Biomarkers in the evaluation and management of idiopathic pulmonary fibrosis

Current Topics in Medicinal Chemistry

Volumn 16, Issue 14, 2016, Pages 1587-1598

  Tzouvelekis, Argyris ^a   Herazo Maya, Jose ^a   Sakamoto, Koji ^a   Bouros, Demosthenes ^b  

^a YALE UNIVERSITY SCHOOL OF MEDICINE   (United States)

^b UNIVERSITY OF ATHENS MEDICAL SCHOOL   (Greece)

Author keywords

Alveolar epithelial cell injury; Biomarkers; Extracellular matrix; Idiopathic Pulmonary Fibrosis; Immunity

Indexed keywords

CXCL13 CHEMOKINE; DEFENSIN; HEAT SHOCK PROTEIN 70; MATRIX METALLOPROTEINASE; MUCIN 5B; MUCIN LIKE CARCINOMA ASSOCIATED ANTIGEN; OSTEOPONTIN; PROTEIN LYSINE 6 OXIDASE; SURFACTANT ASSOCIATED PROTEIN; TOLL LIKE RECEPTOR 3; BIOLOGICAL MARKER;

ADAPTIVE IMMUNITY; ARTICLE; BLOOD VESSEL PERMEABILITY; BRONCHOALVEOLAR LAVAGE FLUID; CELL DAMAGE; DISEASE COURSE; DISEASE PREDISPOSITION; DISEASE SEVERITY; EMPHYSEMA; FIBROSING ALVEOLITIS; GENE; GENE MUTATION; HUMAN; INNATE IMMUNITY; INTERSTITIAL LUNG DISEASE; LUNG ADENOCARCINOMA; LUNG FUNCTION TEST; MICROBIOME; PHENOTYPE; PULMONARY HYPERTENSION; REGULATORY T LYMPHOCYTE; SINGLE NUCLEOTIDE POLYMORPHISM; TELOMERE; TOLLIP GENE; TREATMENT RESPONSE; IDIOPATHIC PULMONARY FIBROSIS; LUNG NEOPLASMS;

BIOMARKERS; HUMANS; IDIOPATHIC PULMONARY FIBROSIS; LUNG NEOPLASMS;

EID: 84964226751     PISSN: 15680266     EISSN: 18734294     Source Type: Journal    
DOI: 10.2174/1568026616666150930120959     Document Type: Article

References (121)

1. [1] Raghu, G., Collard, H.R., Egan, J.J., Martinez, F.J., Behr, J., Brown, K.K., Colby, T.V., Cordier, J.F., Flaherty, K.R., Lasky, J.A., Lynch, D.A., Ryu, J.H., Swigris, J.J., Wells, A.U., Ancochea, J., Bouros, D., Carvalho, C., Costabel, U., Ebina, M., Hansell, DM., Johkoh, T., Kim, D.S., King, Jr. T.E.,, Kondoh, Y., Myers, J., Muller, N.L., Nicholson, A.G., Richeldi, L., Selman, M., Dudden, R.F., Griss, B.S., Protzko, S.L., Schunemann, H.J. Fibrosis AEJACoIP. 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.
2. [2] Raghu, G., Weycker, D., Edelsberg, J., Bradford, W.Z., Oster, G. Incidence and prevalence of idiopathic pulmonary fibrosis. Am. J. Respir. Crit. Care Med., 2006, 174, 810-816.
3. [3] Ley, B., Brown, K.K., Collard, H.R. Molecular biomarkers in idiopathic pulmonary fibrosis. Am. J. Physiol. Lung Cell. Mol. Physiol., 2014, 307, L681-691.
4. [4] Spagnolo, P., Sverzellati, N., Rossi, G., Cavazza, A., Tzouvelekis, A., Crestani, B., Vancheri, C. Idiopathic pulmonary fibrosis, An update. Annals Med., 2015, 47, 15-27.
5. [5] Rosas, I.O., Kaminski, N. Update in diffuse parenchymal lung disease 2013. Am. J. Respir. Crit. Care Med., 2015, 191, 270-274.
6. [6] Zhang, Y., Kaminski, N. Biomarkers in idiopathic pulmonary fibrosis. Curr. Opin. Pulm. Med., 2012, 18, 441-446.
7. [7] Tzouvelekis, A., Kouliatsis, G., Anevlavis, S., Bouros, D. Serum biomarkers in interstitial lung diseases. Respir. Res., 2005, 6, 78.
8. [8] Selman, M., Carrillo, G., Estrada, A., Mejia, M., Becerril, C., Cisneros, J., Gaxiola, M., Perez-Padilla, R., Navarro, C., Richards, T., Dauber, J., King, Jr. TE.,, Pardo, A., Kaminski, N. Accelerated variant of idiopathic pulmonary fibrosis, clinical behavior and gene expression pattern. PloS one, 2007, 2, e482.
9. [9] Flaherty, K.R., Mumford, J.A., Murray, S., Kazerooni, E.A., Gross, B.H., Colby, T.V., Travis, W.D., Flint, A., Toews, G.B., Lynch, 3rd J.P.., Martinez, F.J. Prognostic implications of physiologic and radiographic changes in idiopathic interstitial pneumonia. Am. J. Respir. Crit. Care Med., 2003, 168, 543-548.
10. [10] Latsi, PI., du Bois, R.M., Nicholson, A.G., Colby, T.V., Bisirtzoglou, D., Nikolakopoulou, A., Veeraraghavan, S., Hansell, D.M., Wells, A.U. Fibrotic idiopathic interstitial pneumonia, the prognostic value of longitudinal functional trends. Am. J. Respir. Crit. Care Med., 2003, 168, 531-537.
11. [11] du, Bois, R.M., Weycker, D., Albera, C., Bradford, W.Z., Costabel, U., Kartashov, A., King, T.E. Jr., Lancaster, L., Noble, P.W., Sahn, S.A., Thomeer, M., Valeyre, D., Wells, A.U. Forced vital capacity in patients with idiopathic pulmonary fibrosis, test properties and minimal clinically important difference. Am. J. Respir. Crit. Care Med., 2011, 184, 1382-1389.
