPF treatment. AREAS COVERED: In this review, the latest investigations using various types of cells for IPF therapy have been presented...
2016: Expert Opinion on Biological Therapy
https://www.readbyqxmd.com/read/26331942/tollip-muc5b-and-the-response-to-n-acetylcysteine-among-individuals-with-idiopathic-pulmonary-fibrosis
#7
Justin M Oldham, Shwu-Fan Ma, Fernando J Martinez, Kevin J Anstrom, Ganesh Raghu, David A Schwartz, Eleanor Valenzi, Leah Witt, Cathryn Lee, Rekha Vij, Yong Huang, Mary E Strek, Imre Noth
RATIONALE: Idiopathic pulmonary fibrosis (IPF) is a devastating lung disease of unknown etiology. The genes TOLLIP and MUC5B play important roles in lung host defense, which is an immune process influenced by oxidative signaling. Whether polymorphisms in TOLLIP and MUC5B modify the effect of immunosuppressive and antioxidant therapy in individuals with IPF is unknown. OBJECTIVES: To determine whether single-nucleotide polymorphisms (SNPs) within TOLLIP and MUC5B modify the effect of interventions in subjects participating in the Evaluating the Effectiveness of Prednisone, Azathioprine, and N-Acetylcysteine in Patients with Idiopathic Pulmonary Fibrosis (PANTHER-IPF) clinical trial...
December 15, 2015: American Journal of Respiratory and Critical Care Medicine
https://www.readbyqxmd.com/read/26177183/an-official-ats-ers-jrs-alat-clinical-practice-guideline-treatment-of-idiopathic-pulmonary-fibrosis-an-update-of-the-2011-clinical-practice-guideline
#8
Ganesh Raghu, Bram Rochwerg, Yuan Zhang, Carlos A Cuello Garcia, Arata Azuma, Juergen Behr, Jan L Brozek, Harold R Collard, William Cunningham, Sakae Homma, Takeshi Johkoh, Fernando J Martinez, Jeffrey Myers, Shandra L Protzko, Luca Richeldi, David Rind, Moisés Selman, Arthur Theodore, Athol U Wells, Henk Hoogsteden, Holger J Schünemann
BACKGROUND: This document updates the American Thoracic Society/European Respiratory Society/Japanese Respiratory Society/Latin American Thoracic Association guideline on idiopathic pulmonary fibrosis treatment. METHODS: Systematic reviews and, when appropriate, meta-analyses were performed to summarize all available evidence pertinent to our questions. The evidence was assessed using the GRADE (Grading of Recommendations, Assessment, Development and Evaluation) approach and then discussed by a multidisciplinary panel...
July 15, 2015: American Journal of Respiratory and Critical Care Medicine
https://www.readbyqxmd.com/read/25964315/streptococcus-pneumoniae-triggers-progression-of-pulmonary-fibrosis-through-pneumolysin
#9
Sarah Knippenberg, Bianca Ueberberg, Regina Maus, Jennifer Bohling, Nadine Ding, Meritxell Tort Tarres, Heinz-Gerd Hoymann, Danny Jonigk, Nicole Izykowski, James C Paton, Abiodun D Ogunniyi, Sandro Lindig, Michael Bauer, Tobias Welte, Werner Seeger, Andreas Guenther, Thomas H Sisson, Jack Gauldie, Martin Kolb, Ulrich A Maus
RATIONALE: Respiratory tract infections are common in patients suffering from pulmonary fibrosis. The interplay between bacterial infection and fibrosis is characterised poorly. OBJECTIVES: To assess the effect of Gram-positive bacterial infection on fibrosis exacerbation in mice. METHODS: Fibrosis progression in response to Streptococcus pneumoniae was examined in two different mouse models of pulmonary fibrosis. MEASUREMENTS AND MAIN RESULTS: We demonstrate that wild-type mice exposed to adenoviral vector delivery of active transforming growth factor-β1 (TGFß1) or diphteria toxin (DT) treatment of transgenic mice expressing the DT receptor (DTR) under control of the surfactant protein C (SPC) promoter (SPC-DTR) to induce pulmonary fibrosis developed progressive fibrosis following infection with Spn, without exhibiting impaired lung protective immunity against Spn...
