pulmonary fibrosis

Summary

Summary: Chronic inflammation and progressive fibrosis of the pulmonary alveolar walls, with steadily progressive dyspnea, resulting finally in death from oxygen lack or right heart failure.

Top Publications

  1. ncbi Anticoagulant therapy for idiopathic pulmonary fibrosis
    Hiroshi Kubo
    Department of Geriatric and Respiratory Medicine, Tohoku University School of Medicine, 1 1 Seiryo machi, Aoba ku, Sendai 980 8574, Japan
    Chest 128:1475-82. 2005
  2. pmc Integrating mechanisms of pulmonary fibrosis
    Thomas A Wynn
    Program in Barrier Immunity and Repair and the Immunopathogenesis Section, Laboratory of Parasitic Diseases, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, MD 20892, USA
    J Exp Med 208:1339-50. 2011
  3. ncbi Transforming growth factor beta in tissue fibrosis
    W A Border
    Division of Nephrology, University of Utah School of Medicine, Salt Lake City 84132
    N Engl J Med 331:1286-92. 1994
  4. pmc Circulating fibrocytes traffic to the lungs in response to CXCL12 and mediate fibrosis
    Roderick J Phillips
    Division of Pulmonary and Critical Care Medicine, David Geffen School of Medicine at University of California, Los Angeles, USA
    J Clin Invest 114:438-46. 2004
  5. pmc Mesenchymal stem cell engraftment in lung is enhanced in response to bleomycin exposure and ameliorates its fibrotic effects
    Luis A Ortiz
    Division of Occupational Medicine, Department of Environmental and Occupational Health, University of Pittsburgh, Pittsburgh, PA 15261, USA
    Proc Natl Acad Sci U S A 100:8407-11. 2003
  6. pmc Bone marrow-derived mesenchymal stem cells in repair of the injured lung
    Mauricio Rojas
    Division of Pulmonary, Allergy and Critical Care Medicine, Center for Translational Research of the Lung, Department of Medicine, Emory University School of Medicine and Atlanta Veterans Affairs Medical Center, Atlanta, GA 30322, USA
    Am J Respir Cell Mol Biol 33:145-52. 2005
  7. pmc Bleomycin and IL-1beta-mediated pulmonary fibrosis is IL-17A dependent
    Mark S Wilson
    Immunopathogenesis Section, Laboratory of Parasitic Diseases, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, MD 20892, USA
    J Exp Med 207:535-52. 2010
  8. pmc miR-21 mediates fibrogenic activation of pulmonary fibroblasts and lung fibrosis
    Gang Liu
    Department of Medicine, University of Alabama at Birmingham, Birmingham, AL 35294, USA
    J Exp Med 207:1589-97. 2010
  9. pmc Induction of epithelial-mesenchymal transition in alveolar epithelial cells by transforming growth factor-beta1: potential role in idiopathic pulmonary fibrosis
    Brigham C Willis
    Department of Anesthesiology Critical Care Medicine, Childrens Hospital Los Angeles, California, USA
    Am J Pathol 166:1321-32. 2005
  10. ncbi Incidence and prevalence of idiopathic pulmonary fibrosis
    Ganesh Raghu
    University of Washington Medical Center, Seattle, WA 98195 6522, USA
    Am J Respir Crit Care Med 174:810-6. 2006

Detail Information

Publications259 found, 100 shown here

  1. ncbi Anticoagulant therapy for idiopathic pulmonary fibrosis
    Hiroshi Kubo
    Department of Geriatric and Respiratory Medicine, Tohoku University School of Medicine, 1 1 Seiryo machi, Aoba ku, Sendai 980 8574, Japan
    Chest 128:1475-82. 2005
    To evaluate the effect of anticoagulant therapy on the survival of patients with idiopathic pulmonary fibrosis (IPF).
  2. pmc Integrating mechanisms of pulmonary fibrosis
    Thomas A Wynn
    Program in Barrier Immunity and Repair and the Immunopathogenesis Section, Laboratory of Parasitic Diseases, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, MD 20892, USA
    J Exp Med 208:1339-50. 2011
    b>Pulmonary fibrosis is a highly heterogeneous and lethal pathological process with limited therapeutic options...
  3. ncbi Transforming growth factor beta in tissue fibrosis
    W A Border
    Division of Nephrology, University of Utah School of Medicine, Salt Lake City 84132
    N Engl J Med 331:1286-92. 1994
  4. pmc Circulating fibrocytes traffic to the lungs in response to CXCL12 and mediate fibrosis
    Roderick J Phillips
    Division of Pulmonary and Critical Care Medicine, David Geffen School of Medicine at University of California, Los Angeles, USA
    J Clin Invest 114:438-46. 2004
    ..No studies have demonstrated that these cells actually contribute to fibrosis, however. Pulmonary fibrosis was originally thought to be mediated solely by resident lung fibroblasts...
  5. pmc Mesenchymal stem cell engraftment in lung is enhanced in response to bleomycin exposure and ameliorates its fibrotic effects
    Luis A Ortiz
    Division of Occupational Medicine, Department of Environmental and Occupational Health, University of Pittsburgh, Pittsburgh, PA 15261, USA
    Proc Natl Acad Sci U S A 100:8407-11. 2003
    ..Collectively, these studies demonstrate that murine MSCs home to lung in response to injury, adopt an epithelium-like phenotype, and reduce inflammation and collagen deposition in lung tissue of mice challenged with BLM...
  6. pmc Bone marrow-derived mesenchymal stem cells in repair of the injured lung
    Mauricio Rojas
    Division of Pulmonary, Allergy and Critical Care Medicine, Center for Translational Research of the Lung, Department of Medicine, Emory University School of Medicine and Atlanta Veterans Affairs Medical Center, Atlanta, GA 30322, USA
    Am J Respir Cell Mol Biol 33:145-52. 2005
    ..BMDMSC localize to the injured lung and assume lung cell phenotypes, but protection from injury and fibrosis also involves suppression of inflammation and triggering production of reparative growth factors...
  7. pmc Bleomycin and IL-1beta-mediated pulmonary fibrosis is IL-17A dependent
    Mark S Wilson
    Immunopathogenesis Section, Laboratory of Parasitic Diseases, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, MD 20892, USA
    J Exp Med 207:535-52. 2010
    Idiopathic pulmonary fibrosis (IPF) is a destructive inflammatory disease with limited therapeutic options...
  8. pmc miR-21 mediates fibrogenic activation of pulmonary fibroblasts and lung fibrosis
    Gang Liu
    Department of Medicine, University of Alabama at Birmingham, Birmingham, AL 35294, USA
    J Exp Med 207:1589-97. 2010
    ..production by fibroblasts in response to tissue injury contributes to fibrotic diseases, such as idiopathic pulmonary fibrosis (IPF), a progressive and ultimately fatal process that currently has no cure...
  9. pmc Induction of epithelial-mesenchymal transition in alveolar epithelial cells by transforming growth factor-beta1: potential role in idiopathic pulmonary fibrosis
    Brigham C Willis
    Department of Anesthesiology Critical Care Medicine, Childrens Hospital Los Angeles, California, USA
    Am J Pathol 166:1321-32. 2005
    The hallmark of idiopathic pulmonary fibrosis (IPF) is the myofibroblast, the cellular origin of which in the lung is unknown...
  10. ncbi Incidence and prevalence of idiopathic pulmonary fibrosis
    Ganesh Raghu
    University of Washington Medical Center, Seattle, WA 98195 6522, USA
    Am J Respir Crit Care Med 174:810-6. 2006
    Idiopathic pulmonary fibrosis is a chronic interstitial lung disease of unknown etiology; its epidemiology in the United States has not been well characterized.
  11. pmc Pulmonary expression of interleukin-13 causes inflammation, mucus hypersecretion, subepithelial fibrosis, physiologic abnormalities, and eotaxin production
    Z Zhu
    Section of Pulmonary and Critical Care Medicine, Department of Internal Medicine, Yale University School of Medicine, New Haven, Connecticut 06520 8057, USA
    J Clin Invest 103:779-88. 1999
    ..IL-13 may play an important role in the pathogenesis of similar responses in asthma or other Th2-polarized tissue responses...
  12. pmc Aberrant Wnt/beta-catenin pathway activation in idiopathic pulmonary fibrosis
    Marco Chilosi
    Department of Pathology, University of Verona, Verona, Italy
    Am J Pathol 162:1495-502. 2003
    ..the molecular events that may underpin dysfunctional repair processes that characterize idiopathic pulmonary fibrosis/usual interstitial pneumonia (IPF/UIP), we analyzed the expression patterns of beta-catenin on 20 IPF/UIP ..
  13. ncbi Idiopathic pulmonary fibrosis: prevailing and evolving hypotheses about its pathogenesis and implications for therapy
    M Selman
    Instituto Nacional de Enfermedades Respiratorias, Tlalpan 4502, CP 14080, Mexico DF, Mexico
    Ann Intern Med 134:136-51. 2001
    Idiopathic pulmonary fibrosis is a progressive and usually fatal lung disease characterized by fibroblast proliferation and extracellular matrix remodeling, which result in irreversible distortion of the lung's architecture...
