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REVIEW
Pathogenesis of combined pulmonary fibrosis and emphysema: Common pathogenetic pathways
1
1st Department of Pneumonology, Hospital for Diseases of the Chest “Sotiria”, Medical School, National and Kapodistrian University of Athens, Athens, Greece
Corresponding author
Demosthenes Bouros
1st Department of Pneumonology, Hospital for Diseases of the Chest “Sotiria”, Medical School, National and Kapodistrian University of Athens, 152 Messogion Av., Athens 11527, Greece
1st Department of Pneumonology, Hospital for Diseases of the Chest “Sotiria”, Medical School, National and Kapodistrian University of Athens, 152 Messogion Av., Athens 11527, Greece
Pneumon 2015;28(2):139-146
KEYWORDS
REFERENCES (78)
1.
Snider GL. Chronic obstructive pulmonary disease: a definition and implications of structural determinants of airflow obstruction for epidemiology. Am Rev Respir Dis 1989;140:S3-8.
2.
Raghu G, Collard HR, Egan JJ, et al. 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.
3.
Wiggins J, Strickland B, Turner-Warwick M. Combined cryptogenic fibrosing alveolitis and emphysema: the value of high resolution computed tomography in assessment. Respir Med 1990;84:365-9.
4.
Cottin V, Nunes H, Brillet P, et al. Combined pulmonary fibrosis and emphysema: a distinct underrecognised entity. Eur Respir J 2005;26:586–93.
5.
Cottin V, Cordier JF. Combined pulmonary fibrosis and emphysema in connective tissue disease. Curr Opin Pulm Med 2012;18:418-27.
6.
Tzouvelekis A, Zacharis G, Oikonomou A, et al. Increased incidence of autoimmune markers in patients with combined pulmonary fibrosis and emphysema. BMC Pulm Med 2013;13:31.
7.
Flaherty KR, Fell C, Aubry MC, et al. Smoking-related idiopathic interstitial pneumonia. Eur Respir J 2014; 44:594–602.
8.
Vassallo R, Ryu JH. Smoking-related interstitial lung diseases. Clin Chest Med 2012; 33:165–78.
9.
Travis WD, Costabel U, Hansell DM, et al. An Official American Thoracic Society/EuropeanRespiratory Society Statement: Update of the International Multidisciplinary Classification of the Idiopathic Interstitial Pneumonias. Am J Respir Crit Care Med 2013;188:733–48.
10.
Yousem SA. Respiratory bronchiolitis-associated interstitial lung disease with fibrosis is a lesion distinct fromfibrotic nonspecific interstitial pneumonia: a proposal. Mod Pathol 2006;19:1474–9.
11.
Katzenstein AL, Mukhopadhyay S, Zanardi C, Dexter E. Clinically occult interstitial lfibrosis in smokers: classification and significance of a surprisingly commonfinding in lobectomy specimens. Hum Pathol 2010;41:316–25.
12.
Katzenstein AL. Smoking-related interstitial fibrosis (SRIF): pathologic findings and distinction from other chronicfibrosing lung diseases. J Clin Pathol 2013;66:882–7.
13.
Kawabata Y, Hoshi E, Murai K, et al. Smoking related changes in the background lung of specimens resected for lung cancer: a semiquantitative study with correlation to postoperative course. Histopathology 2008;53:707–14.
14.
Yamada T, Nakanishi Y, Homma T, et al. Airspace enlargement with fibrosis shows characteristic histology and immunohistology different from usual interstitial pneumonia, nonspecific interstitial pneumonia and centrilobular emphysema. Pathol Int 2013;63:206–13.
15.
Reddy TL, Mayo J, Churg A. Respiratory Bronchiolitis with Fibrosis High-Resolution Computed Tomography Findings and Correlation with Pathology. Ann Am Thorac Soc 2013; 10:590-601.
16.
Jankowich MD, Rounds SI. Combined pulmonary fibrosis and emphysema syndrome: a review. Chest 2012; 141:222–31.
17.
Armanios M. Telomeres and age-related disease: how telomere biology informs clinical paradigms. J Clin Invest 2013;123:996– 1002.
