Matthias Griese, Matthias Kappler, Claudia Eismann, Manfred Ballmann, Sibylle Junge, Ernst Rietschel, Silke van Koningsbruggen-Rietschel, Doris Staab, Claudia Rolinck-Werninghaus, Uwe Mellies, Thomas Köhnlein, Thomas Wagner, Susanne König, Helmut Teschler, Hans-Eberhard Heuer, Matthias Kopp, Susanne Heyder, Jutta Hammermann, Peter Küster, Marguerite Honer, Ulrich Mansmann, Ingrid Beck-Speier, Dominik Hartl, Carola Fuchs, Andreas Hector
RATIONALE: Glutathione is the major antioxidant in the extracellular lining fluid of the lungs and depleted in patients with cystic fibrosis (CF). OBJECTIVES: We aimed to assess glutathione delivered by inhalation as a potential treatment for CF lung disease. METHODS: This randomized, double-blind, placebo-controlled trial evaluated inhaled glutathione in subjects with CF 8 years of age and older and FEV1 of 40-90% of predicted. Subjects were randomized to receive 646 mg glutathione in 4 ml (n = 73) or placebo (n = 80) via an investigational eFlow nebulizer every 12 hours for 6 months...
July 1, 2013: American Journal of Respiratory and Critical Care Medicine
Vanja Pekovic-Vaughan, Julie Gibbs, Hikari Yoshitane, Nan Yang, Dharshika Pathiranage, Baoqiang Guo, Aya Sagami, Keiko Taguchi, David Bechtold, Andrew Loudon, Masayuki Yamamoto, Jefferson Chan, Gijsbertus T J van der Horst, Yoshitaka Fukada, Qing-Jun Meng
The disruption of the NRF2 (nuclear factor erythroid-derived 2-like 2)/glutathione-mediated antioxidant defense pathway is a critical step in the pathogenesis of several chronic pulmonary diseases and cancer. While the mechanism of NRF2 activation upon oxidative stress has been widely investigated, little is known about the endogenous signals that regulate the NRF2 pathway in lung physiology and pathology. Here we show that an E-box-mediated circadian rhythm of NRF2 protein is essential in regulating the rhythmic expression of antioxidant genes involved in glutathione redox homeostasis in the mouse lung...
March 15, 2014: Genes & Development
Kundan Iqbal, Clive Kelly
Rheumatoid arthritis (RA) is a systemic autoimmune disease affecting 0.5-1% of the worldwide population. Whilst predominantly causing chronic pain and inflammation in synovial joints, it is also associated with significant extra-articular manifestations in a large proportion of patients. Among the various pulmonary manifestations, interstitial lung disease (ILD), a progressive fibrotic disease of the lung parenchyma, is the commonest and most important, contributing significantly to increased morbidity and mortality...
December 2015: Therapeutic Advances in Musculoskeletal Disease
Amanda T Goodwin, Gisli Jenkins
Idiopathic pulmonary fibrosis (IPF) is a devastating and incurable progressive fibrotic lung condition associated with a significant disease burden. In recent years there has been an exponential increase in the number of preclinical and clinical studies performed in IPF. IPF is defined according to rigid diagnostic criteria; hence, a significant subset of patients with unclassifiable disease has been excluded from these studies. The traditional diagnostic classification of all progressive fibrotic lung diseases uses specific clinical, radiological, and histopathological features to define each condition...
January 2016: Chest
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
Banu A Karimi-Shah, Badrul A Chowdhury
Idiopathic pulmonary fibrosis (IPF) is a devastating disease of unknown cause, and for years the only effective treatment available was lung transplantation. In October 2014, two drugs became available in the United States for patients with IPF when the Food and Drug Administration (FDA) approved..
March 26, 2015: New England Journal of Medicine
Joy D Cogan, Jonathan A Kropski, Min Zhao, Daphne B Mitchell, Lynette Rives, Cheryl Markin, Errine T Garnett, Keri H Montgomery, Wendi R Mason, David F McKean, Julia Powers, Elissa Murphy, Lana M Olson, Leena Choi, Dong-Sheng Cheng, Elizabeth Marchani Blue, Lisa R Young, Lisa H Lancaster, Mark P Steele, Kevin K Brown, Marvin I Schwarz, Tasha E Fingerlin, David A Schwartz, William E Lawson, James E Loyd, Zhongming Zhao, John A Phillips, Timothy S Blackwell
RATIONALE: Up to 20% of cases of idiopathic interstitial pneumonia cluster in families, comprising the syndrome of familial interstitial pneumonia (FIP); however, the genetic basis of FIP remains uncertain in most families. OBJECTIVES: To determine if new disease-causing rare genetic variants could be identified using whole-exome sequencing of affected members from FIP families, providing additional insights into disease pathogenesis. METHODS: Affected subjects from 25 kindreds were selected from an ongoing FIP registry for whole-exome sequencing from genomic DNA...