12. [12] Ley, B., Ryerson, C.J., Vittinghoff, E., Ryu, J.H., Tomassetti, S., Lee, J.S., Poletti, V., Buccioli, M., Elicker, B.M., Jones, K.D., King, T.E. Jr., Collard, H.R. A multidimensional index and staging system for idiopathic pulmonary fibrosis. Ann. Intern. Med., 2012, 156, 684-691.
13. [13] Wells, A.U., Desai, S.R., Rubens, M.B., Goh, N.S., Cramer, D., Nicholson, A.G., Colby, T.V., du Bois, R.M., Hansell, D.M. Idiopathic pulmonary fibrosis, a composite physiologic index derived from disease extent observed by computed tomography. Am. J. Respir. Crit. Care Med., 2003, 167, 962-969.
14. [14] Swigris, J.J., Wamboldt, F.S., Behr, J., du Bois, R.M., King, T.E., Raghu, G., Brown, K.K. The 6 minute walk in idiopathic pulmonary fibrosis, longitudinal changes and minimum important difference. Thorax, 2010, 65, 173-177.
15. [15] Du Bois, R.M., Weycker, D., Albera, C., Bradford, W.Z., Costabel, U., Kartashov, A., Lancaster, L., Noble, P.W., Sahn, SA., Szwarcberg, J., Thomeer, M., Valeyre, D., King, T.E. Jr. Six-minutewalk test in idiopathic pulmonary fibrosis, test validation and minimal clinically important difference. Am. J. Respir. Crit. Care Med., 2011, 183, 1231-1237.
16. [16] Maher, T.M. Profileing idiopathic pulmonary fibrosis, rethinking biomarker discovery. Eur. Respir. Rev. J. Euro. Respir. Soc., 2013, 22, 148-152.
17. [17] Possmayer, F. A proposed nomenclature for pulmonary surfactantassociated proteins. Am. Rev. Respir. Dis., 1988, 138, 990-998.
18. [18] Tanjore, H., Blackwell, T.S., Lawson, W.E. Emerging evidence for endoplasmic reticulum stress in the pathogenesis of idiopathic pulmonary fibrosis. Am. J. Physiol. Lung cell. Mol. Physiol., 2012, 302, L721-729.
19. [19] Bridges, J.P., Xu, Y., Na, C.L., Wong, H.R., Weaver, T.E. Adaptation and increased susceptibility to infection associated with constitutive expression of misfolded SP-C. J. Cell Biol., 2006, 172, 395-407.
20. [20] Bridges, J.P., Wert, S.E., Nogee, L.M., Weaver, T.E. Expression of a human surfactant protein C mutation associated with interstitial lung disease disrupts lung development in transgenic mice. J. Biol. Chem., 2003, 278, 52739-52746.
21. [21] Maitra, M., Dey, M., Yuan, W.C., Nathanielsz, P.W., Garcia, C.K. Lung fibrosis-associated surfactant protein A1 and C variants induce latent transforming growth factor beta1 secretion in lung epithelial cells. J. Biol. Chem., 2013, 288, 27159-27171.
22. [22] Maitra, M., Cano, C.A., Garcia, C.K. Mutant surfactant A2 proteins associated with familial pulmonary fibrosis and lung cancer induce TGF-beta1 secretion. Proc. Natl. Acad. Sci. U. S. A., 2012, 109, 21064-21069.
23. [23] Maitra, M., Wang, Y., Gerard, R.D., Mendelson, C.R., Garcia, C.K. Surfactant protein A2 mutations associated with pulmonary fibrosis lead to protein instability and endoplasmic reticulum stress. J. Biol. Chem., 2010, 285, 22103-22113.
24. [24] Lawson, W.E., Cheng, D.S., Degryse, A.L., Tanjore, H., Polosukhin, V.V., Xu, X.C., Newcomb, D.C., Jones, B.R., Roldan, J., Lane, K.B., Morrisey, E.E., Beers, M.F., Yull, F.E., Blackwell, T.S. Endoplasmic reticulum stress enhances fibrotic remodeling in the lungs. Proc. Natl. Acad. Sci. U. S. A., 2011, 108, 10562-10567.
25. [25] Ohnishi, H., Yokoyama, A., Kondo, K., Hamada, H., Abe, M., Nishimura, K., Hiwada, K., Kohno, N. Comparative study of KL-6, surfactant protein-A., surfactant protein-D., and monocyte chemoattractant protein-1 as serum markers for interstitial lung diseases. Am. J. Respir. Crit. Care Med., 2002, 165, 378-381.
26. [26] Ishii, H., Mukae, H., Kadota, J., Kaida, H., Nagata, T., Abe, K., Matsukura, S., Kohno, S. High serum concentrations of surfactant protein A in usual interstitial pneumonia compared with nonspecific interstitial pneumonia. Thorax, 2003, 58, 52-57.
27. [27] Greene, KE., Ye, S., Mason, R.J., Parsons, P.E. Serum surfactant protein-A levels predict development of ARDS in at-risk patients. Chest, 1999, 116, 90S-91S.
28. [28] Song, J.W., Do, K.H., Jang, S.J., Colby, T.V., Han, S., Kim, D.S. Blood biomarkers MMP-7 and SP-A, predictors of outcome in idiopathic pulmonary fibrosis. Chest, 2013, 143, 1422-1429.
29. [29] Kohno, N., Akiyama, M., Kyoizumi, S., Hakoda, M., Kobuke, K., Yamakido, M. Detection of soluble tumor-associated antigens in sera and effusions using novel monoclonal antibodies., KL-3 and KL-6, against lung adenocarcinoma. Jpn. J. Clin. Oncol., 1988, 18, 203-216.
30. [30] Collard, H.R., Calfee, C.S., Wolters, P.J., Song, J.W., Hong, S.B., Brady, S., Ishizaka, A., Jones, K.D., King, T.E. Jr., Matthay, M.A., Kim, D.S. Plasma biomarker profiles in acute exacerbation of idiopathic pulmonary fibrosis. Am. J. Physiol. Lung Cell. Mol. Physiol., 2010, 299, L3-7.
