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Special Report

Permanent alveolar collapse is the predominant mechanism in idiopathic pulmonary fibrosis

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References

  • Raghu G, Collard HR, Egan JJ, et al. ATS/ERS/JRS/ALAT Committee on Idiopathic Pulmonary Fibrosis. An official ATS/ERS/JRS/ALAT statement: idiopathic pulmonary fibrosis: evidence-based guidelines for diagnosis and management. Am J Respir Crit Care Med 2011;183:788-824
  • Coalson JJ. The ultrastructure of human fibrosing alveolitis. Virchows Arch A Pathol Anat Histol 1982;395:181-99
  • Kawanami O, Ferrans VJ, Crystal RG. Structure of alveolar epithelial cells in patients with fibrotic lung disorders. Lab Invest 1982;46:39-53
  • Zoz DF, Lawson WE, Blackwell TS. Idiopathic pulmonary fibrosis: a disorder of epithelial cell dysfunction. Am J Med Sci 2011;341:435-8
  • Camelo A, Dunmore R, Sleeman MA, Clarke DL. The epithelium in idiopathic pulmonary fibrosis: breaking the barrier. Front Pharmacol 2014;4:173
  • Moore BB, Hogaboam CM. Murine models of pulmonary fibrosis. Am J Physiol Lung Cell Mol Physiol 2008;294:L152-60
  • Kropski JA, Lawson WE, Young LR, Blackwell TS. Genetic studies provide clues on the pathogenesis of idiopathic pulmonary fibrosis. Dis Model Mech 2013;6:9-17
  • Armanios MY, Chen JJ, Cogan JD, et al. Telomerase mutations in families with idiopathic pulmonary fibrosis. N Engl J Med 2007;356:1317-26
  • Alder JK, Chen JJ, Lancaster L, et al. Short telomeres are a risk factor for idiopathic pulmonary fibrosis. Proc Natl Acad Sci USA 2008;105:13051-6
  • Waisberg DR, Barbas-Filho JV, Parra ER, et al. Abnormal expression of telomerase/apoptosis limits type II alveolar epithelial cell replication in the early remodeling of usual interstitial pneumonia/idiopathic pulmonary fibrosis. Hum Pathol 2010;41:385-9
  • Thomas AQ, Lane K, Phillips J, et al. Heterozygosity for a surfactant protein C gene mutation associated with usual interstitial pneumonitis and cellular nonspecific interstitial pneumonitis in one kindred. Am J Respir Crit Care Med 2002;165:1322-8
  • Wang Y, Kuan PJ, Xing C, et al. Genetic defects in surfactant protein A2 are associated with pulmonary fibrosis and lung cancer. Am J Hum Genet 2009;84:52-9
  • Tanjore H, Blackwell TS, Lawson WE. Emerging evidence for endoplasmic reticulum stress in the pathogenesis of idiopathic pulmonary fibrosis. Am J Physiol Lung Cell Mol Physiol 2012;302:L721-9
  • Kelsen SG. Respiratory epithelial cell responses to cigarette smoke: the unfolded protein response. Pulm Pharmacol Ther 2012;25:447-52
  • Baumgartner KB, Samet JM, Stidley CA, et al. Cigarette smoking: a risk factor for idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 1997;155:242-8
  • Hengstermann A, Müller T. Endoplasmic reticulum stress induced by aqueous extracts of cigarette smoke in 3T3 cells activates the unfolded-protein-response-dependent PERK pathway of cell survival. Free Radic Biol Med 2008;44:1097-107
  • Sepper R, Prikk K, Metsis M, et al. Mucin5B expression by lung alveolar macrophages is increased in long-term smokers. J Leukoc Biol 2012;92:319-24
  • Todd NW, Luzina IG, Atamas SP. Molecular and cellular mechanisms of pulmonary fibrosis. Fibrogenesis Tissue Repair 2012;5:11
  • Chambers RC. Procoagulant signaling mechanisms in lung inflammation and fibrosis: novel opportunities for pharmacological intervention? Br J Pharmacol 2008;153:S367-78
  • Feghali-Bostwick CA, Tsai CG, Valentine VG, et al. Cellular and humoral autoreactivity in idiopathic pulmonary fibrosis. J Immunol 2007;179:2592-9
  • Ley B, Collard HR. Epidemiology of idiopathic pulmonary fibrosis. Clin Epidemiol 2013;5:483-92