18.
Greider CW, Blackburn EH. A telomeric sequence in the RNA of Tetrahymena telomerase required for telomere repeat synthesis. Nature 1989;337(6205):331–7.
19.
Lingner J, Hughes TR, Shevchenko A, Mann M, Lundblad V, Cech TR. Reverse transcriptase motifs in the catalytic subunit of telomerase. Science 1997;276(5312):561–7.
20.
Alder JK, Guo N, Kembou F, et al. Telomere Length is a Determinant of Emphysema Susceptibility. Am J Respir Crit Care Med 2011; 184:904–12.
21.
Armanios M. Telomerase and idiopathic pulmonary fibrosis. Mutat Res 2012;730(1-2):52-8.
22.
Stanley SE, Chen JJ, Podlevsky JD, et al. Telomerase mutations in smokers with severe emphysema. J Clin Invest 2015;125:563-70.
23.
Armanios MY, Chen JJ, Cogan JD, et al. Telomerase mutations in families withidiopathic pulmonary fibrosis. N Engl J Med 2007;356:1317–26.
24.
Tsakiri KD, Cronkhite JT, Kuan PJ, et al. Adult-onset pulmonary fibrosis caused by mutations in telomerase. Proc Natl Acad Sci U S A 2007; 104:7552–7.
25.
Alder JK, Chen JJ, Lancaster L, et al. Short telomeres are a risk factor for idiopathic pulmonary fibrosis. Proc Natl Acad Sci U S A 2008;105:13051–6.
26.
Cronkhite JT, Xing C, Raghu G, et al. Telomere shortening in familial and sporadic pulmonary fibrosis. Am J Respir Crit Care Med 2008; 178:729–37.
27.
Nunes H, Monnet I, Kannengiesser C, et al. Is telomeropathy the explanation for combined pulmonary fibrosis and emphysema syndrome?: Report of a family with TERT mutation. Am J Respir Crit Care Med 2014;189:753-4.
28.
Armanios M. Reply: telomerase makes connections between pulmonary fibrosis and emphysema. Am J Respir Crit Care Med 2014; 189:754-5.
29.
Armanios M. Syndromes of telomere shortening. Annu Rev Genomics Hum Genet 2009; 10:45–61.
30.
Gribbin J, Hubbard R, Smith C. Role of diabetes mellitus and gastro-oesophageal reflux in the aetiology of idiopathic pulmonary fibrosis. Respir Med 2009; 103:927–31.
31.
Garcia-Sancho Figueroa MC, Carrillo G, Perez-Padilla R, et al. Risk factors for idiopathic pulmonary fibrosis in a Mexican population. A case-control study. Respir Med 2010; 104:305–9.
32.
Armanios M, Chen JL, Chang YP, et al. Haploinsufficiency of telomerase reverse transcriptase leads to anticipation in autosomal dominant dyskeratosis congenita. Proc Natl Acad Sci U S A 2005;102:15960–4.
33.
Diaz de Leon A, Cronkhite JT, Katzenstein AL, et al. Telomere lengths, pulmonary fibrosis and telomerase (TERT) mutations. PLoS One 2010; 5 e10680.
34.
Kirwan M, Vulliamy T, Marrone A, et al. Defining the pathogenic role of telomerase mutations in myelodysplastic syndrome and acute myeloid leukemia. Hum Mutat 2009; 30:1567–73.
35.
Parry EM, Alder JK, Qi X, Chen JJ, Armanios M. Syndrome complex of bone marrow failure and pulmonary fibrosis predicts germline defects in telomerase. Blood 2011; 117:5607–11.
36.
Silhan LL, Shah PD, Chambers DC, et al. Lung transplantation in telomerase mutation carriers with pulmonary fibrosis. Eur Respir 2014;44:178-87.
37.
Amin RS, Wert SE, Baughman RP, et al. Surfactant protein deficiency in familial interstitial lung disease. J Pediatr 2001; 139:85-92.
38.
Thomas AQ, Lane K, Phillips J, 3rd, 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.
39.
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-L729.
40.