  14. ncbi The integrin alpha v beta 6 binds and activates latent TGF beta 1: a mechanism for regulating pulmonary inflammation and fibrosis
    J S Munger
    Department of Medicine, and Kaplan Cancer Center, New York University School of Medicine, New York 10016 6402, USA
    Cell 96:319-28. 1999
    ..explains why mice lacking this integrin develop exaggerated inflammation and, as we show, are protected from pulmonary fibrosis. These data identify a novel mechanism for locally regulating TGF beta 1 function in vivo by regulating ..
  15. ncbi Unusual inflammatory and fibrogenic pulmonary responses to single-walled carbon nanotubes in mice
    Anna A Shvedova
    Health Effects Laboratory Div, NIOSH, Morgantown, WV, USA
    Am J Physiol Lung Cell Mol Physiol 289:L698-708. 2005
    ..Equal doses of ultrafine carbon black particles or fine crystalline silica (SiO2) did not induce granulomas or alveolar wall thickening and caused a significantly weaker pulmonary inflammation and damage...
  16. ncbi Molecular targets in pulmonary fibrosis: the myofibroblast in focus
    Chris J Scotton
    Centre for Respiratory Research, University College London, Rayne Institute, London, UK
    Chest 132:1311-21. 2007
    Idiopathic pulmonary fibrosis (IPF) is one of a group of interstitial lung diseases that are characterized by excessive matrix deposition and destruction of the normal lung architecture...
  17. ncbi Modeling pulmonary fibrosis with bleomycin
    Marios A Mouratis
    Institute of Immunology, Biomedical Sciences Research Center Alexander Fleming, Athens, Greece
    Curr Opin Pulm Med 17:355-61. 2011
    Idiopathic pulmonary fibrosis is a chronic pulmonary disease of unknown origin ultimately leading to death. No treatment exists yet and animal models have been employed in order to elucidate its etiopathogenesis...
  18. pmc Pulmonary fibrosis: patterns and perpetrators
    Paul W Noble
    Division of Pulmonary, Allergy, and Critical Care Medicine, Department of Medicine, Duke University School of Medicine, Durham, NC, USA
    J Clin Invest 122:2756-62. 2012
    b>Pulmonary fibrosis occurs in a variety of clinical settings, constitutes a major cause of morbidity and mortality, and represents an enormous unmet medical need...
  19. pmc Alveolar epithelial cell mesenchymal transition develops in vivo during pulmonary fibrosis and is regulated by the extracellular matrix
    Kevin K Kim
    Department of Medicine and Cardiovascular Research Institute, University of California, San Francisco, 94143, USA
    Proc Natl Acad Sci U S A 103:13180-5. 2006
    ..in lung epithelial cells were generated, and their fates were followed in an established model of pulmonary fibrosis, overexpression of active TGF-beta1...
  20. pmc WNT1-inducible signaling protein-1 mediates pulmonary fibrosis in mice and is upregulated in humans with idiopathic pulmonary fibrosis
    Melanie Konigshoff
    Department of Medicine, University of Giessen Lung Center, Giessen, Germany
    J Clin Invest 119:772-87. 2009
    Idiopathic pulmonary fibrosis (IPF) is characterized by distorted lung architecture and loss of respiratory function...
  21. pmc Up-regulation and profibrotic role of osteopontin in human idiopathic pulmonary fibrosis
    Annie Pardo
    Facultad de Ciencias, Universidad Nacional Autonoma de Mexico, Mexico City, Mexico
    PLoS Med 2:e251. 2005
    Idiopathic pulmonary fibrosis (IPF) is a progressive and lethal disorder characterized by fibroproliferation and excessive accumulation of extracellular matrix in the lung.
  22. ncbi Idiopathic pulmonary fibrosis
    T J Gross
    Department of Internal Medicine, University of Iowa College of Medicine and Veterans Affairs Medical Center, Iowa City, USA
    N Engl J Med 345:517-25. 2001
    Idiopathic pulmonary fibrosis is a rapidly progressive illness of unknown cause characterized by sequential acute lung injury with subsequent scarring and end-stage lung disease...
  23. ncbi High-dose acetylcysteine in idiopathic pulmonary fibrosis
    Maurits Demedts
    University Hospital, Katholieke Universiteit Leuven, Leuven, Belgium
    N Engl J Med 353:2229-42. 2005
    Idiopathic pulmonary fibrosis is a chronic progressive disorder with a poor prognosis.
  24. ncbi Double-blind, placebo-controlled trial of pirfenidone in patients with idiopathic pulmonary fibrosis
    Arata Azuma
    Fourth Department of Internal Medicine, Nippon Medical School, Tokyo, Japan
    Am J Respir Crit Care Med 171:1040-7. 2005
    Idiopathic pulmonary fibrosis (IPF) is a fatal disorder without an effective therapy to date. In a double-blind, randomized, placebo-controlled trial, 107 patients were prospectively evaluated for efficacy of a novel compound, pirfenidone...
  25. pmc Multiple stromal populations contribute to pulmonary fibrosis without evidence for epithelial to mesenchymal transition
    Jason R Rock
    Department of Cell Biology, Duke University Medical Center, Durham, NC 27710, USA
    Proc Natl Acad Sci U S A 108:E1475-83. 2011
    There are currently few treatment options for pulmonary fibrosis. Innovations may come from a better understanding of the cellular origin of the characteristic fibrotic lesions...
  26. ncbi The role of transforming growth factor beta in lung development and disease
    Ulrike Bartram
    University Children s Hospital, Josef Schnewider Strasse 2, 97080 Wuerzburg, Germany
    Chest 125:754-65. 2004
    ..TGFbeta plays a key role in mediating fibrotic tissue remodeling by increasing the production and decreasing the degradation of connective tissue via several mechanisms...
  27. ncbi Partial inhibition of integrin alpha(v)beta6 prevents pulmonary fibrosis without exacerbating inflammation
    Gerald S Horan
    Department of Exploratory Biology, Biogen Idec, 12 Cambridge Center, Cambridge, MA 02142, USA
    Am J Respir Crit Care Med 177:56-65. 2008
    ..growth factor (TGF)-beta has a central role in driving many of the pathological processes that characterize pulmonary fibrosis. Inhibition of the integrin alpha(v)beta6, a key activator of TGF-beta in lung, is an attractive therapeutic ..
  28. pmc Inhibition of platelet-derived growth factor signaling attenuates pulmonary fibrosis
    Amir Abdollahi
    Department of Radiation Oncology, German Cancer Research Center, Heidelberg 69120, Germany
    J Exp Med 201:925-35. 2005
    b>Pulmonary fibrosis is the consequence of a variety of diseases with no satisfying treatment option. Therapy-induced fibrosis also limits the efficacy of chemotherapy and radiotherapy in numerous cancers...
  29. pmc Human umbilical cord mesenchymal stem cells reduce fibrosis of bleomycin-induced lung injury
    Yuben Moodley
    Department of Medicine, Monash Immunology and Stem Cell Laboratories, School of Biomedical Sciences, Monash University, Melbourne, Australia
    Am J Pathol 175:303-13. 2009
    ..The results of this study suggest that uMSCs have antifibrotic properties and may augment lung repair if used to treat acute respiratory distress syndrome...
  30. pmc Pulmonary fibrosis: pathogenesis, etiology and regulation
    M S Wilson
    Immunopathogenesis Section, Laboratory of Parasitic Diseases, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, Maryland 20892, USA
    Mucosal Immunol 2:103-21. 2009
    b>Pulmonary fibrosis and architectural remodeling of tissues can severely disrupt lung function, often with fatal consequences...
  31. pmc Short telomeres are a risk factor for idiopathic pulmonary fibrosis
    Jonathan K Alder
    Department of Oncology, School of Medicine, Johns Hopkins University, Baltimore, MD 21287, USA
    Proc Natl Acad Sci U S A 105:13051-6. 2008
    ..Idiopathic pulmonary fibrosis (IPF) is the most common of IIPs and shares with IIPs an increased incidence with age and unexplained ..
  32. pmc Bone marrow-derived progenitor cells in pulmonary fibrosis
    Naozumi Hashimoto
    Department of Pathology, University of Michigan, Ann Arbor, Michigan 48109, USA
    J Clin Invest 113:243-52. 2004
    The origin of fibroblasts in pulmonary fibrosis is assumed to be intrapulmonary, but their extrapulmonary origin and especially derivation from bone marrow (BM) progenitor cells has not been ruled out...
  33. ncbi Telomerase mutations in families with idiopathic pulmonary fibrosis
    Mary Y Armanios
    Department of Oncology, Johns Hopkins University School of Medicine, Baltimore, MD 21231, USA
    N Engl J Med 356:1317-26. 2007
    Idiopathic pulmonary fibrosis is progressive and often fatal; causes of familial clustering of the disease are unknown...
  34. pmc Prostaglandin E2 and the pathogenesis of pulmonary fibrosis
    Paul D Bozyk
    Department of Internal Medicine, University of Michigan, Ann Arbor, Michigan, USA
    Am J Respir Cell Mol Biol 45:445-52. 2011
    ..This review highlights these recent advances and offers new insights into the potential ways that PGE(2) and its downstream signals can be regulated for therapeutic benefit in a disease that has no validated treatment options...