  • Thannickal VJ, Zhou Y, Gaggar A, Duncan SR. Fibrosis: ultimate and proximate causes. J Clin Invest 2014;124:4673-7
  • Barbas-Filho JV, Ferreira MA, Sesso A, et al. Evidence of type II pneumocyte apoptosis in the pathogenesis of idiopathic pulmonary fibrosis (IFP)/usual interstitial pneumonia (UIP). J Clin Pathol 2001;54(2):132-8
  • Zuo W, Zhang T, Wu DZ, et al.p63(+)Krt5(+) distal airway stem cells are essential for lung regeneration. Nature 2015;517:616-20
  • Vaughan AE, Brumwell AN, Xi Y, et al. Lineage-negative progenitors mobilize to regenerate lung epithelium after major injury. Nature 2015;517:621-5
  • Raghu G, Weycker D, Edelsberg J, et al. Incidence and prevalence of idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 2006;174:810-16
  • Marchal-Somme J, Uzunhan Y, Marchand-Adam S, et al. Cutting edge: nonproliferating mature immune cells form a novel type of organized lymphoid structure in idiopathic pulmonary fibrosis. J Immunol 2006;176:5735-9
  • Todd NW, Scheraga RG, Galvin JR, et al. Lymphocyte aggregates persist and accumulate in the lungs of patients with idiopathic pulmonary fibrosis. J Inflamm Res 2013;6:63-70
  • Wynn TA. Integrating mechanisms of pulmonary fibrosis. J Exp Med 2011;208:1339-50
  • Crooks MG, Aslam I, Hart SP. Inflammation and pulmonary fibrosis. In: Khatami M, editor. Inflammatory diseases-immunopathology. Intech; Rijeka: 2012. p. 99-128
  • Fahy JV, Dickey BF. Airway mucus function and dysfunction. N Engl J Med 2010;363:2233-47
  • Seibold MA, Wise AL, Speer MC, et al. A common MUC5B promoter polymorphism and pulmonary fibrosis. N Engl J Med 2011;364:1503-12
  • Noth I, Zhang Y, Ma SF, et al. Genetic variants associated with idiopathic pulmonary fibrosis susceptibility and mortality: a genome-wide association study. Lancet Respir Med 2013;1:309-17
  • Fingerlin TE, Murphy E, Zhang W, et al. Genome-wide association study identifies multiple susceptibility loci for pulmonary fibrosis. Nat Genet 2013;45:613-20
  • Luzina IG, Todd NW, Iacono AT, Atamas SP. Roles of T lymphocytes in pulmonary fibrosis. J Leukoc Biol 2008;83:237-44
  • Pochetuhen K, Luzina IG, Lockatell V, et al. Complex regulation of pulmonary inflammation and fibrosis by CCL18. Am J Pathol 2007;171:428-37
  • Barnes PJ, Adcock IM. Glucocorticoid resistance in inflammatory diseases. Lancet 2009;373:1905-17
  • Horowitz JC, Thannickal VJ. Epithelial-mesenchymal interactions in pulmonary fibrosis. Semin Respir Crit Care Med 2006;27:600-12
  • Selman M, King TE, Pardo A. Idiopathic pulmonary fibrosis: prevailing and evolving hypotheses about its pathogenesis and implications for therapy. Ann Intern Med 2001;134:136-51
  • Chapman HA. Epithelial-mesenchymal interactions in pulmonary fibrosis. Annu Rev Physiol 2011;73:413-35
  • Ahluwalia N, Shea BS, Tager AM. New therapeutic targets in idiopathic pulmonary fibrosis: aiming to rein in runaway wound healing responses. Am J Respir Crit Care Med 2014;190:867-78
  • Richeldi L, du Bois RM, Raghu G, et al. INPULSIS Trial Investigators: Efficacy and safety of nintedanib in idiopathic pulmonary fibrosis. N Engl J Med 2014;370:2071-82
  • King TEJrBradford WZ, Castro-Bernardini S, et al. ASCEND Study Group: A phase 3 trial of pirfenidone in patients with idiopathic pulmonary fibrosis. N Engl J Med 2014;370:2083-92
  • Crystal RG, Fulmer JD, Roberts WC, et al. Idiopathic pulmonary fibrosis. Clinical, histologic, radiographic, physiologic, scintigraphic, cytologic, and biochemical aspects. Ann Intern Med 1976;85:769-88
  • Hance AJ, Horwitz AL, Cowan MJ, et al. Biochemical approaches to the investigation of fibrotic lung disease. Chest 1976;69:257-61