Cottin V, Reix P, Khouatra C, Thivolet-Béjui F, Feldmann D, Cordier JF. Combined pulmonary fibrosis and emphysema syndrome associated with familial SFTPCmutation. Thorax 2011; 66:918–9.
41.
Bullard JE, Wert SE, Whitsett JA, Dean M, Nogee LM. ABCA3 mutations associated with pediatric interstitial lung disease. Am J Respir Crit Care Med 2005; 172:1026-31.
42.
Crossno PF, Polosukhin VV, Blackwell TS, et al. Identification of early interstitial lung disease in an individual with genetic variations in ABCA3 and SFTPC. Chest 2010; 137:969-73.
43.
Young LR, Nogee LM, Barnett B, Panos RJ, Colby TV, Deutsch GH. Usual interstitial pneumonia in an adolescent with ABCA3 mutations. Chest 2008;134:192-5.
44.
Epaud R, Delestrain C, Louha M, Simon S, Fanen P, Tazi A. Combined pulmonary fibrosis and emphysema syndrome associated with ABCA3 mutations. Eur Respir 2014;43:638-41.
45.
Chaudhuri R, Mcsharry C, Spears M, et al. Sputum matrix metalloproteinase-9 is associated with the degree of emphysema on computed tomography in COPD. Translational Respiratory Medicine 2013;1:11.
46.
Yu Q, Stamenkovic I. Cell surface-localized matrix metalloproteinase-9 proteolytically activates TGF-β and promotes tumor invasion and angiogenesis. Genes Dev 2000; 14:163-176.
47.
Fernandez IE, Eickelberg O. The impact of TGF-β on lung fibrosis: from targeting to biomarkers. Proc Am Thorac Soc 2012;9:111-6.
48.
Xu B, Chen H, Xu W, et al. Molecular mechanisms of MMP9 overexpression and its role in emphysema pathogenesis of Smad3-deficient mice. Am J Physiol Lung Cell Mol Physiol 2012;303:L89-96.
49.
Warburton D, Shi W, Xu B. TGF-β-Smad3 signaling in emphysema and pulmonary fibrosis: an epigenetic aberration of normal development? Am J Physiol Lung Cell Mol Physiol 2013;304:L83-5.
50.
Gauldie J, Kolb M, Ask K, et al. Smad3 signaling involved in pulmonary fibrosis and emphysema. Proc Am Thorac Soc 2006;3:696-702.
51.
Lundblad LK, Thompson-Figueroa J, Leclair T, et al. Tumor necrosis factor-α overexpression in lung disease: a single cause behind a complex phenotype. Am J Respir Crit Care Med 2005;171:1363–70.
52.
Hoyle GW, Li J, Finkelstein JB, et al. Emphysematous lesions, inflammation, and fibrosis in the lungs of transgenic mice overexpressing platelet-derived growth factor. Am J Pathol 1999; 154:1763–75.
53.
Lucattelli M, Bartalesi B, Cavarra E, et al. Is neutrophil elastase the missing link between emphysema and fibrosis? Evidence from two mouse models. Respir Res 2005;6:83.
54.
Annes JP, Munger JS, Rifkin DB. Making sense of latent TGFbeta activation. J Cell Sci 2003;116:217–224.
55.
Keane MP, Arenberg DA, Lynch JP, III, et al. The CXC chemokines, IL-8 and IP-10, regulate angiogenic activity in idiopathic pulmonary fibrosis. J Immunol 1997;159:1437–43.
56.
Keane MP, Belperio JA, Burdick MD, et al. ENA-78 is an important angiogenic factor in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 2001;164:2239–42.
57.
Mukaida N. Pathophysiological roles of interleukin-8/CXCL8 in pulmonary diseases. Am J Physiol Lung Cell Mol Physiol 2003; 284:L566–773.
58.
Tasaka S, Mizoguchi K, Funatsu Y, et al. Cytokine profile of bronchoalveolar lavage fluid in patients with combined pulmonary fibrosis and emphysema. Respirology 2012;17:814-20.
59.