31. [31] Taniguchi, H., Ebina, M., Kondoh, Y., Ogura, T., Azuma, A., Suga, M., Taguchi, Y., Takahashi, H., Nakata, K., Sato, A., Takeuchi, M., Raghu, G., Kudoh, S., Nukiwa, T., Pirfenidone Clinical Study Group in J. Pirfenidone in idiopathic pulmonary fibrosis. Euro. Respir. J., 2010, 35, 821-829.
32. [32] Azuma, A., Nukiwa, T., Tsuboi, E., Suga, M., Abe, S., Nakata, K., Taguchi, Y., Nagai, S., Itoh, H., Ohi, M., Sato, A., Kudoh, S. Double-blind placebo-controlled trial of pirfenidone in patients with idiopathic pulmonary fibrosis. Am. J. Respir. Crit. Care Med., 2005, 171, 1040-1047.
33. [33] Kohno, N., Kyoizumi, S., Awaya, Y., Fukuhara, H., Yamakido, M., Akiyama, M. New serum indicator of interstitial pneumonitis activity. Sialylated carbohydrate antigen KL-6. Chest, 1989, 96, 68-73.
34. [34] Yokoyama, A., Kohno, N., Hamada, H., Sakatani, M., Ueda, E., Kondo, K., Hirasawa, Y., Hiwada, K. Circulating KL-6 predicts the outcome of rapidly progressive idiopathic pulmonary fibrosis. Am. J. Respir. Crit. Care Med., 1998, 158, 1680-1684.
35. [35] Yokoyama, A., Kohno, N., Kondo, K., Ueda, S., Hirasawa, Y., Watanabe, K., Takada, Y., Hiwada, K. Comparative evaluation of sialylated carbohydrate antigens, KL-6, CA19-9 and SLX as serum markers for interstitial pneumonia. Respirology, 1998, 3, 199-202.
36. [36] Kohno, N., Awaya, Y., Oyama, T., Yamakido, M., Akiyama, M., Inoue, Y., Yokoyama, A., Hamada, H., Fujioka, S., Hiwada, K. KL-6, a mucin-like glycoprotein, in bronchoalveolar lavage fluid from patients with interstitial lung disease. Am. Rev. Respir. Dis., 1993, 148, 637-642.
37. [37] Kishaba, T., Tamaki, H., Shimaoka, Y., Fukuyama, H., Yamashiro, S. Staging of acute exacerbation in patients with idiopathic pulmonary fibrosis. Lung, 2014, 192, 141-149.
38. [38] Yano, M., Sasaki, H., Moriyama, S., Hikosaka, Y., Yokota, K., Kobayashi, S., Hara, M., Fujii, Y. Post-operative acute exacerbation of pulmonary fibrosis in lung cancer patients undergoing lung resection. Interact. Cardiovasc. Thorac. Surg., 2012, 14, 146-150.
39. [39] Satoh, H., Kurishima, K., Ishikawa, H., Ohtsuka, M. Increased levels of KL-6 and subsequent mortality in patients with interstitial lung diseases. J. Intern. Med., 2006, 260, 429-434.
40. [40] Roy, M.G., Livraghi-Butrico, A., Fletcher, A.A., McElwee, M.M., Evans, S.E., Boerner, R.M., Alexander, S.N., Bellinghausen, L.K., Song, A.S., Petrova, Y.M., Tuvim, M.J., Adachi, R., Romo, I., Bordt, A.S., Bowden, M.G., Sisson, J.H., Woodruff, P.G., Thornton, D.J., Rousseau, K., De la Garza, M.M., Moghaddam, S.J., Karmouty-Quintana, H., Blackburn, M.R., Drouin, S.M., Davis, C.W., Terrell, K.A., Grubb, B.R., O'Neal, W.K., Flores, S.C., Cota-Gomez, A., Lozupone, C.A., Donnelly, J.M., Watson, A.M., Hennessy, C.E., Keith, R.C., Yang, IV., Barthel, L., Henson, P.M., Janssen, W.J., Schwartz, D.A., Boucher, R.C., Dickey, B.F., Evans, C.M. Muc5b is required for airway defence. Nature, 2014, 505, 412-416.
41. [41] Seibold, M.A., Wise, A.L., Speer, M.C., Steele, M.P., Brown, K.K., Loyd, J.E., Fingerlin, T.E., Zhang, W., Gudmundsson, G., Groshong, S.D., Evans, C.M., Garantziotis, S., Adler, K.B., Dickey, B.F., du Bois, R.M., Yang, I.V., Herron, A., Kervitsky, D., Talbert, J.L., Markin, C., Park, J., Crews, A.L., Slifer, S.H., Auerbach, S., Roy, M.G., Lin, J., Hennessy, C.E., Schwarz, M.I., Schwartz, D.A. A common MUC5B promoter polymorphism and pulmonary fibrosis. N. Engl. J. Med., 2011, 364, 1503-1512.
42. [42] Borie, R., Crestani, B., Dieude, P., Nunes, H., Allanore, Y., Kannengiesser, C., Airo, P., Matucci-Cerinic, M., Wallaert, B., Israel-Biet, D., Cadranel, J., Cottin, V., Gazal, S., Peljto, A.L., Varga, J., Schwartz, D.A., Valeyre, D., Grandchamp, B. 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, e70621.
43. [43] Zhang, Y., Noth, I., Garcia, J.G., Kaminski, N. A variant in the promoter of MUC5B and idiopathic pulmonary fibrosis. N. Engl. J. Med., 2011, 364, 1576-1577.
44. [44] Wang, C., Zhuang, Y., Guo, W., Cao, L., Zhang, H., Xu, L., Fan, Y., Zhang, D., Wang, Y. Mucin, 5B promoter polymorphism is associated with susceptibility to interstitial lung diseases in Chinese males. PloS one, 2014, 9, e104919.