July 2015: Thorax
https://www.readbyqxmd.com/read/25876206/we-should-prohibit-warfarin-in-idiopathic-pulmonary-fibrosis
#10
Khuder Alagha, Veronique Secq, Laurie Pahus, Tunde Sofalvi, Alain Palot, Arnaud Bourdin, Pascal Chanez
No abstract text is available yet for this article.
April 15, 2015: American Journal of Respiratory and Critical Care Medicine
https://www.readbyqxmd.com/read/25931482/combination-therapy-in-idiopathic-pulmonary-fibrosis-the-way-ahead-will-be-hard
#11
EDITORIAL
Athol Wells
No abstract text is available yet for this article.
May 2015: European Respiratory Journal: Official Journal of the European Society for Clinical Respiratory Physiology
https://www.readbyqxmd.com/read/25931485/where-do-we-go-from-here-clinical-drug-development-in-idiopathic-pulmonary-fibrosis
#12
EDITORIAL
Harold R Collard
No abstract text is available yet for this article.
May 2015: European Respiratory Journal: Official Journal of the European Society for Clinical Respiratory Physiology
https://www.readbyqxmd.com/read/25439569/combination-therapy-the-future-of-management-for-idiopathic-pulmonary-fibrosis
#13
REVIEW
Wim A Wuyts, Katerina M Antoniou, Keren Borensztajn, Ulrich Costabel, Vincent Cottin, Bruno Crestani, Jan C Grutters, Toby M Maher, Venerino Poletti, Luca Richeldi, Carlo Vancheri, Athol U Wells
Findings from recently published placebo-controlled trials in idiopathic pulmonary fibrosis have established that pirfenidone and nintedanib prevent about 50% of the decline in forced vital capacity typically seen in this disease; future trials are therefore unlikely to use placebo as a control group for ethical reasons. Future clinical assessment will probably include add-on trials in which a new drug is combined with an intervention with established efficacy; this development is in turn likely to herald the use of combination regimens in clinical practice...
November 2014: Lancet Respiratory Medicine
https://www.readbyqxmd.com/read/25745043/mode-of-action-of-nintedanib-in-the-treatment-of-idiopathic-pulmonary-fibrosis
#14
REVIEW
Lutz Wollin, Eva Wex, Alexander Pautsch, Gisela Schnapp, Katrin E Hostettler, Susanne Stowasser, Martin Kolb
Idiopathic pulmonary fibrosis (IPF) is a progressive and ultimately fatal disease characterised by fibrosis of the lung parenchyma and loss of lung function. Although the pathogenic pathways involved in IPF have not been fully elucidated, IPF is believed to be caused by repetitive alveolar epithelial cell injury and dysregulated repair, in which there is uncontrolled proliferation of lung fibroblasts and differentiation of fibroblasts into myofibroblasts, which excessively deposit extracellular matrix (ECM) proteins in the interstitial space...
May 2015: European Respiratory Journal: Official Journal of the European Society for Clinical Respiratory Physiology
https://www.readbyqxmd.com/read/25329221/pirfenidone-reduced-disease-progression-in-idiopathic-pulmonary-fibrosis
#15
COMMENT
Fiona Lake
No abstract text is available yet for this article.
October 21, 2014: Annals of Internal Medicine
https://www.readbyqxmd.com/read/25504994/safety-and-pharmacokinetics-of-nintedanib-and-pirfenidone-in-idiopathic-pulmonary-fibrosis
#16
RANDOMIZED CONTROLLED TRIAL
Takashi Ogura, Hiroyuki Taniguchi, Arata Azuma, Yoshikazu Inoue, Yasuhiro Kondoh, Yoshinori Hasegawa, Masashi Bando, Shinji Abe, Yoshiro Mochizuki, Kingo Chida, Matthias Klüglich, Tsuyoshi Fujimoto, Kotaro Okazaki, Yusuke Tadayasu, Wataru Sakamoto, Yukihiko Sugiyama
A randomised, double-blind, phase II, dose escalation trial was conducted to assess the safety, tolerability and pharmacokinetics of the tyrosine kinase inhibitor nintedanib, alone and when added to ongoing pirfenidone therapy, in Japanese patients with idiopathic pulmonary fibrosis. 50 Japanese patients were randomised to receive nintedanib or placebo in one of three cohorts (nintedanib 50 mg twice daily or 100 mg twice daily for 14 days, or 150 mg twice daily for 28 days). Patients receiving pirfenidone at inclusion were stratified to every nintedanib dose group and placebo...
