516
Views
34
CrossRef citations to date
0
Altmetric
Reviews

Pathogenesis of idiopathic pulmonary fibrosis and its clinical implications

, &

References

  • Travis WD, Costabel U, Hansell DM, et al. An official American Thoracic Society/European Respiratory Society statement: update of the international multidisciplinary classification of the idiopathic interstitial pneumonias. Am J Respir Crit Care Med 2013;188(6):733-48
  • Raghu G, Collard HR, Egan JJ, et al. Idiopathic pulmonary fibrosis: evidence based guidelines for diagnosis and management. Am J Respir Crit Care Med 2011;183(6):788-824
  • Ley B, Collard HR, King TE Jr. Clinical course and prediction of survival in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 2011;183(4):431-40
  • Bjoraker JA, Ryu JH, Edwin MK, et al. Prognostic significance of histopathologic subsets in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 1998;157(1):199-203
  • Flaherty KR, Toews GB, Travis WD, et al. Clinical significance of histological classification of idiopathic interstitial pneumonia. Eur Respir J 2002;19(2):275-83
  • Nicholson AG, Colby TV, du Bois RM, et al. The prognostic significance of the histologic pattern of interstitial pneumonia in patients presenting with the clinical entity of cryptogenic fibrosing alveolitis. Am J Respir Crit Care Med 2000;162(6):2213-17
  • King TE Jr, Schwarz MI, Brown K, et al. Idiopathic pulmonary fibrosis: relationship between histopathologic features and mortality. Am J Respir Crit Care Med 2001;164(6):1025-32
  • Noble PW, Albera C, Bradford WZ, et al. Pirfenidone in patients with idiopathic pulmonary fibrosis (CAPACITY): two randomised trials. Lancet 2011;377(9779):1760-9
  • Richeldi L, Costabel U, Selman M, et al. Efficacy of a tyrosine kinase inhibitor in idiopathic pulmonary fibrosis. N Engl J Med 2011;365(12):1079-87
  • 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(2):136-51
  • King TE Jr, Pardo A, Selman M. Idiopathic pulmonary fibrosis. Lancet 2011;378(9807):1949-61
  • Rafii R, Juarez MM, Albertson TE, Chan AL. A review of current and novel therapies for idiopathic pulmonary fibrosis. J Thorac Dis 2013;5(1):48-73
  • Maher TM. Idiopathic pulmonary fibrosis: pathobiology and novel approaches to treatment. Clin Chest Med 2012;33(1):69-83
  • du Bois RM. Strategies for treating idiopathic pulmonary fibrosis. Nat Rev Drug Discov 2010;9(2):129-40
  • Baumgartner KB, Samet JM, Coultas DB, et al. Occupational and environmental risk factors for idiopathic pulmonary fibrosis: a multicenter case-control study. Am J Epidemiol 2000;152(4):307-15
  • Steele MP, Speer MC, Loyd JE, et al. Clinical and pathological features of familial interstitial pneumonia. Am J Respir Crit Care Med 2005;172(9):1146-52
  • Thomas AQ, Lane K, Phillips J III, 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(9):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(1):52-9
  • Noble PW, Barkauskas CE, Jiang D. Pulmonary fibrosis: patterns and perpetrators. J Clin Invest 2012;122(8):2756-62
  • Armanios M. Telomerase and idiopathic pulmonary fibrosis. Mutat Res 2012;730(1-2):52-8
  • 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(35):13051-6
  • Seibold MA, Wise AL, Speer MC, et al. A common MUC5B promoter polymorphism and pulmonary fibrosis. N Engl J Med 2011;364(16):1503-12
  • Stock CJ, Sato H, Fonseca C, et al. Mucin 5B promoter polymorphism is associated with idiopathic pulmonary fibrosis but not with development of lung fibrosis in systemic sclerosis or sarcoidosis. Thorax 2013;68(5):436-41
  • Borie R, Crestani B, Dieude P, et al. The MUC5B variant is associated with idiopathic pulmonary fibrosis but not with systemic sclerosis interstitial lung disease in the European Caucasian population. PLoS One 2013;8(8):e70621