  35. pmc Profibrotic role of miR-154 in pulmonary fibrosis
    Jadranka Milosevic
    Dorothy P and Richard P Simmons Center for Interstitial Lung Disease, Pulmonary, Allergy and Critical Care Medicine, University of Pittsburgh, Pittsburgh, PA 15261, USA
    Am J Respir Cell Mol Biol 47:879-87. 2012
    In this study, we explored the regulation and the role of up-regulated microRNAs in idiopathic pulmonary fibrosis (IPF), a progressive interstitial lung disease of unknown origin...
  36. pmc Telomere shortening in familial and sporadic pulmonary fibrosis
    Jennifer T Cronkhite
    Eugene McDermott Center for Human Growth and Development, University of Texas Southwestern Medical Center, Dallas, TX 75390 8591, USA
    Am J Respir Crit Care Med 178:729-37. 2008
    ..Heterozygous mutations in the coding regions of the telomerase genes, TERT and TERC, have been found in familial and sporadic cases of idiopathic interstitial pneumonia. All affected patients with mutations have short telomeres...
  37. pmc Acute exacerbations of idiopathic pulmonary fibrosis
    Harold R Collard
    Department of Medicine, San Francisco General Hospital, University of California, USA
    Am J Respir Crit Care Med 176:636-43. 2007
    The natural history of idiopathic pulmonary fibrosis (IPF) has been characterized as a steady, predictable decline in lung function over time...
  38. ncbi A vicious circle of alveolar macrophages and fibroblasts perpetuates pulmonary fibrosis via CCL18
    Antje Prasse
    Department of Pneumology, University Hospital, Killianstr 5, 79106 Freiburg, Germany
    Am J Respir Crit Care Med 173:781-92. 2006
    ..Recently, models of macrophage activation have been revised. Macrophages stimulated with Th2 cytokines have been classified as alternatively activated...
  39. pmc Fibrocyte CXCR4 regulation as a therapeutic target in pulmonary fibrosis
    Borna Mehrad
    Department of Medicine, Division of Pulmonary and Critical Care Medicine, University of Virginia, Charlottesville, VA 22908, United States
    Int J Biochem Cell Biol 41:1708-18. 2009
    ..that the regulation of CXCR4 expression on fibrocytes mediates their influx to the lung in the context of pulmonary fibrosis and that pharmacologic inhibition of this process results in attenuated disease severity...
  40. ncbi First in class, potent, and orally bioavailable NADPH oxidase isoform 4 (Nox4) inhibitors for the treatment of idiopathic pulmonary fibrosis
    Benoît Laleu
    Genkyotex, S A, 14 Chemin des Aulx, CH 1228 Plan les Ouates, Switzerland
    J Med Chem 53:7715-30. 2010
    ..of NADPH oxidase isoform 4 (Nox4), an enzyme implicated in several pathologies, in particular idiopathic pulmonary fibrosis, a life-threatening and orphan disease...
  41. pmc miR-29 is a major regulator of genes associated with pulmonary fibrosis
    Leah Cushing
    Pulmonary Center, Department of Medicine, Boston University School of Medicine, Massachusetts, USA
    Am J Respir Cell Mol Biol 45:287-94. 2011
    ..of interstitial cells of the alveolar wall, pleura, and at the entrance of the alveolar duct, known sites of pulmonary fibrosis. miR-29 levels inversely correlated with the expression levels of profibrotic target genes and the severity ..
  42. pmc IL-1R1/MyD88 signaling and the inflammasome are essential in pulmonary inflammation and fibrosis in mice
    Pamela Gasse
    Laboratory of Molecular Immunology and Embryology, University of Orleans and CNRS, Orleans, France
    J Clin Invest 117:3786-99. 2007
    ..In conclusion, bleomycin-induced lung pathology required the inflammasome and IL-1R1/MyD88 signaling, and IL-1 represented a critical effector of pathology and therapeutic target of chronic lung inflammation and fibrosis...
  43. pmc Functional Wnt signaling is increased in idiopathic pulmonary fibrosis
    Melanie Konigshoff
    Department of Medicine, University of Giessen Lung Center, University of Giessen, Giessen, Germany
    PLoS ONE 3:e2142. 2008
    Idiopathic pulmonary fibrosis (IPF) is a fatal lung disease, characterized by distorted lung architecture and loss of respiratory function...
  44. pmc Transient expression of IL-1beta induces acute lung injury and chronic repair leading to pulmonary fibrosis
    M Kolb
    Department of Pathology and Molecular Medicine and Centre for Gene Therapeutics, McMaster University, Hamilton, Ontario, Canada
    J Clin Invest 107:1529-36. 2001
    ..IL-1beta should be considered a valid target for therapeutic intervention in diseases associated with fibrosis and tissue remodeling...
  45. pmc Gene expression analysis reveals matrilysin as a key regulator of pulmonary fibrosis in mice and humans
    Fengrong Zuo
    Roche Bioscience, Palo Alto, CA 94304 Functional Genomics, and Institute of Respiratory Medicine, Sheba Medical Center, Tel Hashomer, 52621 Israel
    Proc Natl Acad Sci U S A 99:6292-7. 2002
    b>Pulmonary fibrosis is a progressive and largely untreatable group of disorders that affects up to 100,000 people on any given day in the United States...
  46. ncbi Blocking IL-17A promotes the resolution of pulmonary inflammation and fibrosis via TGF-beta1-dependent and -independent mechanisms
    Su Mi
    Molecular Immunology and Pharmacology Laboratory, Institute of Materia Medica, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing 100050, People s Republic of China
    J Immunol 187:3003-14. 2011
    b>Pulmonary fibrosis is the pathologic basis for a variety of incurable human chronic lung diseases...
  47. ncbi Inhibition of pulmonary fibrosis by the chemokine IP-10/CXCL10
    Andrew M Tager
    Center for Immunology and Inflammatory Diseases, Division of Rheumatology, Allergy and Immunology, Massachusetts General Hospital, Building 149 8301, 13th Street, Charlestown, MA 02129, USA
    Am J Respir Cell Mol Biol 31:395-404. 2004
    b>Pulmonary fibrosis is an enigmatic and devastating disease with few treatment options, now thought to result from abnormal wound healing in the lung in response to injury...
  48. pmc Thy-1 promoter hypermethylation: a novel epigenetic pathogenic mechanism in pulmonary fibrosis
    Yan Y Sanders
    Department of Pediatrics, Division of Pulmonary Medicine, University of Alabama at Birmingham, Birmingham, AL 35233, USA
    Am J Respir Cell Mol Biol 39:610-8. 2008
    Mechanisms regulating myofibroblastic differentiation of fibroblasts within fibroblastic foci in idiopathic pulmonary fibrosis (IPF) remain unclear...
  49. ncbi Uric acid is a danger signal activating NALP3 inflammasome in lung injury inflammation and fibrosis
    Pamela Gasse
    University of Orleans and CNRS, Orleans, France
    Am J Respir Crit Care Med 179:903-13. 2009
    ..The molecular mechanisms by which lung injury triggers IL-1beta production, inflammation, and fibrosis remain poorly understood...
  50. pmc The bleomycin animal model: a useful tool to investigate treatment options for idiopathic pulmonary fibrosis?
    Antje Moeller
    Department of Medicine, Firestone Institute for Respiratory Health, McMaster University, Hamilton, Ontario, Canada
    Int J Biochem Cell Biol 40:362-82. 2008
    Different animal models of pulmonary fibrosis have been developed to investigate potential therapies for idiopathic pulmonary fibrosis (IPF). The most common is the bleomycin model in rodents (mouse, rat and hamster)...
  51. pmc Combined pulmonary fibrosis and emphysema syndrome: a review
    Matthew D Jankowich
    Vascular Research Laboratory, Providence VA Medical Center, Office 158L, 830 Chalkstone Ave, Providence, RI 02908, USA
    Chest 141:222-31. 2012
    There is increasing clinical, radiologic, and pathologic recognition of the coexistence of emphysema and pulmonary fibrosis in the same patient, resulting in a clinical syndrome known as combined pulmonary fibrosis and emphysema (CPFE) ..
  52. pmc Axin pathway activity regulates in vivo pY654-β-catenin accumulation and pulmonary fibrosis
    Arnau Ulsamer
    Pulmonary and Critical Care Division, Department of Medicine and Cardiovascular Research Institute, University of California, San Francisco, California 94143, USA
    J Biol Chem 287:5164-72. 2012
    ....
  53. ncbi Modern concepts on the role of inflammation in pulmonary fibrosis
    Robert J Homer
    Department of Pathology, Yale School of Medicine, New Haven, Connecticut 06520 8070, USA
    Arch Pathol Lab Med 135:780-8. 2011
    Idiopathic pulmonary fibrosis is a uniformly lethal disease with limited biomarkers and no proven therapeutic intervention short of lung transplantation...
