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Idiopathic Pulmonary Fibrosis (IPF) as the name suggests is a progressive disorder with no known aetiology. It is characterised by the thickening of the alveoli due to scarring resulting in cough. It is known to primarily occur in older adults over 60 years of age. The findings of IPF have a known association of Usual Interstitial Pneumonia (UIP) (Raghu et al., 2011; Kawano-Dourado & Kairalla, 2013; Wells, 2013). It has been deemed that the prognosis is generally poor when UIP has been confirmed (King et al., 2001b). The median survival rate of IPF is 50%, typically around two years after diagnosis (Raghu et al., 2011; King et al., 2001b). Idiopathic Pulmonary Fibrosis (IPF) as the name suggests is a progressive disorder with no known aetiology. It is characterised by the thickening of the alveoli due to scarring resulting in cough. It is known to primarily occur in older adults over 60 years of age. The findings of IPF have a known association of Usual Interstitial Pneumonia (UIP) (Raghu et al., 2011; Kawano-Dourado & Kairalla, 2013; Wells, 2013). It has been deemed that the prognosis is generally poor when UIP has been confirmed (King et al., 2001b). The median survival rate of IPF is 50%, typically around two years after diagnosis (Raghu et al., 2011; King et al., 2001b).
Kim, D.S. (2006). Classification and Natural History of the Idiopathic Interstitial Pneumonias. Proceedings of the American Thoracic Society. [Online]. 3 (4). pp. 285–292. Available from: http://pats.atsjournals.org/cgi/doi/10.1513/pats.200601-005TK. Kinder, B.W., Brown, K.K., McCormack, F.X., Ix, J.H., Kervitsky, A., Schwarz, M.I. & King, T.E. (2009). Serum surfactant protein-A is a strong predictor of early mortality in idiopathic pulmonary fibrosis. Chest. [Online]. 135 (6). pp. 1557–63. Available from: http://www.ncbi.nlm.nih.gov/pubmed/19255294. Kinder, B.W., Brown, K.K., Schwarz, M.I., Ix, J.H., Kervitsky, A. & King, T.E. (2008). Baseline BAL neutrophilia predicts early mortality in idiopathic pulmonary fibrosis. Chest. [Online]. 133 (1). pp. 226–32. Available from: http://www.ncbi.nlm.nih.gov/pubmed/18071016. King, T.E., Albera, C., Bradford, W.Z., Costabel, U., du Bois, R.M., Leff, J.A., Nathan, S.D., Sahn, S.A., Valeyre, D. & Noble, P.W. (2014a). All-cause mortality rate in patients with idiopathic pulmonary fibrosis. Implications for the design and execution of clinical trials. American journal of respiratory and critical care medicine. [Online]. 189 (7). pp. 825– 31. Available from: http://www.ncbi.nlm.nih.gov/pubmed/24476390. King, T.E., Albera, C., Bradford, W.Z., Costabel, U., Hormel, P., Lancaster, L., Noble, P.W., Sahn, S.A., Szwarcberg, J., Thomeer, M., Valeyre, D., du Bois, R.M. & INSPIRE Study Group (2009). Effect of interferon gamma-1b on survival in patients with idiopathic pulmonary fibrosis (INSPIRE): a multicentre, randomised, placebo-controlled trial. Lancet (London, England). [Online]. 374 (9685). pp. 222–8. Available from: http://www.ncbi.nlm.nih.gov/pubmed/19570573. King, T.E., Behr, J., Brown, K.K., du Bois, R.M., Lancaster, L., de Andrade, J.A., Stähler, G., Leconte, I., Roux, S. & Raghu, G. (2008). BUILD-1: a randomized placebo-controlled trial of bosentan in idiopathic pulmonary fibrosis. American journal of respiratory and critical care medicine. [Online]. 177 (1). pp. 75–81. Available from: http://www.ncbi.nlm.nih.gov/pubmed/17901413. King, T.E., Bradford, W.Z., Castro-Bernardini, S., Fagan, E.A., Glaspole, I., Glassberg, M.K., Gorina, E., Hopkins, P.M., Kardatzke, D., Lancaster, L., Lederer, D.J., Nathan, S.D., © 2017-2018 All Rights Reserved, No part of this document should be modified/used without prior consent Tutors India - Your trusted mentor since 2001 www.tutorindia.com I UK # +44-1143520021, info@tutorsindia.com Page 136 of 172