  • Noth I, Zhang Y, Ma S-F, et al. Genetic variants associated with idiopathic pulmonary fibrosis susceptibility and mortality: a genome-wide association study. Lancet Respir Med 2013;1(4):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(6):613-20
  • Mulugeta S, Nguyen V, Russo SJ, et al. A surfactant protein C precursor protein BRICHOS domain mutation causes endoplasmic reticulum stress, proteasome dysfunction, and caspase 3 activation. Am J Respir Cell Mol Biol 2005;32(6):521-30
  • Lawson WE, Crossno PF, Polosukhin VV, et al. Endoplasmic reticulum stress in alveolar epithelial cells is prominent in IPF: association with altered surfactant protein processing and herpesvirus infection. Am J Physiol Lung Cell Mol Physiol 2008;294(6):L1119-26
  • Korfei M, Ruppert C, Mahavadi P, et al. Epithelial endoplasmic reticulum stress and apoptosis in sporadic idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 2008;178(8):838-46
  • Plantier L, Crestani B, Wert SE, et al. Ectopic respiratory epithelial cell differentiation in bronchiolised distal airspaces in idiopathic pulmonary fibrosis. Thorax 2011;66(8):651-7
  • Seibold MA, Smith RW, Urbanek C, et al. The idiopathic pulmonary fibrosis honeycomb cyst contains a mucociliary pseudostratified epithelium. PLoS One 2013;8(3):e58658
  • Günther A, Korfei M, Mahavadi P, et al. Unravelling the progressive pathophysiology of idiopathic pulmonary fibrosis. Eur Respir Rev 2012;21(124):152-60
  • Wynn TA. Integrating mechanisms of pulmonary fibrosis. J Exp Med 2011;208(7):1339-50
  • Cipriani NA, Strek M, Noth I, et al. Pathologic quantification of connective tissue disease–associated versus idiopathic usual interstitial pneumonia. Arch Pathol Lab Med 2012;136(10):1253-8
  • Song JW, Do KH, Kim MY, et al. Pathologic and radiologic differences between idiopathic and collagen vascular disease-related usual interstitial pneumonia. Chest 2009;136(1):23-30
  • Takemura T, Akashi T, Kamiya H, et al. Pathological differentiation of chronic hypersensitivity pneumonitis from idiopathic pulmonary fibrosis/usual interstitial pneumonia. Histopathology 2012;61(6):1026-35
  • Trahan S, Hanak V, Ryu JH, Myers JL. Role of surgical lung biopsy in separating chronic hypersensitivity pneumonia from usual interstitial pneumonia/idiopathic pulmonary fibrosis: analysis of 31 biopsies from 15 patients. Chest 2008;134(1):126-32
  • Larsen BT, Colby TV. Update for pathologists on idiopathic interstitial pneumonias. Arch Pathol Lab Med 2012;136(10):1234-41
  • Myers JL, Katzenstein ALA. Beyond a consensus classification for idiopathic interstitial pneumonias: progress and controversies. Histopathology 2009;54(1):90-103
  • Churg A, Muller NL, Flint J, Wright JL. Chronic hypersensitivity pneumonitis. Am J Surg Pathol 2006;30(2):201-8
  • Chilosi M, Carloni A, Rossi A, Poletti V. Premature lung aging and cellular senescence in the pathogenesis of idiopathic pulmonary fibrosis and COPD/emphysema. Transl Res 2013;162(3):156-73
  • 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(6):591-600
  • Wolters PJ, Collard HR, Jones KD. Pathogenesis of idiopathic pulmonary fibrosis. Annu Rev Pathol Mech Dis 2014;9:157-79
  • 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(1):242-8
  • Tobin RW, Pope CE II, Pellegrini CA, et al. Increased prevalence of gastroesophageal reflux in patients with idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 1998;158(6):1804-8
  • Lawson WE, Grant SW, Ambrosini V, et al. Genetic mutations in surfactant protein C are a rare cause of sporadic cases of IPF. Thorax 2004;59(11):977-80
  • Borensztaj K, Crestani B, Kolb M. Idiopathic pulmonary fibrosis: from epithelial injury to biomarkers - insights from the benchside. Respiration 2013;86(6):441-52