45. [45] Hunninghake, G.M., Hatabu, H., Okajima, Y., Gao, W., Dupuis, J., Latourelle, J.C., Nishino, M., Araki, T., Zazueta, O.E., Kurugol, S., Ross, J.C., San Jose, E.R., Murphy, E., Steele, M.P., Loyd, J.E., Schwarz, M.I., Fingerlin, T.E., Rosas, I.O., Washko, G.R., O'Connor, G.T., Schwartz, D.A. MUC5B promoter polymorphism and interstitial lung abnormalities. N. Engl. J. Med., 2013, 368, 2192-2200.
46. [46] Fingerlin, T.E., Murphy, E., Zhang, W., Peljto, A.L., Brown, K.K., Steele, M.P., Loyd, J.E., Cosgrove, G.P., Lynch, D., Groshong, S., Collard, H.R., Wolters, P.J., Bradford, W.Z., Kossen, K., Seiwert, S.D., du Bois, R.M., Garcia, C.K., Devine, M.S., Gudmundsson, G., Isaksson, H.J., Kaminski, N., Zhang, Y., Gibson, K.F., Lancaster, L.H., Cogan, J.D., Mason, W.R., Maher, T.M., Molyneaux, P.L., Wells, A.U., Moffatt, M.F., Selman, M., Pardo, A., Kim, D.S., Crapo, J.D., Make, B.J., Regan, E.A., Walek, D.S., Daniel, J.J., Kamatani, Y., Zelenika, D., Smith, K., McKean, D., Pedersen, B.S., Talbert, J., Kidd, R.N., Markin, C.R., Beckman, K.B., Lathrop, M., Schwarz, M.I., Schwartz, D.A. Genome-wide association study identifies multiple susceptibility loci for pulmonary fibrosis. Nature Genet., 2013, 45, 613-620.
47. [47] Peljto, A.L., Zhang, Y., Fingerlin, T.E., Ma, S.F., Garcia, J.G., Richards, T.J., Silveira, L.J., Lindell, K.O., Steele, M.P., Loyd, J.E., Gibson, K.F., Seibold, M.A., Brown, K.K., Talbert, J.L., Markin, C., Kossen, K., Seiwert, S.D., Murphy, E., Noth, I., Schwarz, M.I., Kaminski, N., Schwartz, D.A.Association between the MUC5B promoter polymorphism and survival in patients with idiopathic pulmonary fibrosis. JAMA, J. Am. Med. Assoc., 2013, 309, 2232-2239.
48. [48] Lee, H.W., Blasco, M.A., Gottlieb, G.J., Horner, 2nd J.W., Greider, C.W., DePinho, RA. Essential role of mouse telomerase in highly proliferative organs. Nature, 1998, 392, 569-574.
49. [49] Jaskelioff, M., Muller, F.L., Paik, J.H., Thomas, E., Jiang, S., Adams, A.C., Sahin, E., Kost-Alimova, M., Protopopov, A., Cadinanos, J., Horner, J.W., Maratos-Flier, E., Depinho, R.A. Telomerase reactivation reverses tissue degeneration in aged telomerasedeficient mice. Nature, 2011, 469, 102-106.
50. [50] Liu, T., Nozaki, Y., Phan, S.H. Regulation of telomerase activity in rat lung fibroblasts. Am. J. Respir. Cell Mol. Biol., 2002, 26, 534-540.
51. [51] Nozaki, Y., Liu, T., Hatano, K., Gharaee-Kermani, M., Phan, S.H. Induction of telomerase activity in fibroblasts from bleomycininjured lungs. Am. J. Respir. Cell Mol. Biol., 2000, 23, 460-465.
52. [52] Armanios, M.Y., Chen, J.J., Cogan, J.D., Alder, J.K., Ingersoll, R.G., Markin, C., Lawson, W.E., Xie, M., Vulto, I., Phillips, 3rd J.A., Lansdorp, P.M., Greider, C.W., Loyd, J.E. Telomerase mutations in families with idiopathic pulmonary fibrosis. N. Engl. J. Med., 2007, 356, 1317-1326.
53. [53] Tsakiri, K.D., Cronkhite, J.T., Kuan, P.J., Xing, C., Raghu, G., Weissler, J.C., Rosenblatt, R.L., Shay, J.W., Garcia, C.K. Adultonset pulmonary fibrosis caused by mutations in telomerase. Proc. Natl. Acad. Sci. U. S. A., 2007, 104, 7552-7557.
54. [54] Kannengiesser, C., Borie, R., Menard, C., Reocreux, M., Nitschke, P., Gazal, S., Mal, H., Taille, C., Cadranel, J., Nunes, H., Valeyre, D., Cordier, J.F., Callebaut, I., Boileau, C., Cottin, V., Grandchamp, B., Revy, P., Crestani, B. Heterozygous RTEL1 mutations are associated with familial pulmonary fibrosis. Euro. Respir. J., 2015, 46(2), 474-85
55. [55] Cronkhite, J.T., Xing, C., Raghu, G., Chin, K.M., Torres, F., Rosenblatt, R.L., Garcia, C.K. Telomere shortening in familial and sporadic pulmonary fibrosis. Am. J. Respir. Crit. Care Med., 2008, 178, 729-737.
56. [56] Stuart, B.D., Lee, J.S., Kozlitina, J., Noth, I., Devine, M.S., Glazer, C.S., Torres, F., Kaza, V., Girod, C.E., Jones, K.D., Elicker, B.M., Ma, S.F., Vij, R., Collard, H.R., Wolters, P.J., Garcia, C.K. Effect of telomere length on survival in patients with idiopathic pulmonary fibrosis, an observational cohort study with independent validation. lancet Respir. Med., 2014, 2, 557-565.
57. [57] Silhan, L.L., Shah, P.D., Chambers, D.C., Snyder, L.D., Riise, G.C., Wagner, C.L., Hellstrom-Lindberg, E., Orens, J.B., Mewton, J.F., Danoff, S.K., Arcasoy, M.O., Armanios, M. Lung transplantation in telomerase mutation carriers with pulmonary fibrosis. Euro. Respir. J., 2014, 44(1),178-87.