Fulkerson PC, Fischetti CA, Hassman LM, et al. Persistent effects induced by IL-13 in the lung. Am J Respir Cell Mol Biol 2006; 35:337–46.
60.
Lumsden RV, Worrell JC, Boylan D, et al. Modulation of pulmonary fibrosis by IL-13Rα2. Am J Physiol Lung Cell Mol Physiol 2015;308:L710-8.
61.
Murray LA, Zhang H, Oak SR, et al. Targeting interleukin-13 with tralokinumab attenuates lung fibrosis and epithelial damage in a humanized SCID idiopathic pulmonary fibrosis model. Am J Respir Cell Mol Biol 2014;50:985-94.
62.
Volonte D, Kahkonen B, Shapiro S, et al. Caveolin-1 expression is required for the development of pulmonary emphysema through activation of the ATM-p53-p21 pathway. J Biol Chem 2009;284:5462-6.
63.
Wang XM, Zhang Y, Kim HP, et al. Caveolin-1: a critical regulator of lung fibrosis in idiopathic pulmonary fibrosis. J Exp Med 2006; 203:2895–906.
64.
Castello-Cros R, Whitaker-Menezes D, Molchansky A, et al. Scleroderma-like properties of skin from caveolin-1-deficient mice: implications for new treatment strategies in patients with fibrosis and systemic sclerosis. Cell Cycle 2011;10:2140-50.
65.
Tzouvelekis A, Bouros E, Bouros D. The Immunology of pulmonary fibrosis: The role of Th1/Th2/Th17/Treg cells. Pneumon 2010;1:17-20.
66.
Kotsianidis I, Nakou E, Bouchliou I, et al.Global impairment of CD4+CD25+FOXP3+ regulatory T cells in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 2009;179:1121-30.
67.
Lee SH, Goswami S, Grudo A, et al. Antielastin autoimmunity in tobacco smoking–induced emphysema. Nat Med 2007;13:567–9.
68.
Isajevs S, Taivans I, Strazda G, et al. Decreased FOXP3 expression in small airways of smokers with COPD. Eur Respir J 2009;33:61–7.
69.
Hou J, Sun Y, Hao Y, et al. Imbalance between subpopulations of regulatory T cells in COPD. Thorax 2013;68:1131–9.
70.
Tzouvelekis A, Bouros D. COPD and IPF: it›s all about regulation and balance. Thorax 2014;69:490.
71.
Corte TJ, Wort SJ, Gatzoulis MA, et al. Pulmonary vascular resistance predicts early mortality in patients with diffuse fibrotic lung disease and suspected pulmonary hypertension. Thorax 2009;64:883–8.
72.
Hamada K, Nagai S, Tanaka S, et al. Significance of pulmonary arterial pressure and diffusion capacity of the lung as prognosticator in patients with idiopathic pulmonary fibrosis. Chest 2007;131:650–6.
73.
Lettieri CJ, Nathan SD, Barnett SD, et al. Prevalence and outcomes of pulmonary arterial hypertension in advanced idiopathic pulmonary fibrosis. Chest 2006;129:746–52.
74.
Kwak N, Park CM, Lee J, et al. Lung cancer risk among patients with combined pulmonary fibrosis and emphysema. Respir Med 2014;108:524e30.
75.
Usui K, Tanai C, Tanaka Y, Noda H, Ishihara T. The prevalence of pulmonary fibrosis combined with emphysema in patients with lung cancer. Respirology 2011;16:326-31.
76.
Tzouvelekis A, Laurent G, Bouros D. Stem cell therapy in chronic obstructive pulmonary disease. Seeking the Prometheus effect. Curr Drug Targets 2013;14:246-52.
77.
Tzouvelekis A, Antoniadis A, Bouros D. Stem cell therapy in pulmonary fibrosis. Curr Opin Pulm Med 2011;17:368-73.
78.
Tzouvelekis A, Paspaliaris V, Koliakos G, et al. A prospective, non-randomized, no placebo-controlled, phase Ib clinical trial to study the safety of the adipose derived stromal cells-stromal vascular fraction in idiopathic pulmonary fibrosis. J Transl Med 2013;11:171.
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