  • Hashimoto N, Jin H, Liu T, et al. Bone marrow-derived progenitor cells in pulmonary fibrosis. J Clin Invest 2004;113(2):243-52
  • Willis BC, duBois RM, Borok Z. Epithelial origin of myofibroblasts during fibrosis in the lung. Proc Am Thorac Soc 2006;3(4):377-82
  • Armanios A. Syndromes of telomere shortening. Annu Rev Genomics Hum Genet 2009;10:45-61
  • Chilosi M, Poletti V, Zamò A, et al. Aberrant Wnt/β-catenin pathway activation in idiopathic pulmonary fibrosis. Am J Pathol 2003;162(5):1495-502
  • Meyer KC, Raghu G, Baughman RP, et al. An official American Thoracic Society clinical practice guideline: the clinical utility of bronchoalveolar lavage cellular analysis in interstitial lung disease. Am J Respir Crit Care Med 2012;185(9):1004-14
  • Kosanovic D, Dahal BK, Wygrecka M, et al. Mast cell chymase: an indispensable instrument in the pathological symphony of idiopathic pulmonary fibrosis? Histol Histopathol 2013;28(6):691-9
  • Song S, Ouyang N, Hobelt M, et al. Influence of alternatively and classically activated macrophages on fibrogenic activities of human fibroblasts. Cell Immunol 2000;204(1):19-28
  • Fujishima S, Shiomi T, Yamashita S, et al. Production and activation of metalloproteinase 7 (matrisylin 1) in the lungs of patients with idiopathic pulmonary fibrosis. Arch Pathol Lab Med 2010;134(8):1136-42
  • Martinet Y, Rom WN, Grotendorst GR. Exaggerated spontaneous release of platelet-derived growth factor by alveolar macrophages from patients with idiopathic pulmonary fibrosis. New Engl J Med 1987;317(4):202-9
  • Murray LA, Chen Q, Kramer MS, et al. TGF-beta driven lung fibrosis is macrophage dependent and blocked by serum amyloid P. Int J Biochem Cell Biol 2011;43(1):154-62
  • Wynn TA. Cellular and molecular mechanisms of fibrosis. J Pathol 2008;214(2):199-210
  • Clarke D, Carruthers AM, Mustelin T, Murray LA. Matrix regulation of idiopathic pulmonary fibrosis: the role of enzymes. Fibrogenesis Tissue Repair 2013;6(1):20
  • Nakazato H, Oku H, Yamane S, et al. A novel anti-fibrotic agent pirfenidone suppresses tumor necrosis factor-alpha at the translational level. Eur J Pharmacol 2002;446(1-3):177-85
  • Azuma A, Nukiwa T, Tsuboi E, et al. Double-blind, placebo-controlled trial of pirfenidone in patients with idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 2005;171(9):1040-7
  • Taniguchi H, Ebina M, Kondoh Y, et al. Pirfenidone in idiopathic pulmonary fibrosis. Eur Respir J 2010;35(4):821-9
  • Spagnolo P, Del Giovane C, Luppi F, et al. Non-steroid agents for idiopathic pulmonary fibrosis. Cochrane Database Syst Rev 2010;9:CD003134
  • Available from: http://phx.corporate-ir.net/phoenix.zhtml?c=100067&p=irol-newsArticle&ID=1903095&highlight
  • Conte E, Gili E, Fagone E, et al. Effect of pirfenidone on proliferation, TGF-β-induced myofibroblast differentiation and fibrogenic activity of primary human lung fibroblasts. Eur J Pharm Sci 2014; doi:10.1016/j.ejps.2014.02.014
  • Schaefer CJ, Ruhrmund DW, Pan L, et al. Antifibrotic activities of pirfenidone in animal models. Eur Respir Rev 2011;20(120):85-97
  • Hilberg F, Roth GJ, Krssak M, et al. BIBF 1120: triple angiokinase inhibitor with sustained receptor blockade and good antitumor efficacy. Cancer Res 2008;68(12):4774-82
  • Moore BB, Lawson WE, Oury TD, et al. Animal models of fibrotic lung disease. Am J Respir Cell Mol Biol 2013;49(2):167-79
  • Morell F, Villar A, Montero M-A, et al. Chronic hypersensitivity pneumonitis in patients diagnosed with idiopathic pulmonary fibrosis: a prospective case-cohort study. Lancet Respir Med 2013;1(9):685-94
  • Kropski JA, Lawson WE, Young LR, Blackwell TS. Genetic studies provide clues on the pathogenesis of idiopathic pulmonary fibrosis. Dis Model Mech 2013;6(1):9-17