58. [58] Borie, R., Kannengiesser, C., Hirschi, S., Le Pavec, J., Mal, H., Bergot, E., Jouneau, S., Naccache, J.M., Revy, P., Boutboul, D., Peffault de laTour, R., Wemeau-Stervinou, L., Philit, F., Cordier, J.F., Thabut, G., Crestani, B., Cottin, V., Groupe d'Etudes et de Recherche sur les Maladies "Orphelines P. Severe hematologic complications after lung transplantation in patients with telomerase complex mutations. J. Heart Lung Transplant., 2015, 34, 538-546.
59. [59] Alder, J.K., Chen, J.J., Lancaster, L., Danoff, S., Su, S.C., Cogan, J.D., Vulto, I., Xie, M., Qi, X., Tuder, R.M., Phillips, 3rd J.A., Lansdorp, P.M., Loyd, J.E., Armanios, M.Y. Short telomeres are arisk factor for idiopathic pulmonary fibrosis. Proc. Natl. Acad. Sci. U. S. A., 2008, 105, 13051-13056.
60. [60] Povedano, J.M., Martinez, P., Flores, J.M., Mulero, F., Blasco, M.A. Mice with Pulmonary Fibrosis Driven by Telomere Dysfunction. Cell reports, 2015, 12, 286-299.
61. [61] Armanios, M. Telomerase mutations and the pulmonary fibrosisbone marrow failure syndrome complex. N. Engl. J. Med., 2012, 367: 384, DOI: 10.1056/NEJMc1206730
62. [62] Parry, E.M., Alder, J.K., Qi, X., Chen, J.J., Armanios, M. Syndrome complex of bone marrow failure and pulmonary fibrosis predicts germline defects in telomerase. Blood, 2011, 117, 5607-5611.
63. [63] Idiopathic Pulmonary Fibrosis Clinical Research N., Raghu, G., Anstrom, K.J., King, T.E. Jr., Lasky, J.A., Martinez, F.J. Prednisone., azathioprine., and N-acetylcysteine for pulmonary fibrosis. N. Engl. J. Med., 2012, 366, 1968-1977.
64. [64] Luzina, I.G., Tsymbalyuk, N., Choi, J., Hasday, J.D., Atamas, S.P. CCL18-stimulated upregulation of collagen production in lung fibroblasts requires Sp1 signaling and basal Smad3 activity. J. Cell. Physiol., 2006, 206, 221-228.
65. [65] Prasse, A., Pechkovsky, D.V., Toews, G.B., Jungraithmayr, W., Kollert, F., Goldmann, T., Vollmer, E., Muller-Quernheim, J., Zissel, G. A vicious circle of alveolar macrophages and fibroblasts perpetuates pulmonary fibrosis via CCL18. Am. J. Respir. Crit. Care Med., 2006, 173, 781-792.
66. [66] Prasse, A., Probst, C., Bargagli, E., Zissel, G., Toews, G.B., Flaherty, K.R., Olschewski, M., Rottoli, P., Muller-Quernheim, J. Serum CC-chemokine ligand 18 concentration predicts outcome in idiopathic pulmonary fibrosis. Am. J. Respir. Crit. Care Med., 2009, 179, 717-723.
67. [67] Richards, T.J., Kaminski, N., Baribaud, F., Flavin, S., Brodmerkel, C., Horowitz, D., Li, K., Choi, J., Vuga, L.J., Lindell, K.O., Klesen, M., Zhang, Y., Gibson, K.F. Peripheral blood proteins predict mortality in idiopathic pulmonary fibrosis. Am. J. Respir. Crit. Care Med., 2012, 185, 67-76.
68. [68] O'Dwyer, D.N., Armstrong, M.E., Kooblall, M., Donnelly, S.C. Targeting defective Toll-like receptor-3 function and idiopathic pulmonary fibrosis. Expert Opin. Ther. Targets, 2014, 19(4), 507-14
69. [69] Fang, F., Ooka, K., Sun, X., Shah, R., Bhattacharyya, S., Wei, J., Varga, J. A synthetic TLR3 ligand mitigates profibrotic fibroblast responses by inducing autocrine IFN signaling. J. Immunol., 2013, 191, 2956-2966.
70. [70] O'Dwyer, D.N., Armstrong, M.E., Trujillo, G., Cooke, G., Keane, M.P., Fallon, P.G., Simpson, A.J., Millar, A.B., McGrath, E.E., Whyte, M.K., Hirani, N., Hogaboam, C.M., Donnelly, S.C. The Toll-like receptor 3 L412F polymorphism and disease progression in idiopathic pulmonary fibrosis. Am. J. Respir. Crit. Care Med., 2013, 188, 1442-1450.
71. [71] Konishi, K., Gibson, K.F., Lindell, K.O., Richards, T.J., Zhang, Y., Dhir, R., Bisceglia, M., Gilbert, S., Yousem, S.A., Song, J.W., Kim, D.S., Kaminski, N. Gene expression profiles of acute exacerbations of idiopathic pulmonary fibrosis. Am. J. Respir. Crit. Care Med., 2009, 180, 167-175.
72. [72] Yang, I.V., Luna, L.G., Cotter, J., Talbert, J., Leach, S.M., Kidd, R., Turner, J., Kummer, N., Kervitsky, D., Brown, K.K., Boon, K., Schwarz, M.I., Schwartz, D.A., Steele, M.P. The peripheral blood transcriptome identifies the presence and extent of disease in idiopathic pulmonary fibrosis. PloS one, 2012, 7, e37708.
73. [73] Noth, I., Zhang, Y., Ma, S.F., Flores, C., Barber, M., Huang, Y., Broderick, S.M., Wade, M.S., Hysi, P., Scuirba, J., Richards, T.J., Juan-Guardela, B.M., Vij, R., Han, M.K., Martinez, F.J., Kossen, K., Seiwert, S.D., Christie, J.D., Nicolae, D., Kaminski, N., Garcia, J.G. Genetic variants associated with idiopathic pulmonary fibrosis susceptibility and mortality, a genome-wide association study. lancet Respir. Med., 2013, 1, 309-317.