Pereira, C.A., Sahn, S.A., Sussman, R., Swigris, J.J., Noble, P.W. & ASCEND Study Group (2014b). A phase 3 trial of pirfenidone in patients with idiopathic pulmonary fibrosis. The New England journal of medicine. [Online]. 370 (22). pp. 2083–92. Available from: http://www.ncbi.nlm.nih.gov/pubmed/24836312. King, T.E., Schwarz, M.I., Brown, K., Tooze, J.A., Colby, T. V, Waldron, J.A., Flint, A., Thurlbeck, W. & Cherniack, R.M. (2001a). Idiopathic pulmonary fibrosis: relationship between histopathologic features and mortality. American journal of respiratory and critical care medicine. [Online]. 164 (6). pp. 1025–32. Available from: http://www.ncbi.nlm.nih.gov/pubmed/11587991. King, T.E., Tooze, J.A., Schwarz, M.I., Brown, K.R. & Cherniack, R.M. (2001b). Predicting survival in idiopathic pulmonary fibrosis: scoring system and survival model. American journal of respiratory and critical care medicine. [Online]. 164 (7). pp. 1171–81. Available from: http://www.ncbi.nlm.nih.gov/pubmed/11673205. Kline, J.N., Schwartz, D.A., Monick, M.M., Floerchinger, C.S. & Hunninghake, G.W. (1993). Relative release of interleukin-1 beta and interleukin-1 receptor antagonist by alveolar macrophages. A study in asbestos-induced lung disease, sarcoidosis, and idiopathic pulmonary fibrosis. Chest. 104 (1). pp. 47–53. ten Klooster, L., van Moorsel, C.H.M., Kwakkel-van Erp, J.M., van Velzen-Blad, H. & Grutters, J.C. (2015). Immunoglobulin A in serum: an old acquaintance as a new prognostic biomarker in idiopathic pulmonary fibrosis. Clinical & Experimental Immunology. [Online]. 181 (2). pp. 357–361. Available from: http://doi.wiley.com/10.1111/cei.12636. Kolb, M., Jenkins, G. & Richeldi, L. (2016). Study the past to divine the future. Confucius’ wisdom doesn’t work for idiopathic pulmonary fibrosis. Thorax. [Online]. 71 (5). pp. 399–400. Available from: http://thorax.bmj.com/lookup/doi/10.1136/thoraxjnl-2016- 208670. Kolodsick, J.E., Toews, G.B., Jakubzick, C., Hogaboam, C., Moore, T.A., McKenzie, A., Wilke, C.A., Chrisman, C.J. & Moore, B.B. (2004). Protection from fluorescein isothiocyanate-induced fibrosis in IL-13-deficient, but not IL-4-deficient, mice results © 2017-2018 All Rights Reserved, No part of this document should be modified/used without prior consent Tutors India - Your trusted mentor since 2001 www.tutorindia.com I UK # +44-1143520021, info@tutorsindia.com Page 137 of 172
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Pereira, C.A., Sahn, S.A., Sussman, R., Swigris, J.J., Noble, P.W. & ASCEND Study<br />
Group (2014b). A phase 3 trial of pirfenidone in patients with idiopathic pulmonary<br />
fibrosis. The New England journal of medicine. [Online]. 370 (22). pp. 2083–92.<br />
Available from: http://www.ncbi.nlm.nih.gov/pubmed/24836312.<br />
King, T.E., Schwarz, M.I., Brown, K., Tooze, J.A., Colby, T. V, Waldron, J.A., Flint, A.,<br />
Thurlbeck, W. & Cherniack, R.M. (2001a). Idiopathic pulmonary fibrosis: relationship<br />
between histopathologic features and mortality. American journal of respiratory and<br />
critical care medicine. [Online]. 164 (6). pp. 1025–32. Available from:<br />
http://www.ncbi.nlm.nih.gov/pubmed/11587991.<br />
King, T.E., Tooze, J.A., Schwarz, M.I., Brown, K.R. & Cherniack, R.M. (2001b). Predicting<br />
survival in idiopathic pulmonary fibrosis: scoring system and survival model. American<br />
journal of respiratory and critical care medicine. [Online]. 164 (7). pp. 1171–81.<br />
Available from: http://www.ncbi.nlm.nih.gov/pubmed/11673205.<br />
Kline, J.N., Schwartz, D.A., Monick, M.M., Floerchinger, C.S. & Hunninghake, G.W. (1993).<br />
Relative release of interleukin-1 beta and interleukin-1 receptor antagonist by alveolar<br />
macrophages. A study in asbestos-induced lung disease, sarcoidosis, and idiopathic<br />
pulmonary fibrosis. Chest. 104 (1). pp. 47–53.<br />
ten Klooster, L., van Moorsel, C.H.M., Kwakkel-van Erp, J.M., van Velzen-Blad, H. &<br />
Grutters, J.C. (2015). Immunoglobulin A in serum: an old acquaintance as a new<br />
prognostic biomarker in idiopathic pulmonary fibrosis. Clinical & Experimental<br />
Immunology. [Online]. 181 (2). pp. 357–361. Available from:<br />
http://doi.wiley.com/10.1111/cei.12636.<br />
Kolb, M., Jenkins, G. & Richeldi, L. (2016). Study the past to divine the future. Confucius’<br />
wisdom doesn’t work for idiopathic pulmonary fibrosis. Thorax. [Online]. 71 (5). pp.<br />
399–400. Available from: http://thorax.bmj.com/lookup/doi/10.1136/thoraxjnl-2016-<br />
208670.<br />
Kolodsick, J.E., Toews, G.B., Jakubzick, C., Hogaboam, C., Moore, T.A., McKenzie, A.,<br />
Wilke, C.A., Chrisman, C.J. & Moore, B.B. (2004). Protection from fluorescein<br />
isothiocyanate-induced fibrosis in IL-13-deficient, but not IL-4-deficient, mice results<br />
© 2017-2018 All Rights Reserved, No part of this document should be modified/used without prior consent<br />
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