  • Ding Q, Luckhardt T, Hecker L, et al. New insights into the pathogenesis and treatment of idiopathic pulmonary fibrosis. Drugs 2011;71(8):981-1001
  • Yokoyama A, Kondo K, Nakajima M, et al. Prognostic value of circulating KL-6 in idiopathic pulmonary fibrosis. Respirology 2006;11(2):164-8
  • Kinder BW, Brown KK, McCormack FX, et al. Serum surfactant protein -A is a string predictor of early mortality in idiopathic pulmonary fibrosis. Chest 2009;135(6):1557-63
  • Prasse A, Probst C, Bargagli E, et al. Serum CC-chemokine ligand 18 concentration predicts outcome in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 2009;179(8):717-23
  • Rosas IO, Richards TJ, Konishi K, et al. MMP1 and MMP7 as potential peripheral blood biomarkers in idiopathic pulmonary fibrosis. PLoS Med 2008;5(4):e93
  • Egger G, Liang G, Aparicio A, Jones PA. Epigenetics in human disease and prospects for epigenetic therapy. Nature 2004;429(6990):457-63
  • Yang IV. Epigenomics of idiopathic pulmonary fibrosis. Epigenomics 2012;4(2):195-203
  • Bazzini AA, Lee MT, Giraldez AJ. Ribosome profiling shows that miR-430 reduces translation before causing mRNA decay in zebrafish. Science 2012;336(6078):233-7
  • Liu G, Friggeri A, Yang Y, et al. miR-21 mediates fibrogenic activation of pulmonary fibroblasts and lung fibrosis. J Exp Med 2010;207(8):1589-97
  • Dakhlallah D, Batte K, Wang Y, et al. Epigenetic regulation of miR-17∼92 contributes to the pathogenesis of pulmonary fibrosis. Am J Respir Crit Care Med 2013;187(4):397-405
  • Sanders YY, Ambalavanan N, Halloran B, et al. Altered DNA methylation profile in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 2012;186(6):525-35
  • Ley B, Ryerson CJ, Vittinghoff E, et al. A multidimensional index and staging system for idiopathic pulmonary fibrosis. Ann Intern Med 2012;156(10):684-91
  • Raghu G, Collard HR, Anstrom KJ, et al. Idiopathic pulmonary fibrosis: clinically meaningful primary endpoints in phase 3 clinical trials. Am J Respir Crit Care Med 2012;185(10):1044-8
  • du Bois RM, Nathan SD, Richeldi L, et al. Idiopathic pulmonary fibrosis: lung function is a clinically meaningful endpoint for phase 3 trials. Am J Respir Crit Care Med 2012;186(8):712-15
  • Wells AU, Behr J, Costabel U, European IPF Consensus Group. Hot of the breath: mortality as a primary end-point in IPF treatment trials: the best is the enemy of the good. Thorax 2012;67(11):938-40
  • Spagnolo P, Luppi F, Maher TM, et al. Primary endpoints in phase 3 clinical trials in idiopathic pulmonary fibrosis: one step at a time. Am J Respir Crit Care Med 2013;187(11):1271-2
  • du Bois RM, Weycker D, Albera C, et al. Forced vital capacity in patients with idiopathic pulmonary fibrosis: test properties and minimal clinically important difference. Am J Respir Crit Care Med 2011;184(12):1382-9
  • Herazo-Maya JD, Kaminski N. Personalised medicine: applying “omics” to lung fibrosis. Biomark Med 2013;6(4):529-40
  • Korthagen NM, van Moorsel CH, Barlo NP, et al. Association between variations in cell cycle genes and idiopathic pulmonary fibrosis. PLoS One 2012;7(1):e30442
  • Hodgson U, Pulkkinen V, Dixon M, et al. ELMOD2 is a candidate gene for familial idiopathic pulmonary fibrosis. Am J Hum Genet 2006;79(1):149-54
  • Xue J, Gochuico BR, Alawad AS, et al. The HLA class II allele DRB1*1501 is over-represented in patients with idiopathic pulmonary fibrosis. PLoS One 2011;6(2):e14715
  • Whyte M, Hubbard R, Meliconi R, et al. Increased risk of fibrosing alveolitis associated with interleukin-1 receptor antagonist and tumor necrosis factor-alpha gene polymorphisms. Am J Respir Crit Care Med 2000;162(2 Pt 1):755-8
  • Korthagen NM, van Moorsel CH, Kazemier KM, et al. IL1RN genetic variations and risk of IPF: a meta-analysis and mRNA expression study. Immunogenetics 2012;64(5):371-7