  54. ncbi Chronic hypersensitivity pneumonitis: differentiation from idiopathic pulmonary fibrosis and nonspecific interstitial pneumonia by using thin-section CT
    C Isabela S Silva
    Department of Radiology, Vancouver General Hospital, University of British Columbia, 3350 950 W 10th Ave, Vancouver, BC, Canada V5Z 4E3
    Radiology 246:288-97. 2008
    ..thin-section computed tomography (CT) in distinguishing chronic hypersensitivity pneumonitis (HP) from idiopathic pulmonary fibrosis (IPF) and nonspecific interstitial pneumonia (NSIP), with histologic results as the reference standard.
  55. ncbi Role of epithelial cells in idiopathic pulmonary fibrosis: from innocent targets to serial killers
    Moises Selman
    Instituto Nacional de Enfermedades Respiratorias, Tlalpan 4502 CP 14080, Mexico DF, Mexico
    Proc Am Thorac Soc 3:364-72. 2006
    Idiopathic pulmonary fibrosis (IPF), a progressive and relentless lung scarring of unknown etiology, has been recognized as the most lethal interstitial lung disease...
  56. pmc Contribution of epithelial-derived fibroblasts to bleomycin-induced lung fibrosis
    Harikrishna Tanjore
    Division of Allergy, Pulmonary, and Critical Care Medicine, Department of Medicine, Vanderbilt University School of Medicine, Nashville, TN 37232 2650, USA
    Am J Respir Crit Care Med 180:657-65. 2009
    ..Lung fibroblasts are key mediators of fibrosis resulting in accumulation of excessive interstitial collagen and extracellular matrix, but their origins are not well defined...
  57. ncbi Attenuation of bleomycin-induced pulmonary fibrosis by follistatin
    Fumiaki Aoki
    Institute for Molecular and Cellular Regulation, Gunma University, Maebashi 371 8512, Japan
    Am J Respir Crit Care Med 172:713-20. 2005
    ..Activins are members of the transforming growth factor-beta superfamily thought to be involved in repair processes after tissue injury...
  58. ncbi Blockade of the Wnt/β-catenin pathway attenuates bleomycin-induced pulmonary fibrosis
    Tae Hyung Kim
    Department of Internal Medicine, Hanyang University College of Medicine, Seoul, Korea
    Tohoku J Exp Med 223:45-54. 2011
    Idiopathic pulmonary fibrosis (IPF) is a progressive fibrotic lung disease and characterized by abnormal growth of fibroblasts and lung scarring...
  59. pmc Adult-onset pulmonary fibrosis caused by mutations in telomerase
    Kalliopi D Tsakiri
    McDermott Center for Human Growth and Development, University of Texas Southwestern Medical Center, 5323 Harry Hines Boulevard, Dallas, TX 75390, USA
    Proc Natl Acad Sci U S A 104:7552-7. 2007
    Idiopathic pulmonary fibrosis (IPF) is an adult-onset, lethal, scarring lung disease of unknown etiology. Some individuals with IPF have a familial disorder that segregates as a dominant trait with incomplete penetrance...
  60. ncbi Epithelial-mesenchymal interactions in pulmonary fibrosis
    Harold A Chapman
    Department of Medicine, Cardiovascular Research Institute, University of California, San Francisco, 94143, USA
    Annu Rev Physiol 73:413-35. 2011
    ..Understanding specific signaling mechanisms driving the mesenchymal response to TGFβ1 may reveal therapeutics to attenuate fibrogenesis yet preserve the important homeostatic functions of TGFβ1...
  61. pmc Global analysis of gene expression in pulmonary fibrosis reveals distinct programs regulating lung inflammation and fibrosis
    N Kaminski
    Lung Biology Center, Department of Anesthesia, University of California, San Francisco, CA 94143, USA
    Proc Natl Acad Sci U S A 97:1778-83. 2000
    The molecular mechanisms of pulmonary fibrosis are poorly understood...
  62. ncbi Circulating peripheral blood fibrocytes in human fibrotic interstitial lung disease
    Borna Mehrad
    Division of Pulmonary and Critical Care Medicine, University of Virginia, Charlottesville, VA, USA
    Biochem Biophys Res Commun 353:104-8. 2007
    ..work has shown fibrocytes to traffic to the lung via the CXCL12-CXCR4 chemokine axis in an animal model of pulmonary fibrosis. We therefore assessed the relevance of fibrocytes in patients with fibrotic interstitial lung disease...
  63. ncbi Treatment of idiopathic pulmonary fibrosis with etanercept: an exploratory, placebo-controlled trial
    Ganesh Raghu
    Division of Pulmonary and Critical Care Medicine, Campus Box 356175, University of Washington, Seattle, WA 98195 6522, USA
    Am J Respir Crit Care Med 178:948-55. 2008
    An efficacious medical therapy for idiopathic pulmonary fibrosis (IPF) remains elusive.
  64. ncbi The lysophosphatidic acid receptor LPA1 links pulmonary fibrosis to lung injury by mediating fibroblast recruitment and vascular leak
    Andrew M Tager
    Center for Immunology and Inflammatory Diseases, Division of Rheumatology, Allergy and Immunology, Massachusetts General Hospital, Harvard Medical School, CNY149 8301, 149 13th Street, Charlestown, Massachusetts 02129 USA
    Nat Med 14:45-54. 2008
    Aberrant wound-healing responses to injury have been implicated in the development of pulmonary fibrosis, but the mediators directing these pathologic responses have yet to be fully identified...
  65. ncbi TGF-beta, Smad3 and the process of progressive fibrosis
    J Gauldie
    Department of Pathology and Molecular Medicine, McMaster University, 1200 Main St West, Hamilton, ON, Canada L8N 3Z5
    Biochem Soc Trans 35:661-4. 2007
    ..These results suggest that mechanisms of progressive fibrosis involve factors presented within the context of the matrix that define the microenvironment for progressive matrix deposition...
  66. ncbi Ly6Chi monocytes direct alternatively activated profibrotic macrophage regulation of lung fibrosis
    Michael A Gibbons
    Medical Research Council University of Edinburgh Centre for Inflammation Research, Queen s Medical Research Institute, Edinburgh, United Kingdom
    Am J Respir Crit Care Med 184:569-81. 2011
    Idiopathic pulmonary fibrosis (IPF) is a devastating disease. Antiinflammatory therapies, including corticosteroids, are of no benefit. The role of monocytes and macrophages is therefore controversial.
  67. ncbi Combined pulmonary fibrosis and emphysema: a distinct underrecognised entity
    V Cottin
    Groupe d Etudes et de Recherche sur les Maladies Orphelines Pulmonaires, Hopital Cardiovasculaire et Pneumologique Louis Pradel, Universite Claude Bernard, Lyon Bron, France
    Eur Respir J 26:586-93. 2005
    The syndrome resulting from combined pulmonary fibrosis and emphysema has not been comprehensively described...
  68. ncbi Alternatively activated alveolar macrophages in pulmonary fibrosis-mediator production and intracellular signal transduction
    Dmitri V Pechkovsky
    Department of Pneumology, Medical Center, Albert Ludwigs University, Freiburg, Germany
    Clin Immunol 137:89-101. 2010
    ..These results suggest an important role for M2 polarized AM in the pathogenesis of pulmonary fibrosis and indicate that both IL-4 and IL-10 account for human AM phenotype shift to M2, as seen in patients with ..
  69. pmc An essential role for fibronectin extra type III domain A in pulmonary fibrosis
    Andres F Muro
    Division of Pulmonary and Critical Care Medicine, University of Michigan Medical School, 6301 MSRB III 0642, 1150 W Medical Center Drive, Ann Arbor, MI 48109 0642, USA
    Am J Respir Crit Care Med 177:638-45. 2008
    ..Fibronectin containing extra type III domain A (EDA) is implicated in the regulation of wound healing. EDA-containing fibronectin is deposited during wound repair, and its presence precedes that of collagen...
  70. ncbi The myofibroblast in pulmonary fibrosis
    Sem H Phan
    Department of Pathology, University of Michigan, Ann Arbor, MI 48109 0602, USA
    Chest 122:286S-289S. 2002
    The pathogenesis of pulmonary fibrosis remains incompletely understood...
  71. pmc Dietary curcumin increases antioxidant defenses in lung, ameliorates radiation-induced pulmonary fibrosis, and improves survival in mice
    James C Lee
    Pulmonary, Allergy and Critical Care Division, Department of Medicine, University of Pennsylvania Medical Center, Philadelphia, Pennsylvania 19104, USA
    Radiat Res 173:590-601. 2010
    ..by evaluating bronchoalveolar lavage (BAL) fluid content for proteins, neutrophils and at 4 months for pulmonary fibrosis. In a separate series of experiments, an orthotopic model of lung cancer using intravenously injected Lewis ..
  72. pmc Targeted injury of type II alveolar epithelial cells induces pulmonary fibrosis
    Thomas H Sisson
    Division of Pulmonary and Critical Care Medicine, Department of Internal Medicine, University of Michigan Medical Center, 1150 West Medical Center Drive, 6301 MSRB III, Ann Arbor, MI 48109 5642, USA
    Am J Respir Crit Care Med 181:254-63. 2010
    Ineffective repair of a damaged alveolar epithelium has been postulated to cause pulmonary fibrosis. In support of this theory, epithelial cell abnormalities, including hyperplasia, apoptosis, and persistent denudation of the alveolar ..