March 15, 2015: American Journal of Respiratory and Critical Care Medicine
Takashi Kurita, Shinsuke Yasuda, Koji Oba, Toshio Odani, Michihito Kono, Kotaro Otomo, Yuichiro Fujieda, Kenji Oku, Toshiyuki Bohgaki, Olga Amengual, Tetsuya Horita, Tatsuya Atsumi
OBJECTIVE: Interstitial lung diseases (ILDs) complicated with PM or DM are frequently aggressive and refractory to treatment. Recently some reports have suggested the potential benefit of tacrolimus for severe ILD complicated with PM/DM. However, little evidence has yet shown the efficacy of tacrolimus in these settings. The aim of this study was to evaluate the efficacy of tacrolimus as a treatment for PM-/DM-related ILD. METHODS: This retrospective study comprised 49 previously untreated patients diagnosed as PM-/DM-related ILD admitted to Hokkaido University Hospital from January 2000 to July 2013...
January 2015: Rheumatology
Federica Sangiuolo, Ermanno Puxeddu, Gabriella Pezzuto, Francesco Cavalli, Giuliana Longo, Alessia Comandini, Donato Di Pierro, Marco Pallante, Gianluigi Sergiacomi, Giovanni Simonetti, Maurizio Zompatori, Augusto Orlandi, Andrea Magrini, Massimo Amicosante, Francesca Mariani, Monica Losi, Daniela Fraboni, Alberto Bisetti, Cesare Saltini
In idiopathic pulmonary fibrosis (IPF), lung accumulation of excessive extracellular iron and macrophage haemosiderin may suggest disordered iron homeostasis leading to recurring microscopic injury and fibrosing damage. The current study population comprised 89 consistent IPF patients and 107 controls. 54 patients and 11 controls underwent bronchoalveolar lavage (BAL). Haemosiderin was assessed by Perls' stain, BAL fluid malondialdehyde (MDA) by high-performance liquid chromatography, BAL cell iron-dependent oxygen radical generation by fluorimetry and the frequency of hereditary haemochromatosis HFE gene variants by reverse dot blot hybridisation...
February 2015: European Respiratory Journal: Official Journal of the European Society for Clinical Respiratory Physiology
Samy Suissa, Pierre Ernst
No abstract text is available yet for this article.
May 2015: Thorax
Jong Sun Park, Hyo Jin Park, Young Sik Park, Sang-Min Lee, Jae-Joon Yim, Chul-Gyu Yoo, Sung Koo Han, Young Whan Kim
BACKGROUND: Idiopathic pulmonary fibrosis (IPF) is a fatal lung disease of unknown causes. Three proteins (mammalian target of rapamycin, mTOR; zinc finger E-box-binding homeobox 1, ZEB1; Rho-associated, coiled-coil containing protein kinase 1, ROCK1) may be related to pulmonary fibrosis. However, they have not been assessed in human pulmonary fibrosis. We assessed the clinical significance of mTOR, ZEB1, and ROCK1 expression in human pulmonary fibrosis of usual interstitial pneumonia (UIP) pattern...
October 31, 2014: BMC Pulmonary Medicine
Jacob Hull Kristensen, Morten Asser Karsdal, Federica Genovese, Simon Johnson, Birte Svensson, Susanne Jacobsen, Per Hägglund, Diana Julie Leeming
This review discusses the role of extracellular matrix (ECM) quality in the pathogenesis of pulmonary fibrosis (PF). In PF, the highly ordered structure of collagens and elastin within the ECM of the lung is severely disrupted and lacks its original tissue quality. Discussions about the ECM have focused on the role of protein quantity in relation to the progression of PF, while the importance of lung ECM quality, defined by the levels of ECM protein modifications and by the protein distribution in lung tissue, has not been properly addressed...
2014: Respiration; International Review of Thoracic Diseases
Ulrich Costabel, Carlo Albera, Williamson Z Bradford, Phil Hormel, Talmadge E King, Paul W Noble, Steven A Sahn, Dominique Valeyre, Roland M du Bois
BACKGROUND: RECAP is an open-label extension study evaluating pirfenidone in patients with idiopathic pulmonary fibrosis (IPF) who completed the Phase 3 CAPACITY program. OBJECTIVE: We examined the effect of pirfenidone on lung function and survival in patients who were previously randomised to the placebo group in one of the two CAPACITY studies and received pirfenidone for the first time in RECAP. METHODS: Eligible patients received oral pirfenidone 2403 mg/day...
October 20, 2014: Sarcoidosis, Vasculitis, and Diffuse Lung Diseases: Official Journal of WASOG
Haidong Huang, Paul Zarogoulidis, Sofia Lampaki, John Organtzis, Dimitris Petridis, Konstantinos Porpodis, Antonis Papaiwannou, Vasilis Karageorgiou, Georgia Pitsiou, Ioannis Kioumis, Wolfgang Hohenforst-Schmidt, Qiang Li, Kaid Darwiche, Lutz Freitag, Aggeliki Rapti, Konstantinos Zarogoulidis
INTRODUCTION: Pulmonary hypertension (PH) has been identified either as a symptom or a primary entity. Several drugs are already on the market and other are being investigated. Idiopathic pulmonary fibrosis (IPF) is also a disease were several drugs are being investigated. MATERIALS AND METHODS: Three jet nebulizers and three ultrasound nebulizers were used for our experiments with seven different residual cups and four different loadings. Bonsetan, treprostinil, sidenafil and pirfenidone were modified in order to be produced as aerosol in an effort to identify parameters which influence the droplet size production size...