  • Fulmer JD, Bienkowski RS, Cowan MJ, et al. Collagen concentration and rates of synthesis in idiopathic pulmonary fibrosis. Am Rev Respir Dis 1980;122:289-301
  • Seyer JM, Hutcheson ET, Kang AH. Collagen polymorphism in idiopathic chronic pulmonary fibrosis. J Clin Invest 1976;57:1498-507
  • Selman M, Montaño M, Ramos C, Chapela R. Concentration, biosynthesis and degradation of collagen in idiopathic pulmonary fibrosis. Thorax 1986;41:355-9
  • Kirk JM, Da Costa PE, Turner-Warwick M, et al. Biochemical evidence for an increased and progressive deposition of collagen in lungs of patients with pulmonary fibrosis. Clin Sci (Lond) 1986;70:39-45
  • Coxson HO, Hogg JC, Mayo JR, et al. Quantification of idiopathic pulmonary fibrosis using computed tomography and histology. Am J Respir Crit Care Med 1997;155:1649-56
  • Katzenstein AL. Pathogenesis of "fibrosis" in interstitial pneumonia: an electron microscopic study. Hum Pathol 1985;16:1015-24
  • Moore BB, Hogaboam CM. Murine models of pulmonary fibrosis. Am J Physiol Lung Cell Mol Physiol 2008;294:L152-60
  • Vittal R, Mickler EA, Fisher AJ, et al. Type V collagen induced tolerance suppresses collagen deposition, TGF-β and associated transcripts in pulmonary fibrosis. PLoS One 2013;8:e76451
  • Parra ER, Teodoro WR, Velosa AP, et al. Interstitial and vascular type V collagen morphologic disorganization in usual interstitial pneumonia. J Histochem Cytochem 2006;54:1315-25
  • Madri JA, Furthmayr H. Collagen polymorphism in the lung. An immunochemical study of pulmonary fibrosis. Hum Pathol 1980;11:353-66
  • Wilkes DS, Chew T, Flaherty KR, et al. Oral immunotherapy with type V collagen in idiopathic pulmonary fibrosis. Eur Respir J 2015;45:1393-402
  • Floyd R. Organization of Pneumonic Exudates. Am J Med Sci 1922;163:527-48
  • Burkhardt A. Alveolitis and collapse in the pathogenesis of pulmonary fibrosis. Am Rev Respir Dis 1989;140:513-24
  • Crouch E. Pathobiology of pulmonary fibrosis. Am J Physiol 1990;259:L159-84
  • Galvin JR, Frazier AA, Franks TJ. Collaborative radiologic and histopathologic assessment of fibrotic lung disease. Radiology 2010;255:692-706
  • Myers JL, Katzenstein AL. Epithelial necrosis and alveolar collapse in the pathogenesis of usual interstitial pneumonia. Chest 1988;94:1309-11
  • Lutz D, Gazdhar A, Lopez-Rodriguez E, et al. Alveolar Derecruitment and Collapse Induration as Crucial Mechanisms in Lung Injury and Fibrosis. Am J Respir Cell Mol Biol 2015;52:232-43
  • Zhou Y, Huang X, Hecker L, et al. Inhibition of mechanosensitive signaling in myofibroblasts ameliorates experimental pulmonary fibrosis. J Clin Invest 2013;123:1096-108
  • Wipff PJ, Rifkin DB, Meister JJ, Hinz B. Myofibroblast contraction activates latent TGF-beta1 from the extracellular matrix. J Cell Biol 2007;179:1311-23
  • Golan-Gerstl R, Wallach-Dayan SB, Amir G, Breuer R. Epithelial cell apoptosis by fas ligand-positive myofibroblasts in lung fibrosis. Am J Respir Cell Mol Biol 2007;36:270-5
  • Hagimoto N, Kuwano K, Inoshima I, et al. TGF-beta 1 as an enhancer of Fas-mediated apoptosis of lung epithelial cells. J Immunol 2002;168:6470-8
  • Leslie KO. Idiopathic pulmonary fibrosis may be a disease of recurrent, tractional injury to the periphery of the aging lung: a unifying hypothesis regarding etiology and pathogenesis. Arch Pathol Lab Med 2012;136:591-600
  • Fowler WS. Intrapulmonary distribution of inspired gas. Physiol Rev 1952;32:1-20
  • Kacmarek RM, Stoller JK, Heuer AH. Egan’s fundamentals of respiratory care. 10th edition. Elsevier Health Sciences, Saint Louis, Missouri; 2013

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