  73. pmc Inhibition of Wnt/beta-catenin/CREB binding protein (CBP) signaling reverses pulmonary fibrosis
    William R Henderson
    Center for Allergy and Inflammation and Department of Medicine, University of Washington, Seattle, WA 98109, USA
    Proc Natl Acad Sci U S A 107:14309-14. 2010
    Idiopathic pulmonary fibrosis (IPF)/usual interstitial pneumonia is a ravaging condition of progressive lung scarring and destruction...
  74. ncbi MicroRNAs in idiopathic pulmonary fibrosis
    Kusum V Pandit
    Dorothy P and Richard P Simmons Center for Interstitial Lung Disease, Division of Pulmonary, Allergy and Critical Care Medicine, University of Pittsburgh School of Medicine, Pittsburgh, PA, USA
    Transl Res 157:191-9. 2011
    In this review, we describe the recent advances in the understanding of the role of microRNAs in idiopathic pulmonary fibrosis (IPF), a chronic progressive and lethal fibrotic lung disease...
  75. pmc Bleomycin-induced pulmonary fibrosis in transgenic mice that either lack or overexpress the murine plasminogen activator inhibitor-1 gene
    D T Eitzman
    Division of Cardiology, University of Michigan Medical Center, Ann Arbor 48109, USA
    J Clin Invest 97:232-7. 1996
    ..injured tissues to normal, reduced fibrinolytic activity may contribute to the subsequent development of pulmonary fibrosis. To examine the relationship between the fibrinolytic system and pulmonary fibrosis, lung inflammation was ..
  76. ncbi Fibrocytes are a potential source of lung fibroblasts in idiopathic pulmonary fibrosis
    Annika Andersson-Sjöland
    Department of Clinical Medical Science, Division of Respiratory Medicine and Allergology, Lund University, Sweden
    Int J Biochem Cell Biol 40:2129-40. 2008
    Idiopathic pulmonary fibrosis is characterized by the accumulation of fibroblasts/myofibroblasts and aberrant remodeling of the lung parenchyma. However, the sources of fibroblasts in IPF lungs are unclear...
  77. pmc Syndromes of telomere shortening
    Mary Armanios
    Department of Oncology, Johns Hopkins University School of Medicine, Baltimore, Maryland 21285, USA
    Annu Rev Genomics Hum Genet 10:45-61. 2009
    ..More recently, mutations in telomerase components were identified in adults with idiopathic pulmonary fibrosis. These findings have revealed that the spectrum of telomere-mediated disease is broad and includes clinical ..
  78. pmc Th17-polarized immune response in a murine model of hypersensitivity pneumonitis and lung fibrosis
    Philip L Simonian
    Department of Medicine, University of Colorado Denver, Denver, CO 80262, USA
    J Immunol 182:657-65. 2009
    ..lung disease characterized by a diffuse mononuclear cell infiltrate in the lung that can progress to pulmonary fibrosis with chronic exposure to an inhaled Ag...
  79. pmc Epithelial origin of myofibroblasts during fibrosis in the lung
    Brigham C Willis
    Division of Pulmonary and Critical Care Medicine, University of Southern California, IRD 620, 2020 Zonal Avenue, Los Angeles, CA 90033, USA
    Proc Am Thorac Soc 3:377-82. 2006
    An understanding of the mechanisms underlying pulmonary fibrosis remains elusive...
  80. pmc Epithelial endoplasmic reticulum stress and apoptosis in sporadic idiopathic pulmonary fibrosis
    Martina Korfei
    University of Giessen Lung Center, Department of Internal Medicine II, Klinikstrasse 36, 35392 Giessen, Germany
    Am J Respir Crit Care Med 178:838-46. 2008
    The molecular pathomechanisms underlying idiopathic pulmonary fibrosis (IPF) are elusive, but chronic epithelial injury has recently been suggested as key event.
  81. pmc Endothelial-mesenchymal transition in bleomycin-induced pulmonary fibrosis
    Naozumi Hashimoto
    Department of Respiratory Medicine, Nagoya University Graduate School of Medicine, Showa Ku, Japan
    Am J Respir Cell Mol Biol 43:161-72. 2010
    The pathological hallmark lesions in idiopathic pulmonary fibrosis are the fibroblastic foci, in which fibroblasts are thought to be involved in the tissue remodeling, matrix deposition, and cross-talk with alveolar epithelium...
  82. ncbi Changes in clinical and physiologic variables predict survival in idiopathic pulmonary fibrosis
    Harold R Collard
    Division of Pulmonary Sciences and Critical Care Medicine, Department of Medicine, University of Colorado Health Sciences Center, 1400 Jackson Street, Room F107, Denver, CO 80206, USA
    Am J Respir Crit Care Med 168:538-42. 2003
    There is significant heterogeneity in survival time among patients with idiopathic pulmonary fibrosis. Studies of baseline clinical and physiologic variables as predictors of survival time have reported inconsistent results...
  83. pmc MMP1 and MMP7 as potential peripheral blood biomarkers in idiopathic pulmonary fibrosis
    Ivan O Rosas
    Dorothy P and Richard P Simmons Center for Interstitial Lung Diseases, Division of Pulmonary, Allergy and Critical Care Medicine, University of Pittsburgh School ofMedicine, Pittsburgh, Pennsylvania, United States of America
    PLoS Med 5:e93. 2008
    Idiopathic pulmonary fibrosis (IPF) is a chronic progressive fibrotic lung disease associated with substantial morbidity and mortality...
  84. ncbi A placebo-controlled trial of interferon gamma-1b in patients with idiopathic pulmonary fibrosis
    Ganesh Raghu
    Division of Pulmonary Medicine, University of Washington, Seattle 98195, USA
    N Engl J Med 350:125-33. 2004
    Idiopathic pulmonary fibrosis is a progressive, fatal disease with no known efficacious therapy.
  85. ncbi Idiopathic pulmonary fibrosis: outcome in relation to smoking status
    Katerina M Antoniou
    Interstitial Lung Disease Unit, c o Emmanuel Kaye Building, Manresa Road, London SW3 6LR, UK
    Am J Respir Crit Care Med 177:190-4. 2008
    The pathogenic importance of smoking status in idiopathic pulmonary fibrosis (IPF) is uncertain. In theory, increased oxidative stress in current and former smokers might promote disease progression...
  86. ncbi Mortality from pulmonary fibrosis increased in the United States from 1992 to 2003
    Amy L Olson
    Division of Pulmonary Sciences and Critical Care Medicine, University of Colorado Health Sciences Center, Denver Colorado 80262, USA
    Am J Respir Crit Care Med 176:277-84. 2007
    From the late 1970s to the early 1990s, studies found that mortality rates for pulmonary fibrosis were increasing. Recent data for mortality from pulmonary fibrosis are unavailable.
  87. pmc Inhibition of airway remodeling in IL-5-deficient mice
    Jae Youn Cho
    Department of Medicine, University of California San Diego, La Jolla, California 92093 0635, USA
    J Clin Invest 113:551-60. 2004
    ..Increased airway epithelial expression of alphaVbeta6 integrin may contribute to the increased activation of latent TGF-beta. These results suggest an important role for IL-5, eosinophils, alphaVbeta6, and TGF-beta in airway remodeling...
  88. pmc The role of CXC chemokines in pulmonary fibrosis
    Robert M Strieter
    Division of Pulmonary and Critical Care Medicine, Department of Medicine, University of Virginia School of Medicine, Charlottesville, VA 22908, USA
    J Clin Invest 117:549-56. 2007
    ..These functions of CXC chemokines are important in the pathogenesis of pulmonary fibrosis and other fibroproliferative disorders...
  89. pmc Gender-based differences in bleomycin-induced pulmonary fibrosis
    Mehrnaz Gharaee-Kermani
    Department of Internal Medicine, University of Michigan Medical School, 210 Washtenaw, Box 2216, Ann Arbor, MI 48109 2216, USA
    Am J Pathol 166:1593-606. 2005
    ..To examine gender influence on pulmonary fibrosis, male and female rats were given endotracheal injections of either saline or bleomycin...
  90. ncbi High-resolution computed tomography in idiopathic pulmonary fibrosis: diagnosis and prognosis
    David A Lynch
    Department of Radiology, and Division of Pulmonary Sciences and Critical Care Medicine, University of Colorado Health Sciences Center, 4200 East Ninth Avenue, Box A030, Room 2233 Denver, CO 80262, USA
    Am J Respir Crit Care Med 172:488-93. 2005
    High-resolution computed tomography (HRCT) is an integral aspect of the evaluation of patients with suspected idiopathic pulmonary fibrosis (IPF). However, few studies have evaluated its use in a large cohort.
  91. pmc Herpesvirus DNA is consistently detected in lungs of patients with idiopathic pulmonary fibrosis
    Yi Wei Tang
    Department of Medicine, Vanderbilt University School of Medicine, Nashville, Tennessee 37232, USA
    J Clin Microbiol 41:2633-40. 2003
    On the basis of earlier reports associating Epstein-Barr Virus (EBV) with half of the cases of idiopathic pulmonary fibrosis (IPF), we hypothesized that chronic infection with EBV or a closely related herpesvirus would be detected in all ..