74. [74] Habibzay, M., Saldana, J.I., Goulding, J., Lloyd, C.M., Hussell, T. Altered regulation of Toll-like receptor responses impairs antibacterial immunity in the allergic lung. Mucosal. immunol., 2012, 5, 524-534.
75. [75] Ritter, M., Mennerich, D., Weith, A., Seither, P. Characterization of Toll-like receptors in primary lung epithelial cells, strong impact of the TLR3 ligand poly(I,C) on the regulation of Toll-like receptors., adaptor proteins and inflammatory response. J. Inflamm., 2005, 2, 16.
76. [76] Shah, J.A., Vary, J.C., Chau, T.T., Bang, N.D., Yen, N.T., Farrar, J.J., Dunstan, S.J., Hawn, T.R. Human TOLLIP regulates TLR2 and TLR4 signaling and its polymorphisms are associated with susceptibility to tuberculosis. J. Immunol., 2012, 189, 1737-1746.
77. [77] Schimming, T.T., Parwez, Q., Petrasch-Parwez, E., Nothnagel, M., Epplen, J.T., Hoffjan, S. Association of toll-interacting protein gene polymorphisms with atopic dermatitis. BMC Dermatol., 2007, 7, 3.
78. [78] Hodgson, U., Pulkkinen, V., Dixon, M., Peyrard-Janvid, M., Rehn, M., Lahermo, P., Ollikainen, V., Salmenkivi, K., Kinnula, V., Kere, J., Tukiainen, P., Laitinen, T. ELMOD2 is a candidate gene for familial idiopathic pulmonary fibrosis. Am. J. Human Genet., 2006, 79, 149-154.
79. [79] Pulkkinen, V., Bruce, S., Rintahaka, J., Hodgson, U., Laitinen, T., Alenius, H., Kinnula, V.L., Myllarniemi, M., Matikainen, S., Kere, J. ELMOD2., a candidate gene for idiopathic pulmonary fibrosis., regulates antiviral responses. FASEB J.,2010, 24, 1167-1177.
80. [80] Feghali-Bostwick, C.A., Tsai, C.G., Valentine, V.G., Kantrow, S., Stoner, M.W., Pilewski, J.M., Gadgil, A., George, M.P., Gibson, K.F., Choi, A.M., Kaminski, N., Zhang, Y., Duncan, S.R. Cellular and humoral autoreactivity in idiopathic pulmonary fibrosis. J. Immunol., 2007, 179, 2592-2599.
81. [81] Gilani, S.R., Vuga, L.J., Lindell, K.O., Gibson, K.F., Xue, J., Kaminski, N., Valentine, V.G., Lindsay, E.K., George, M.P., Steele, C., Duncan, S.R. CD28 down-regulation on circulating CD4 Tcells is associated with poor prognoses of patients with idiopathic pulmonary fibrosis. PloS one, 2010, 5, e8959.
82. Herazo-Maya, J.D., Noth, I., Duncan, S.R., Kim, S., Ma, S.F., Tseng, G.C., Feingold, E., Juan-Guardela, B.M., Richards, T.J., Lussier, Y., Huang, Y., Vij, R., Lindell, K.O., Xue, J., Gibson, K.F., Shapiro, S.D., Garcia, J.G., Kaminski, N. Peripheral blood mononuclear cell gene expression profiles predict poor outcome in idiopathic pulmonary fibrosis. Sci. Transl. Med., 2013, 5, 205ra136.
83. [83] Kotsianidis, I., Nakou, E., Bouchliou, I., Tzouvelekis, A., Spanoudakis, E., Steiropoulos, P., Sotiriou, I., Aidinis, V., Margaritis, D., Tsatalas, C., Bouros, D. Global impairment of CD4+CD25+FOXP3+ regulatory T cells in idiopathic pulmonary fibrosis. Am. J. Respir. Crit. Care Med., 2009, 179, 1121-1130.
84. [84] Reilkoff, R.A., Peng, H., Murray, L.A., Peng, X., Russell, T., Montgomery, R., Feghali-Bostwick, C., Shaw, A., Homer, R.J., Gulati, M., Mathur, A., Elias, J.A., Herzog, E.L. Semaphorin 7a+ regulatory T cells are associated with progressive idiopathic pulmonary fibrosis and are implicated in transforming growth factorbeta1-induced pulmonary fibrosis. Am. J. Respir. Crit. Care Med., 2013, 187, 180-188.
85. [85] Tzouvelekis, A., Bouros, D. COPD and IPF, it's all about regulation and balance. Thorax, 2014, 69, 490.
86. [86] Hou, J., Sun, Y., Hao, Y., Zhuo, J., Liu, X., Bai, P., Han, J., Zheng, X., Zeng, H. Imbalance between subpopulations of regulatory T cells in COPD. Thorax, 2013, 68, 1131-1139.
87. [87] Vuga, L.J., Tedrow, J.R., Pandit, K.V., Tan, J., Kass, D.J., Xue, J., Chandra, D., Leader, J.K., Gibson, K.F., Kaminski, N., Sciurba, F.C., Duncan, S.R. C-X-C motif chemokine 13 (CXCL13) is a prognostic biomarker of idiopathic pulmonary fibrosis. Am. J. Respir. Crit. Care Med., 2014, 189, 966-974.
88. [88] Kahloon, R.A., Xue, J., Bhargava, A., Csizmadia, E., Otterbein, L., Kass, D.J., Bon, J., Soejima, M., Levesque, M.C., Lindell, K.O., Gibson, K.F., Kaminski, N., Banga, G., Oddis, C.V., Pilewski, J.M., Sciurba, F.C., Donahoe, M., Zhang, Y., Duncan, S.R. Patients with idiopathic pulmonary fibrosis with antibodies to heat shock protein 70 have poor prognoses. Am. J. Respir. Crit. Care Med., 2013, 187, 768-775.