  • Ahn MH, Park BL, Lee SH, et al. A promoter SNP rs4073T>A in the common allele of the interleukin 8 gene is associated with the development of idiopathic pulmonary fibrosis via the IL-8 protein enhancing mode. Respir Res 2011;12:73
  • Armanios MY, Chen JJ, Cogan JD, et al. Telomerase mutations in families with idiopathic pulmonary fibrosis. N Engl J Med 2007;356(13):1317-26
  • Tsakiri KD, Cronkhite JT, Kuan PJ, et al. Adult-onset pulmonary fibrosis caused by mutations in telomerase. Proc Natl Acad Sci USA 2007;104(18):7552-7
  • Mushiroda T, Wattanapokayakit S, Takahashi A, et al. A genomewide association study identifies an association of a common variant in TERT with susceptibility to idiopathic pulmonary fibrosis. J Med Genet 2008;45(10):654-6
  • Xaubet A, Marin-Arguedas A, Lario S, et al. Transforming growth factor-b1 gene polymorphisms are associated with disease progression in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 2003;168(4):431-5
  • Son JY, Kim SY, Cho SH, et al. TGF-β1 T869C polymorphism may affect susceptibility to idiopathic pulmonary fibrosis and disease severity. Lung 2013;191(2):199-205
  • Efficacy and safety of pirfenidone in patients with idiopathic pulmonary fibrosis (IPF) (ASCEND). Available from http://clinicaltrials.gov/show/NCT01366209
  • The Idiopathic Pulmonary Fibrosis Clinical Research Network. Prednisone, azathioprine, and N-acetylcysteine for pulmonary fibrosis. N Engl J Med 2012;366(21):1968-77
  • Safety and efficacy of BIBF 1120 at high dose in idiopathic pulmonary fibrosis patients. Available from http://clinicaltrials.gov/show/NCT01335464
  • Safety and efficacy of BIBF 1120 at high dose in idiopathic pulmonary fibrosis patients II. Available from: www.clinicaltrials.gov/ct2/show/NCT01335477
  • Raghu G, Million-Rousseau R, Morganti A, et al. Macitentan for the treatment of idiopathic pulmonary fibrosis: the randomized controlled MUSIC trial. Eur Respir J 2013;42(6):1622-32
  • King TE Jr, Albera C, Bradford WZ, et al. Effect of interferon gamma-1b on survival in patients with idiopathic pulmonary fibrosis (INSPIRE): a multicentre, randomised, placebo-controlled trial. Lancet 2009;374(9685):222-8
  • Idiopathic Pulmonary Fibrosis Clinical Research Network. Zisman DA, Schwarz MI, Anstrom KJ, et al. A controlled trial of sildenafil in advanced idiopathic pulmonary fibrosis. N Engl J Med 2010;363(7):620-8
  • Noth I, Anstrom KJ, Calvert SB, et al. A placebo-controlled randomized trial of warfarin in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 2012;186(1):88-95
  • King TE Jr, Brown KK, Raghu G, et al. BUILD-3: a randomized, controlled trial of bosentan in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 2011;184(1):92-9
  • Raghu G, Behr J, Brown KK, et al. Treatment of idiopathic pulmonary fibrosis with ambrisentan: a parallel, randomized trial. Ann Intern Med 2013;158(9):641-9
  • Daniels CE, Lasky JA, Limper AH, et al. Imatinib treatment for IPF: randomised placebo controlled trial results. Am J Respir Crit Care Med 2010;181(6):604-10

Reprints and Corporate Permissions

Please note: Selecting permissions does not provide access to the full text of the article, please see our help page How do I view content?

To request a reprint or corporate permissions for this article, please click on the relevant link below:

Academic Permissions

Please note: Selecting permissions does not provide access to the full text of the article, please see our help page How do I view content?

Obtain permissions instantly via Rightslink by clicking on the button below:

If you are unable to obtain permissions via Rightslink, please complete and submit this Permissions form. For more information, please visit our Permissions help page.