  92. ncbi IL-17A-producing gammadelta T and Th17 lymphocytes mediate lung inflammation but not fibrosis in experimental silicosis
    Sandra Lo Re
    Louvain Centre for Toxicology and applied Pharmacology, de Duve Institute, Universite Catholique de Louvain, Brussels, Belgium
    J Immunol 184:6367-77. 2010
    ..Although the acute alveolitis induced by silica is IL-17A dependent, this cytokine appears dispensable for the development of the late inflammatory and fibrotic lung responses to silica...
  93. pmc Telomerase and idiopathic pulmonary fibrosis
    Mary Armanios
    Department of Oncology, Johns Hopkins University School of Medicine, Baltimore, MD, USA
    Mutat Res 730:52-8. 2012
    Idiopathic pulmonary fibrosis (IPF) is the most common manifestation of telomere-mediated disorders...
  94. pmc Comprehensive microRNA analysis in bleomycin-induced pulmonary fibrosis identifies multiple sites of molecular regulation
    Ting Xie
    Division of Pulmonary, Allergy, and Critical Care Medicine, Department of Medicine, Duke University School of Medicine, Durham, North Carolina 27710, USA
    Physiol Genomics 43:479-87. 2011
    ..The miRNAs and their potential target genes identified may contribute to the understanding of the complex transcriptional program of lung fibrosis...
  95. ncbi Impact of pulmonary hypertension on gas exchange and exercise capacity in patients with pulmonary fibrosis
    Sven Glaser
    Division of Pulmonary Medicine and Infectious Diseases, University of Greifswald, Sauerbruchstrasse, 17475 Greifswald, Germany
    Respir Med 103:317-24. 2009
    Pulmonary hypertension is a relevant interceding morbidity in patients with pulmonary fibrosis that has significant impact on exercise tolerance and outcome...
  96. ncbi The presence of emphysema further impairs physiologic function in patients with idiopathic pulmonary fibrosis
    Marco Mura
    Unita Operativa di Fisiopatologia Respiratoria, Policlinico Sant Orsola Malpighi, Bologna, Italy
    Respir Care 51:257-65. 2006
    Emphysema, especially in the upper lobes, is frequently observed in association with idiopathic pulmonary fibrosis (IPF). However, the combination of emphysema plus IPF has received little attention.
  97. ncbi Clinical characteristics of combined pulmonary fibrosis and emphysema
    Yoshiaki Kitaguchi
    First Department of Internal Medicine, Shinshu University School of Medicine, Matsumoto, Japan
    Respirology 15:265-71. 2010
    ..This study describes the clinical characteristics of patients with CPFE and compares these with patients with COPD. It should therefore be noteworthy that the prevalence of lung cancer might be high in CPFE patients...
  98. ncbi Prognostic value of circulating KL-6 in idiopathic pulmonary fibrosis
    Akihito Yokoyama
    Department of Molecular and Internal Medicine, Hiroshima University, Hiroshima, Japan
    Respirology 11:164-8. 2006
    ..are elevated in a majority of patients with a number of interstitial lung diseases, including idiopathic pulmonary fibrosis (IPF). However, KL-6 levels vary from patient to patient...
  99. pmc The role of circulating mesenchymal progenitor cells (fibrocytes) in the pathogenesis of pulmonary fibrosis
    Robert M Strieter
    Department of Medicine, Division of Pulmonary Critical Care, Box 800466, Charlottesville, VA 22908 0466, USA
    J Leukoc Biol 86:1111-8. 2009
    b>Pulmonary fibrosis is associated with a number of disorders that affect the lung...
  100. ncbi STAT4 is a genetic risk factor for systemic sclerosis having additive effects with IRF5 on disease susceptibility and related pulmonary fibrosis
    P Dieude
    Université Paris 7 and Service de Rhumatologie, Hopital Bichat Claude Bernard, Assistance Publique Hopitaux de Paris, Paris, France
    Arthritis Rheum 60:2472-9. 2009
    ..The aim of this study was to determine whether the STAT4 rs7574865 single-nucleotide polymorphism is associated with SSc, and whether it interacts with IRF5...
  101. ncbi The prognostic significance of the histologic pattern of interstitial pneumonia in patients presenting with the clinical entity of cryptogenic fibrosing alveolitis
    A G Nicholson
    Department of Histopathology, Interstitial Lung Disease Unit, Royal Brompton Hospital, London, United Kingdom
    Am J Respir Crit Care Med 162:2213-7. 2000
    ..However, in patients with clinically typical CFA, a histologic diagnosis of fibrotic NSIP needs to be interpreted with caution and does not necessarily denote a good outcome...

Research Grants74

  1. Development of a Nonpsychotropic Cannabinoid Agonist for the Treatment of Sclerod
    Robert B Zurier; Fiscal Year: 2012
    ..The progressive fibrosis often leads to organ dysfunction including debilitating esophageal dysfunction and pulmonary fibrosis, the leading cause of death for SSc patients...
  2. Molecular Markers for Progression of Pulmonary Fibrosis in Systemic Sclerosis
    Shervin Assassi; Fiscal Year: 2013
    DESCRIPTION (provided by applicant): Title: Molecular Markers for Progression of Pulmonary Fibrosis in Systemic Sclerosis Candidate: Dr...
  3. Molecular mechanisms of lung disease in ataxia telangiectasia
    JoAnn M Sekiguchi; Fiscal Year: 2013
    ..Develop and characterize a mouse model of A-T ILD using well-established and characterized methods to induce pulmonary fibrosis in animals...
  4. A Bifunctional Katp Channel Activator and Redox Mimetic for BPD
    Kanneganti Murthy; Fiscal Year: 2013
    ..2-tailed) relative to the vehicle control: >30% increase in alveolar density, and >30% reductions in pulmonary fibrosis, pulmonary arteriolar smooth muscle hypertrophy, right ventricular mass, PMN infiltration, lipid ..
  5. Novel Means to Establish Free Radical Balance in the Neonatal Premature Lung
    Prakash Jagtap; Fiscal Year: 2011
    ..model, delayed therapy with R-100 provided near complete arrest and reversal of the progression in pulmonary fibrosis and alveolar inflammation, and blocked vascular hypertrophy and attenuated PAH...
  6. A novel telomerase expressing lung fibroblast phenotype
    Sem H Phan; Fiscal Year: 2012
    ..lung injury and fibrosis, and recent evidence points to a potential role for telomerase in certain types of pulmonary fibrosis in humans related to reduced telomere length...
  7. SARS-CoV Pathogenic Mechanisms in Senescent Mice
    Ralph S Baric; Fiscal Year: 2012
    ..nonlethal SARS-CoV infection in young and senescent mice, determining if chronic disease manifestations of pulmonary fibrosis occur in survivors...
  8. IGFBP-5 and Hsp70 in Extracellular Matrix Homeostasis and Fibrosis
    Kristen L Veraldi; Fiscal Year: 2013
    ..protein 5 (IGFBP-5) in scleroderma fibroblasts and fibrotic lung tissue from patients with idiopathic pulmonary fibrosis. Furthermore, over-expression of IGFBP-5 in vitro and in vivo results in aberrant extracellular matrix (ECM) ..
  9. Mechanism of Irradiation Pulmonary Fibrosis
    Joel S Greenberger; Fiscal Year: 2012
    ..myofibroblast progenitor cells (bone marrow stromal cells/mesenchymal stem cells), which contribute to pulmonary fibrosis. Both migrations are significantly reduced by intrapulmonary manganese superoxide dismutase-plasmid liposome ..
  10. Fate of Lung Stem Cells During Pulmonary Disease
    Susan M Majka; Fiscal Year: 2012
    ..PUBLIC HEALTH RELEVANCE: Pulmonary hypertension, chronic obstructive pulmonary disease, interstitial pulmonary fibrosis and other adult lung conditions are a major cause of morbidity and mortality...
  11. TNF Family Members and Control of Fibrotic Disease
    Michael Croft; Fiscal Year: 2013
    ..We also have found that patients with pulmonary fibrosis due to systemic sclerosis have T cells in the lungs that express LIGHT, which correlates with clinical ..
  12. Research Training in Cardiovascular and Pulmonary Diseases
    S Jamal Mustafa; Fiscal Year: 2012
    ..coronary ischemia, asthma, pulmonary edema, hypertension, cardiac failure, intestinal vascular inflammation, pulmonary fibrosis, peripheral vascular disease, etc...
  13. Epigenetic Alterations in IPF Fibroblastic Foci
    YAN SANDERS; Fiscal Year: 2010
    DESCRIPTION (provided by applicant): Idiopathic Pulmonary Fibrosis (IPF) is a deadly disease without a clearly-defined etiology. Because the incidence increases with age, it is plausible that environmental effects may be important...
  14. Caveolins in the Pathogenesis of Idiopathic Pulmonary Fibrosis
    Dan N Predescu; Fiscal Year: 2010
    ..in type I epithelial cells after radiation-induced lung injury, as well as in a model of bleomicin induced pulmonary fibrosis. More than that, the cav-1 knockout mice develop lung fibrosis and may be a reliable model for studying IPF...