89. [89] Alford, K.A., Glennie, S., Turrell, B.R., Rawlinson, L., Saklatvala, J., Dean, J.L. Heat shock protein 27 functions in inflammatory gene expression and transforming growth factor-beta-activated kinase-1 (TAK1)-mediated signaling. J. Biol. Chem., 2007, 282, 6232-6241.
90. [90] Taille, C., Grootenboer-Mignot, S., Boursier, C., Michel, L., Debray, M.P., Fagart, J., Barrientos, L., Mailleux, A., Cigna, N., Tubach, F., Marchal-Somme, J., Soler, P., Chollet-Martin, S., Crestani, B. Identification of periplakin as a new target for autoreactivity in idiopathic pulmonary fibrosis. Am. J. Respir. Crit. Care Med., 2011, 183, 759-766.
91. [91] Pardo, A., Selman, M. Matrix metalloproteases in aberrant fibrotic tissue remodeling. Proc. Am. Thorac. Soc., 2006, 3, 383-388.
92. [92] Rosas, I.O., Richards, T.J., Konishi, K., Zhang, Y., Gibson, K., Lokshin, A.E., Lindell, K.O., Cisneros, J., Macdonald, S.D., Pardo, A., Sciurba, F., Dauber, J., Selman, M., Gochuico, B.R., Kaminski, N. MMP1 and MMP7 as potential peripheral blood biomarkers in idiopathic pulmonary fibrosis. PLoS Med., 2008, 5, e93.
93. [93] Zuo, F., Kaminski, N., Eugui, E., Allard, J., Yakhini, Z., Ben-Dor, A., Lollini, L., Morris, D., Kim, Y., DeLustro, B., Sheppard, D., Pardo, A., Selman, M., Heller, R.A. Gene expression analysis reveals matrilysin as a key regulator of pulmonary fibrosis in mice and humans. Proc. Natl. Acad. Sci. U. S. A., 2002, 99, 6292-6297.
94. [94] Pardo, A., Gibson, K., Cisneros, J., Richards, T.J., Yang, Y., Becerril, C., Yousem, S., Herrera, I., Ruiz, V., Selman, M., Kaminski, N. Up-regulation and profibrotic role of osteopontin in human idiopathic pulmonary fibrosis. PLoS Med., 2005, 2, e251.
95. [95] Richards, T.J., Park, C., Chen, Y., Gibson, K.F., Peter Di, Y., Pardo, A., Watkins, S.C., Choi, A.M., Selman, M., Pilewski, J., Kaminski, N., Zhang, Y. Allele-specific transactivation of matrix metalloproteinase 7 by FOXA2 and correlation with plasma levels in idiopathic pulmonary fibrosis. Am. J. Physiol. Lung Cell. Mol. Physiol., 2012, 302, L746-754.
96. [96] Veidal, S.S., Bay-Jensen, A.C., Tougas, G., Karsdal, M.A., Vainer, B. Serum markers of liver fibrosis, combining the BIPED classification and the neo-epitope approach in the development of new biomarkers. Dis. Markers, 2010, 28, 15-28.
97. [97] R Gisli Jenkins JKS., Gauri Saini., Jane H Bentley., Anne-Marie Russell., Rebecca Braybrooke., Philip L Molyneaux., Tricia M McKeever, Athol U Wells, Aiden Flynn, Richard B Hubbard, Diana J Leeming, Richard P Marshall, Morten A Karsdal, Pauline T Lukey, Toby M Maher. Relating longitudinal change in collagen degradation biomarkers to outcome in idiopathic pulmonary fibrosis, an analysis from the prospective., multi-centre PROFILE study. lancet Respir. Med., 2015, 3.
98. [98] Barry-Hamilton, V., Spangler, R., Marshall, D., McCauley, S., Rodriguez, H.M., Oyasu, M., Mikels, A., Vaysberg, M., Ghermazien, H., Wai, C., Garcia, C.A., Velayo, A.C., Jorgensen, B., Biermann, D., Tsai, D., Green, J., Zaffryar-Eilot, S., Holzer, A., Ogg, S., Thai, D., Neufeld, G., Van Vlasselaer, P., Smith, V. Allosteric inhibition of lysyl oxidase-like-2 impedes the development of a pathologic microenvironment. Nat. Med., 2010, 16, 1009-1017.
99. [99] Chen, L.J., Li, W.D., Li, S.F., Su, X.W., Lin, G.Y., Huang, Y.J., Yan, G.M. Bleomycin induces upregulation of lysyl oxidase in cultured human fetal lung fibroblasts. Acta Pharmacol. Sin., 2010, 31, 554-559.
100. [100] At the frontiers of lung fibrosis therapy. Nat. Biotechnol., 2013, 31, 781-783.
101. [101] Ovet, H., Oztay, F. The copper chelator tetrathiomolybdate regressed bleomycin-induced pulmonary fibrosis in mice., by reducing lysyl oxidase expressions. Biol. Trace Elem. Res., 2014, 162, 189-199.
102. [102] Chien, J.W., Richards, T.J., Gibson, K.F., Zhang, Y., Lindell, K.O., Shao, L., Lyman, S.K., Adamkewicz, J.I., Smith, V., Kaminski, N., O'Riordan, T. Serum lysyl oxidase-like 2 levels and idiopathic pulmonary fibrosis disease progression. Euro. Respir. J., 2014, 43, 1430-1438.
103. [103] Moeller, A., Gilpin, S.E., Ask, K., Cox, G., Cook, D., Gauldie, J., Margetts, P.J., Farkas, L., Dobranowski, J., Boylan, C., O'Byrne, P.M., Strieter, R.M., Kolb, M. Circulating fibrocytes are an indicator of poor prognosis in idiopathic pulmonary fibrosis. Am. J. Respir. Crit. Care Med.,2009, 179, 588-594.
104. Tzouvelekis, A., Kaminski, N. Epigenetics in idiopathic pulmonary fibrosis. Biochem. Cell Biol., 2015, 93(2), 159-70 1-12.