October 2014: Journal of Thoracic Disease
Xia Li, Chang Chen, Jinfu Xu, Jinming Liu, Xianghua Yi, Xiwen Sun, Jingyun Shi
BACKGROUND: Nonspecific interstitial pneumonia (NSIP) has recently been proposed as a histologic type of idiopathic interstitial pneumonia (IIP), but its broad spectrum of clinicopathologic findings and variable prognosis are poorly understood. It is particularly unclear how NSIP and usual interstitial pneumonia (UIP) are related. The present study investigated the clinicopathologic features and prognosis of NSIP, and its differential diagnosis from UIP. METHODS: The clinicopathologic findings and prognosis in 21 NSIP and 18 UIP patients who underwent surgical or video-assisted thoracoscopic lung biopsy were reviewed...
October 2014: Journal of Thoracic Disease
Amritpal Singh Anand, Priya Brian Joseph, Ernest Vera-Vazquez
INTRODUCTION: Diffuse parenchymal lung disease (DPLD) may be idiopathic or may be due to known associations such as autoimmune diseases. The prognosis in cases associated with autoimmune diseases depends on many factors such as histopathology, baseline lung function, auto-antibody level, et cetera. DPLD and its prognosis is worse in patients with overlap syndromes. CASE DESCRIPTION: We present a rare case of a 71 year old Caucasian lady with gradually worsening pulmonary fibrosis secondary to an overlap syndrome with rheumatoid arthritis (RA), scleroderma sine scleroderma (SSS) and anti neutrophil cytoplasmic antibody (ANCA) associated vasculitis...
2014: SpringerPlus
Leona Dowman, Catherine J Hill, Anne E Holland
BACKGROUND: Interstitial lung disease (ILD) is characterised by reduced functional capacity, dyspnoea and exercise-induced hypoxia. Pulmonary rehabilitation, an intervention that includes exercise training, is beneficial for people with other chronic lung conditions; however its effects in ILD have not been well characterised. OBJECTIVES: • To determine whether pulmonary rehabilitation in patients with ILD has beneficial effects on exercise capacity, symptoms, quality of life and survival compared with no pulmonary rehabilitation in patients with ILD...
October 6, 2014: Cochrane Database of Systematic Reviews
Luiz Marcelo W Espoladore, Bianca Barbosa Gregório, Mariana Silva Lima, Carlos Alberto C de Pereira, Maria Raquel Soares, Ester Nei A M Coletta
OBJECTIVE: To evaluate the value of differential cytology of bronchoalveolar lavage (BAL) in common interstitial lung diseases (ILDs) and its relation with high-resolution computed tomography (HRCT) fibrosis. STUDY DESIGN: A retrospective analysis of BAL from 91 patients. The total and percentage of cells were collected and analyzed according to published guidelines. RESULTS: The main diagnoses were connective tissue diseases (n = 24), hypersensitivity pneumonitis (HP) (n = 15), sarcoidosis (n = 12), idiopathic pulmonary fibrosis (IPF) (n = 14), smoking-related interstitial lung diseases (sr-ILDs) (n = 8), aspiration (n = 8), and others (n = 10)...
August 2014: Analytical and Quantitative Cytopathology and Histopathology
Elie El Agha, Saverio Bellusci
Basic research on embryonic lung development offers unique opportunities to make important discoveries that will impact human health. Developmental biologists interested in the molecular control of branching morphogenesis have intensively studied the developing lung, with its complex and seemingly stereotyped ramified structure. However, it is also an organ that is linked to a vast array of clinical problems in humans such as bronchopulmonary dysplasia in premature babies and emphysema, chronic obstructive pulmonary disease, fibrosis, and cancer in adults...
2014: Scientifica
Sven Lehmann, Madlen Uhlemann, Sergey Leontyev, Joerg Seeburger, Jens Garbade, Denis R Merk, Hartmuth B Bittner, Friedrich W Mohr
OBJECTIVES: It is unknown if uni- or bilateral lung transplant is best for treatment of usual idiopathic pulmonary fibrosis. We reviewed our single-center experience comparing both treatments. MATERIALS AND METHODS: Between 2002 and 2011, one hundred thirty-eight patients at our institution underwent a lung transplant. Of these, 58 patients presented with idiopathic pulmonary fibrosis (56.9%) and were the focus of this study. RESULTS: Thirty-nine patients received a single lung transplant and 19 patients a bilateral sequential lung transplant...
October 2014: Experimental and Clinical Transplantation
2014-10-26 23:07:16
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