  15. Molecular regulation of GM-CSF expression in alveolar epithelial cells
    Robert Paine; Fiscal Year: 2013
    ..Insults, such as exposure to bleomycin, that result in pulmonary fibrosis are associated with decreased AEC expression of GM-CSF;fibrosis is reduced following treatment with GM-CSF...
  16. Periostin Regulation of Lung Fibrosis
    Bethany B Moore; Fiscal Year: 2013
    DESCRIPTION (provided by applicant): Periostin Regulation of Lung Fibrosis Idiopathic pulmonary fibrosis (IPF) is a progressive scarring disorder of the lung that is characterized by the accumulation of myofibroblasts and the deposition ..
  17. Regulation of Lung Fibrosis by Alternatively Activated Macrophages and Arginase-1
    ROBERT MATTHEW TIGHE; Fiscal Year: 2013
    ..The mechanisms that regulate unremitting pulmonary fibrosis are incompletely understood...
  18. CTRIP: Molecular phenotypes of rapidly progressive idiopathic pulmonary fibrosis
    Galen B Toews; Fiscal Year: 2010
    DESCRIPTION (provided by applicant): Molecular Markers of Idiopathic Pulmonary Fibrosis Progression. 2...
  19. Mechanisms of Pleural Fibrosis in Empyema
    Kamal A Mohammed; Fiscal Year: 2013
    ..Furthermore, HO-1 deficiency is known to promote EMT in renal fibrosis4 and HO-1 induction prevented pulmonary fibrosis in animal models5...
  20. Lung Toxicity of Carbon Nanotubes in Models of Pre-Existing Respiratory Disease
    JAMES CHRISTOPHER BONNER; Fiscal Year: 2010
    ..medicine), but have properties similar to asbestos, a fiber that is linked with the development of pulmonary fibrosis (tissue scarring) and mesothelioma (a rare cancer on the pleural surface of the lung)...
  21. IMMUNOLOGIC BASIS OF RESISTANCE AND HYPERSENSITIVITY
    KARL FRANK AUSTEN; Fiscal Year: 2012
    ..CysLTI and CysLT2 null) to characterize acute and chronic innate and adaptive immune contributions to pulmonary fibrosis and allergic and autoantibody-mediated inflammation, respectively...
  22. Effects of lymphangiogenesis stimulation on lung allograft rejection
    Souheil El-Chemaly; Fiscal Year: 2013
    ..of life in advanced lung diseases such as chronic obstructive pulmonary disease (COPD) and idiopathic pulmonary fibrosis (IPF). The median survival after transplantation is currently 5.3 years...
  23. Translational Approaches to Reducing Health Disparities in Lung Disease
    Joe G N Garcia; Fiscal Year: 2012
    ..Airway Diseases (Asthma and COPD);3. Acute Lung Injury;4. Interstitial lung diseases (idiopathic pulmonary fibrosis, sarcoidosis, systemic lupus erythematosus);and 5. Lung Cancer...
  24. Antifibrotic Therapy to Improve Immune reconstitution in HIV
    Timothy W Schacker; Fiscal Year: 2013
    ..of SIV infection that antifibrotic therapy with pirfenidone, a drug used for the treatment of idiopathic pulmonary fibrosis, limits collagen formation in the TZ and enhances preservation of CD4 T cell populations...
  25. Trophokine for regenerative medicine
    Jianmin Fang; Fiscal Year: 2010
    ..benefit to patients with a wide range of diseases, such as liver cirrhosis and failure, renal failure, pulmonary fibrosis, wound healing, and chemotherapy-induced liver or renal damage...
  26. MULTIDISCIPLINARY TRAINING IN PULMONARY RESEARCH
    MARK WALTER FRAMPTON; Fiscal Year: 2013
    ..Major research themes include lung immunology, inflammation and infection;pulmonary fibrosis and remodeling;tobacco smoke and chronic obstructive pulmonary disease;and health effects of air pollution...
  27. MYOFIBROBLASTS IN PULMONARY FIBROSIS
    Sem H Phan; Fiscal Year: 2012
    ..in lung injury and fibrosis, and to characterize the functional phenotype and roles of these cells in pulmonary fibrosis. The myofibroblast is a key source of mediators and extracellular matrix whose expression in fibrotic ..
  28. Mechanisms of Asbestos-Induced Alveolar Epithelial Cell Injury
    David W Kamp; Fiscal Year: 2013
    DESCRIPTION (provided by applicant): Asbestos causes asbestosis (pulmonary fibrosis secondary to asbestos) and malignancies (bronchogenic carcinoma and mesothelioma) by mechanisms that are not fully elucidated...
  29. RESEARCH TRAINING IN RESPIRATORY BIOLOGY
    Anne I Sperling; Fiscal Year: 2013
    ..These complement a new program of Studies in Pulmonary Fibrosis/Luna Transplantation that focuses on cellular and molecular mechanisms of fibrosis and on transplantation ..
  30. The Impact of Oxidative Stress on HIV-induced Lung Disease
    Jesse Roman; Fiscal Year: 2013
    ..are less frequent and noninfectious lung disorders, such as COPD, pulmonary hypertension, lung cancer, and pulmonary fibrosis are emerging as important causes of illness...
  31. Epithelial abnormalities in IPF: role of ER stress and GRP78/BiP
    Beiyun Zhou; Fiscal Year: 2013
    DESCRIPTION (provided by applicant): Idiopathic pulmonary fibrosis/usual interstitial pneumonia (IPF/UIP) is a devastating interstitial lung disease of unknown etiology, characterized by (myo)fibroblast activation/proliferation, ..
  32. Cu,Zn-SOD, MMP-9, and Asbestosis
    A Brent Carter; Fiscal Year: 2013
    DESCRIPTION (provided by applicant): Asbestosis is an important cause of pulmonary fibrosis. Aberrant extracellular matrix deposition is a hallmark of asbestos-induced pulmonary fibrosis and is characterized by an imbalance between matrix ..
  33. HTS for inhibitors of NADPH Oxidase 2 (NOX 2)
    Hugh Rosen; Fiscal Year: 2010
    ..to provide novel therapeutic approaches to inhibit hypertension, aortic aneurysm, myocardial infarction, pulmonary fibrosis, arthritis, Alzheimer's disease, stroke, cancer, and inflammation...
  34. Integrin-dependent mechanism of T cell-mediated pulmonary fibrosis
    Irina G Luzina; Fiscal Year: 2012
    b>Pulmonary fibrosis is a frequent consequence of chronic inflammatory processes and environmental expo- sures. Available antifibrotic therapies are inefficient...
  35. Regulation of Nox Enzymes by Calcium and Novel Subunits
    JOHN DAVID LAMBETH; Fiscal Year: 2013
    ..g., cancer, hypertension, atherosclerosis), fibrotic disease (pulmonary fibrosis, cirrhosis, diabetic nephropathy), inflammatory disorders (ARDS, arthritis, atherosclerosis), and ..
  36. Regulation of Nox Enzymes by Calcium and Novel Subunits
    J Lambeth; Fiscal Year: 2009
    ..g., cancer, hypertension, atherosclerosis), fibrotic disease (pulmonary fibrosis, cirrhosis, diabetic nephropathy), inflammatory disorders (ARDS, arthritis, atherosclerosis), and ..
  37. Host Factors in Fungal Allergy and Fibrosis
    Anuradha Ray; Fiscal Year: 2010
    ..Another clinical and basic science project will be focused on pulmonary fibrosis. The clinical project will use gene expression and protein expression profiling to better define subgroups ..
  38. RV/PA Recoupling by Bone Marrow Derived Mesenchymal Stem Cells
    Luis A Ortiz; Fiscal Year: 2013
    ..in health and disease and RV failure (RVF) is the immediate cause of death in patients with idiopathic pulmonary fibrosis (IPF)...
  39. Regulation and Function of hTERT in Human Cells
    William C Hahn; Fiscal Year: 2013
    ..by stem cell failure including dyskeratosis congenita, subsets of aplastic anemia and idiopathic pulmonary fibrosis. Taken together, these telomerase plays a key role in maintaining telomeres, accumulating evidence suggests ..
  40. Protective Role of the S1P-S1P1 Pathway in Pulmonary Fibrosis
    Andrew M Tager; Fiscal Year: 2013
    DESCRIPTION (provided by applicant): Idiopathic pulmonary fibrosis (IPF) is a progressive and usually fatal disease of unknown etiology...
  41. Mechanisms of Pulmonary Fibrosis in Hermansky-Pudlak Syndrome
    Lisa R Young; Fiscal Year: 2013
    DESCRIPTION (provided by applicant): Pulmonary fibrosis is a final common pathway in many forms of interstitial lung diseases (ILD)...
  42. MATRIX MEDIATORS OF WOUND HEALING
    Paul J Higgins; Fiscal Year: 2010
    ..g., arthritis, psoriasis, cancer, cardiovascular and pulmonary fibrosis, diabetic retinopathy, macular degeneration, excessive scaring disorders)...
  43. Oxidative activation of Src in smoke-induced epithelial mesenchymal transition
    Henry Jay Forman; Fiscal Year: 2013
    ..Cigarette smoking is implicated in idiopathic pulmonary fibrosis and lung cancer. CSE contains thousands of compounds, many of which could be responsible for inducing EMT...