105. [105] Rabinovich, E.I., Kapetanaki, M.G., Steinfeld, I., Gibson, K.F., Pandit, K.V., Yu, G., Yakhini, Z., Kaminski, N. Global methylation patterns in idiopathic pulmonary fibrosis. PloS one, 2012, 7, e33770.
106. [106] Yang, I.V., Pedersen, B.S., Rabinovich, E., Hennessy, C.E., Davidson, E.J., Murphy, E., Guardela, B.J., Tedrow, J.R., Zhang, Y., Singh, M.K., Correll, M., Schwarz, M.I., Geraci, M., Sciurba, F.C., Quackenbush, J., Spira, A., Kaminski, N., Schwartz, D.A. Relationship of DNA methylation and gene expression in idiopathic pulmonary fibrosis. Am. J. Respir. Crit. Care Med.,2014, 190, 1263-1272.
107. [107] Milosevic, J., Pandit, K., Magister, M., Rabinovich, E., Ellwanger, D.C., Yu, G., Vuga, L.J., Weksler, B., Benos, P.V., Gibson, K.F., McMillan, M., Kahn, M., Kaminski N. Profibrotic role of miR-154 in pulmonary fibrosis. Am. J. Respir. Cell Mol. Biol., 2012, 47, 879-887.
108. [108] Huleihel, L., Ben-Yehudah, A., Milosevic, J., Yu, G., Pandit, K., Sakamoto, K., Yousef, H., LeJeune, M., Coon, T.A., Redinger, C.J., Chensny, L., Manor, E., Schatten, G., Kaminski, N. Let-7d microRNA affects mesenchymal phenotypic properties of lung fibroblasts. Am. J. Physiol. Lung Cell. Mol. Physiol., 2014, 306, L534-542.
109. [109] Pandit, K.V., Corcoran, D., Yousef, H., Yarlagadda, M., Tzouvelekis, A., Gibson, K.F., Konishi, K., Yousem, S.A., Singh, M., Handley, D., Richards, T., Selman, M., Watkins, S.C., Pardo, A., Ben-Yehudah, A., Bouros, D., Eickelberg, O., Ray, P., Benos, P.V., Kaminski, N. Inhibition and role of let-7d in idiopathic pulmonary fibrosis. Am. J. Respir. Crit. Care Med.,2010, 182, 220-229.
110. [110] Montgomery, R.L., Yu, G., Latimer, P.A., Stack, C., Robinson, K., Dalby, C.M., Kaminski, N., van Rooij, E. MicroRNA mimicry blocks pulmonary fibrosis. EMBO Mol. Med., 2014, 6, 1347-1356.
111. [111] Parker, M.W., Rossi, D., Peterson, M., Smith, K., Sikstrom, K., White, E.S., Connett, J.E., Henke, C.A., Larsson, O., Bitterman, P.B. Fibrotic extracellular matrix activates a profibrotic positive feedback loop. J. Clini. Invest., 2014, 124, 1622-1635.
112. [112] Xiao, J., Meng, X.M., Huang, X.R., Chung, A.C., Feng, Y.L., Hui, D.S., Yu, C.M., Sung, J.J., Lan, H.Y. miR-29 inhibits bleomycininduced pulmonary fibrosis in mice. Mol. Ther., J. Am. Soc. Gene Ther., 2012, 20, 1251-1260.
113. [113] Cushing, L., Kuang, P.P., Qian, J., Shao, F., Wu, J., Little, F., Thannickal, V.J., Cardoso, W.V., Lu, J. miR-29 is a major regulator of genes associated with pulmonary fibrosis. Am. J. Respir. Cell Mol. Biol., 2011, 45, 287-294.
114. [114] Liu, G., Friggeri, A., Yang, Y., Milosevic, J., Ding, Q., Thannickal, V.J., Kaminski, N., Abraham, E. miR-21 mediates fibrogenic activation of pulmonary fibroblasts and lung fibrosis. J. Exp. Med., 2010, 207, 1589-1597.
115. [115] Oak, S.R., Murray, L., Herath, A., Sleeman, M., Anderson, I., Joshi, A.D., Coelho, A.L., Flaherty, K.R., Toews, G.B., Knight, D., Martinez, F.J., Hogaboam, C.M. A micro RNA processing defect in rapidly progressing idiopathic pulmonary fibrosis. PloS one, 2011, 6, e21253.
116. [116] Ellson, C.D., Dunmore, R., Hogaboam, C.M., Sleeman, M.A., Murray, L.A. Danger-associated molecular patterns and danger signals in idiopathic pulmonary fibrosis. Am. J. Respir. Cell Mol. Biol., 2014, 51, 163-168.
117. [117] Molyneaux, P.L., Maher, T.M. The role of infection in the pathogenesis of idiopathic pulmonary fibrosis. Eur. Respir. Rev., 2013, 22, 376-381.
118. [118] Park, H., Shin, J.W., Park, S.G., Kim, W. Microbial communities in the upper respiratory tract of patients with asthma and chronic obstructive pulmonary disease. PloS one, 2014, 9, e109710.
119. [119] Surette MG. The cystic fibrosis lung microbiome. Ann. Am. Thoracic Soc., 2014, 11 Suppl 1, S61-65.
120. [120] Han, M.K., Zhou, Y., Murray, S., Tayob, N., Noth, I., Lama, V.N., Moore, B.B., White, E.S., Flaherty, K.R., Huffnagle, G.B., Martinez, F.J., Investigators C. Lung microbiome and disease progression in idiopathic pulmonary fibrosis, an analysis of the COMET study. lancet Respir. Med., 2014, 2, 548-556.
121. [121] Molyneaux, P.L., Cox, M.J., Willis-Owen, S.A., Mallia, P., Russell, K.E., Russell, A.M., Murphy, E., Johnston, S.L., Schwartz, D.A., Wells, A.U., Cookson, W.O., Maher, T.M., Moffatt, M.F. The role of bacteria in the pathogenesis and progression of idiopathic pulmonary fibrosis. Am. J. Respir. Crit. Care Med.,2014, 190, 906-913.