May 2015: European Respiratory Journal: Official Journal of the European Society for Clinical Respiratory Physiology
https://www.readbyqxmd.com/read/24881079/management-of-idiopathic-pulmonary-fibrosis-selected-case-reports
#17
Michael Kreuter, Peter Kardos, Victor Hoffstein
In 2011, revised international guidelines were issued jointly by the American Thoracic Society, the European Respiratory Society, the Japanese Respiratory Society and the Latin American Thoracic Association, which provide a valuable framework for the diagnosis and management of idiopathic pulmonary fibrosis (IPF). However, due to the complexity of IPF, these guidelines may not comprehensively account for the management of individual IPF patients in clinical practice. We describe three patient cases that were presented and discussed during the 2013 AIR: Advancing IPF Research meeting in Nice, France...
June 2014: European Respiratory Review: An Official Journal of the European Respiratory Society
https://www.readbyqxmd.com/read/25090037/new-therapeutic-targets-in-idiopathic-pulmonary-fibrosis-aiming-to-rein-in-runaway-wound-healing-responses
#18
REVIEW
Neil Ahluwalia, Barry S Shea, Andrew M Tager
Idiopathic pulmonary fibrosis (IPF) is a devastating disease, with a median survival as short as 3 years from the time of diagnosis and no pharmacological therapies yet approved by the U.S. Food and Drug Administration. To address the great unmet need for effective IPF therapy, a number of new drugs have recently been, or are now being, evaluated in clinical trials. The rationales for most of these therapeutic candidates are based on the current paradigm of IPF pathogenesis, in which recurrent injury to the alveolar epithelium is believed to drive aberrant wound healing responses, resulting in fibrosis rather than repair...
October 15, 2014: American Journal of Respiratory and Critical Care Medicine
https://www.readbyqxmd.com/read/24836849/comprehensive-assessment-of-the-long-term-safety-of-pirfenidone-in-patients-with-idiopathic-pulmonary-fibrosis
#19
Dominique Valeyre, Carlo Albera, Williamson Z Bradford, Ulrich Costabel, Talmadge E King, Jonathan A Leff, Paul W Noble, Steven A Sahn, Roland M du Bois
BACKGROUND AND OBJECTIVE: Pirfenidone is an oral antifibrotic agent that is approved in several countries for the treatment of idiopathic pulmonary fibrosis (IPF). We performed a comprehensive analysis of safety across four clinical trials evaluating pirfenidone in patients with IPF. METHODS: All patients receiving pirfenidone 2403 mg/day in the Phase 3 CAPACITY studies (Studies 004 and 006) and all patients receiving at least one dose of pirfenidone in one of two ongoing open-label studies in patients with IPF (Studies 002 and 012) were selected for inclusion...
July 2014: Respirology: Official Journal of the Asian Pacific Society of Respirology
https://www.readbyqxmd.com/read/24836312/a-phase-3-trial-of-pirfenidone-in-patients-with-idiopathic-pulmonary-fibrosis
#20
RANDOMIZED CONTROLLED TRIAL
Talmadge E King, Williamson Z Bradford, Socorro Castro-Bernardini, Elizabeth A Fagan, Ian Glaspole, Marilyn K Glassberg, Eduard Gorina, Peter M Hopkins, David Kardatzke, Lisa Lancaster, David J Lederer, Steven D Nathan, Carlos A Pereira, Steven A Sahn, Robert Sussman, Jeffrey J Swigris, Paul W Noble
BACKGROUND: In two of three phase 3 trials, pirfenidone, an oral antifibrotic therapy, reduced disease progression, as measured by the decline in forced vital capacity (FVC) or vital capacity, in patients with idiopathic pulmonary fibrosis; in the third trial, this end point was not achieved. We sought to confirm the beneficial effect of pirfenidone on disease progression in such patients. METHODS: In this phase 3 study, we randomly assigned 555 patients with idiopathic pulmonary fibrosis to receive either oral pirfenidone (2403 mg per day) or placebo for 52 weeks...
May 29, 2014: New England Journal of Medicine
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