  44. GRADS Cooperative Research Project: JHU Clinical Center
    David R Moller; Fiscal Year: 2013
    ..granulomatous disorder that involves the lungs in over 90% of affected individuals and may cause end-stage pulmonary fibrosis and death. With an incidence of ~10-40 per 100,000 people in the U.S...
  45. Alveolar Cell Dysfunction and Pulmonary Fibrosis
    Lisa R Young; Fiscal Year: 2011
    ..Abnormalities in alveolar type II cell function are commonly implicated in the pathogenesis of idiopathic pulmonary fibrosis (IPF), and are central features of ILD associated with mutations in surfactant protein C (SP-C) and the ATP-..
  46. Dysregulated Pathways in Pulmonary Fibrosis
    Keith C Olsen; Fiscal Year: 2012
    DESCRIPTION (provided by applicant): Idiopathic Pulmonary fibrosis (IPF) is a deadly disease with no effective treatments...
  47. Telomere Shortening and Stem Cell Maintenance
    Mary Y Armanios; Fiscal Year: 2010
    ..These families display dominant inheritance of aplastic anemia, idiopathic pulmonary fibrosis as well as an increased risk of cancer...
  48. Translational Control in IPF
    Peter B Bitterman; Fiscal Year: 2011
    ..In this revised proposal, we propose to study lung fibroblasts from patients with Idiopathic Pulmonary Fibrosis (IPF), a prototype fibroproliferative disease, and elucidate the mechanism of autonomous function using our ..
  49. MECHANISMS OF PULMONARY FIBROSIS
    Sem H Phan; Fiscal Year: 2010
    b>Pulmonary fibrosis is a common end result of diverse forms'of lung injury and interstitial lung diseases...
  50. PKD Family Kinase Function and Signaling in Lung Fibroblasts
    Hua Tang; Fiscal Year: 2012
    Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive and usually fatal lung disease of unknown etiology, characterized by lung fibroblast activation and proliferation, excessive deposition of extracellular matrix (ECM), and ..
  51. Role of IL-17 in the Induction of Epithelial to Mesenchymal Transition
    Ragini Vittal; Fiscal Year: 2013
    ..has been increasingly recognized in wound healing and fibrogenesis in fibrotic diseases, such as idiopathic pulmonary fibrosis (IPF) and lung transplant associated obliterative bronchiolitis (OB)...
  52. Diagnostic Tests and Immunotherapy of Sarcoidosis Using Mycobacterial Proteins
    David R Moller; Fiscal Year: 2012
    ..granulomatous disorder that involves the lungs in over 90% of affected individuals and may cause end-stage pulmonary fibrosis and death...
  53. Angiopoietins in vascular and lymphatic remodeling of airways and lung
    Donald M McDonald; Fiscal Year: 2013
    ..use three mouse models of lung inflammation, due to mycoplasmal infection, fungal allergen challenge, or pulmonary fibrosis, with complementary gain or loss of function reagents and transgenic mice, as tools to interrogate the ..
  54. Identification of Pathways that Regulate Fibrosis through Collagen Resorption
    Kamran Atabai; Fiscal Year: 2012
    DESCRIPTION (provided by applicant): Pulmonary fibrosis is a common disorder without effective pharmacological treatment...
  55. Extracellular Matrix Components, Oxidants and Antioxidants in Pulmonary Fibrosis
    CORRINE R KLIMENT; Fiscal Year: 2010
    ..However, the mechanisms through which EC-SOD inhibits pulmonary fibrosis and inflammation remain unclear...
  56. Mesenchymal stem cell secretome in lung fibrosis: mitochondria and RNA shuttle
    Luis A Ortiz; Fiscal Year: 2013
    DESCRIPTION (provided by applicant): Pulmonary fibrosis is a lethal disease for which no successful therapy is available. Experimental silicosis is an excellent model to study this disease...
  57. Right Heart Function in Health and Chronic Lung Disease
    Anthony J Baker; Fiscal Year: 2013
    ..We will study the RV-specific model of bleomycin-induced pulmonary fibrosis, which leads to pulmonary hypertension and RV failure. Aim 1...
  58. Selective MRTF/SRF-Transcription Inhibitors: Novel Anti-fibrotics for IPF
    Vijaykumar M Baragi; Fiscal Year: 2013
    ..aims to advance and commercialize proprietary MRTF/SRF-transcription inhibitors for treatment of Idiopathic Pulmonary Fibrosis (IPF). IPF affects a person's ability to breathe and get enough oxygen to vital organs...
  59. Studying a bone marrow failure disease using patient-specific iPS cells
    LUIS ZIRNBERGER BATISTA; Fiscal Year: 2013
    ..that presents with poor life expectancy and multi-systemic tissue defects that include aplastic anemia and pulmonary fibrosis. A combination of technologies will be used to achieve this goal, including genetic correction, high-..
  60. Genetic Susceptibility to Nanoparticle-Induced Respiratory Disease
    JAMES CHRISTOPHER BONNER; Fiscal Year: 2013
    ..is that CNTs have some properties similar to asbestos, a fiber that is linked with the development of pulmonary fibrosis (tissue scarring) and mesothelioma (a rare cancer on the pleural surface of the lung)...
  61. Lung Genomics Research Consortium
    AVRUM E SPIRA; Fiscal Year: 2010
    ..is the fourth leading cause of death in the United States and the incidence and rate of death from pulmonary fibrosis is increasing each year...
  62. Uterine-specific genetic modification and lymphangioleiomyomatosis
    JOSE M TEIXEIRA; Fiscal Year: 2012
    ..that the hormonally responsive mesenchymal stromal cells of the uterus might be the source of the cells for pulmonary fibrosis and account for the female-specificity of LAM...
  63. TGFB AND GENE THERAPY IN SILICA INDUCED LUNG FIBROSIS
    Patricia Sime; Fiscal Year: 2005
    ..life threatening, pulmonary inflammatory and fibrotic pathologies including acute and chronic (nodular pulmonary fibrosis) silicosis...
  64. MECHANISMS OF PULMONARY FIBROSIS CAUSED BY SILICA
    Gerald Davis; Fiscal Year: 2003
    ..particles inhaled through workplace exposure are an important world-wide cause of chronic lung disease and pulmonary fibrosis. The goal of this research is to delineate the cytokine-driven mechanisms of lung scarring caused by the ..
  65. Inhibition of the SDF-1/CXCR4 axis as preventive therapy for radiation-induced pu
    HUI KUO contact SHU; Fiscal Year: 2010
    ..One particularly devastating late injury caused by radiation is pulmonary fibrosis. This problem develops as progressive shortness of breath with increasing interstitial lung fibrosis and ..
  66. LUNG TOXIC INTERACTIONS INVOLVING THERAPEUTIC DRUGS
    JAMES KEHRER; Fiscal Year: 1991
    Numerous cytotoxic drugs have been found to induce lung damage and pulmonary fibrosis. Although the underlying mechanisms are poorly understood, and may differ for each agent, the interaction of two antineoplastic drugs, or one such drug ..
  67. INTEGRIN-DEPENDENT T CELL-MEDIATED MECHANISMS OF PULMONARY FIBROSIS
    Irina G Luzina; Fiscal Year: 2010
    DESCRIPTION (provided by applicant): Pulmonary fibrosis is a major cause of disability and death in patients with autoimmune rheumatic diseases such as scleroderma, rheumatoid arthritis, dermatomyositis and polymyositis...
  68. IMMUNOLOGIC REGULATION OF PULMONARY FIBROSIS
    Robert Mason; Fiscal Year: 2001
    The overall purpose of this SCOR proposal is to investigate the immunologic regulation of pulmonary fibrosis. We have developed a research program to focus on those factors which will minimize or attenuate the fibrotic response to lung ..
  69. A LINKAGE STUDY IN FAMILIAL PULMONARY FIBROSIS
    Mark Steele; Fiscal Year: 2004
    ..goal of this project is to investigate inherited genetic factors that play a role in the development of pulmonary fibrosis. The overall hypothesis of this investigation is that inherited genetic factors predispose individuals to ..
  70. Interferon-gamma regulation of bleomycin pulmonary toxicity
    Edward Chen; Fiscal Year: 2007
    The genetic and immunologic basis of pulmonary fibrosis is poorly understood...
  71. A Role for Advanced Glycation End-Products in Diabetic Lung Injury
    Judson M Englert; Fiscal Year: 2010
    ..Experimental models of asbestosis recapitulate the salient features of idiopathic pulmonary fibrosis (IPF), a debilitating disease with both high morbidity and mortality due to a lack of effective therapies...
  72. ORAL CYCLOPHOSPHAMIDE VS ORAL PLACEBO IN SSC ALVEOLITIS
    Dean Schraufnagel; Fiscal Year: 2003
    In Systemic Sclerosis (SSc), interstitial pulmonary fibrosis is frequent (80%) and is now the leading cause of death...
  73. ORAL CYCLOPHOSPHAMIDE VS ORAL PLACEBO IN SSC ALVEOLITIS
    Daniel Furst; Fiscal Year: 2001
    In Systemic Sclerosis (SSc), interstitial pulmonary fibrosis is frequent (80 percent) and is now the leading cause of death...