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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).

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Bini, L. & Pallini, V. (2005). Cytokine profile and proteome analysis in bronchoalveolar lavage of patients with sarcoidosis, pulmonary fibrosis associated with systemic sclerosis and idiopathic pulmonary fibrosis. Proteomics. 5 (5). pp. 1423–1430. Rush, B., Wiskar, K., Berger, L. & Griesdale, D. (2016). The use of mechanical ventilation in patients with idiopathic pulmonary fibrosis in the United States: A nationwide retrospective cohort analysis. Respiratory Medicine. [Online]. 111. pp. 72–76. Available from: http://linkinghub.elsevier.com/retrieve/pii/S0954611115300962. Russell, A.-M., Ripamonti, E. & Vancheri, C. (2016). Qualitative European survey of patients with idiopathic pulmonary fibrosis: patients’ perspectives of the disease and treatment. BMC Pulmonary Medicine. [Online]. 16 (1). pp. 10. Available from: http://www.biomedcentral.com/1471-2466/16/10. Sagara, H., Okada, T., Okumura, K., Ogawa, H., Ra, C., Fukuda, T. & Nakao, A. (2002). Activation of TGF-beta/Smad2 signaling is associated with airway remodeling in asthma. The Journal of allergy and clinical immunology. 110 (2). pp. 249–254. Saito, A., Okazaki, H., Sugawara, I., Yamamoto, K. & Takizawa, H. (2003). Potential action of IL-4 and IL-13 as fibrogenic factors on lung fibroblasts in vitro. International archives of allergy and immunology. 132 (2). pp. 168–176. Sakamoto, H., Zhao, L.-H., Jain, F. & Kradin, R. (2002). IL-12p40(-/-) mice treated with intratracheal bleomycin exhibit decreased pulmonary inflammation and increased fibrosis. Experimental and molecular pathology. 72 (1). pp. 1–9. Santana, A., Saxena, B., Noble, N.A., Gold, L.I. & Marshall, B.C. (1995). Increased expression of transforming growth factor beta isoforms (beta 1, beta 2, beta 3) in bleomycin-induced pulmonary fibrosis. American journal of respiratory cell and molecular biology. 13 (1). pp. 34–44. Savino, W. (2002). The thymus gland is a target in malnutrition. European journal of clinical nutrition. 56 (3). pp. S46–S49. Schelegle, E.S., Mansoor, J.K. & Giri, S. (1997). Pirfenidone attenuates bleomycin-induced changes in pulmonary functions in hamsters. Proceedings of the Society for © 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 158 of 172

Experimental Biology and Medicine. Society for Experimental Biology and Medicine (New York, N.Y.). [Online]. 216 (3). pp. 392–7. Available from: http://www.ncbi.nlm.nih.gov/pubmed/9402144. Schenker, M. (2000). Exposures and health effects from inorganic agricultural dusts. Environmental health perspectives. 108. pp. 661–664. Scheule, R.K., Perkins, R.C., Hamilton, R. & Holian, A. (1992). Bleomycin stimulation of cytokine secretion by the human alveolar macrophage. The American journal of physiology. 262 (4 Pt 1). pp. L386-91. Schwartz, D.A., Helmers, R.A., Galvin, J.R., Van Fossen, D.S., Frees, K.L., Dayton, C.S., Burmeister, L.F. & Hunninghake, G.W. (1994). Determinants of survival in idiopathic pulmonary fibrosis. American journal of respiratory and critical care medicine. [Online]. 149 (2 Pt 1). pp. 450–4. Available from: http://www.ncbi.nlm.nih.gov/pubmed/8306044. Segel, M.J., Izbicki, G., Cohen, P.Y., Or, R., Christensen, T.G., Wallach-Dayan, S.B. & Breuer, R. (2003). Role of interferon-gamma in the evolution of murine bleomycin lung fibrosis. American journal of physiology. Lung cellular and molecular physiology. 285 (6). pp. L1255-62. Selman, M., Carrillo, G., Estrada, A., Mejia, M., Becerril, C., Cisneros, J., Gaxiola, M., Pérez-Padilla, R., Navarro, C., Richards, T., Dauber, J., King, T.E., Pardo, A. & Kaminski, N. (2007). Accelerated Variant of Idiopathic Pulmonary Fibrosis: Clinical Behavior and Gene Expression Pattern M. You (ed.). PLoS ONE. [Online]. 2 (5). pp. e482. Available from: http://dx.plos.org/10.1371/journal.pone.0000482. Selman, M., Lin, H.-M., Montaño, M., Jenkins, A.L., Estrada, A., Lin, Z., Wang, G., DiAngelo, S.L., Guo, X., Umstead, T.M., Lang, C.M., Pardo, A., Phelps, D.S. & Floros, J. (2003). Surfactant protein A and B genetic variants predispose to idiopathic pulmonary fibrosis. Human genetics. [Online]. 113 (6). pp. 542–50. Available from: http://www.ncbi.nlm.nih.gov/pubmed/13680361. Serrano-Mollar, A., Closa, D., Prats, N., Blesa, S., Martinez-Losa, M., Cortijo, J., Estrela, J.M., Morcillo, E.J. & Bulbena, O. (2003). In vivo antioxidant treatment protects against © 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 159 of 172

Bini, L. & Pallini, V. (2005). Cytokine profile and proteome analysis in bronchoalveolar<br />

lavage of patients with sarcoidosis, pulmonary fibrosis associated with systemic<br />

sclerosis and idiopathic pulmonary fibrosis. Proteomics. 5 (5). pp. 1423–1430.<br />

Rush, B., Wiskar, K., Berger, L. & Griesdale, D. (2016). The use of mechanical ventilation in<br />

patients with idiopathic pulmonary fibrosis in the United States: A nationwide<br />

retrospective cohort analysis. Respiratory Medicine. [Online]. 111. pp. 72–76. Available<br />

from: http://linkinghub.elsevier.com/retrieve/pii/S0954611115300962.<br />

Russell, A.-M., Ripamonti, E. & Vancheri, C. (2016). Qualitative European survey of<br />

patients with idiopathic pulmonary fibrosis: patients’ perspectives of the disease and<br />

treatment. BMC Pulmonary Medicine. [Online]. 16 (1). pp. 10. Available from:<br />

http://www.biomedcentral.com/1471-2466/16/10.<br />

Sagara, H., Okada, T., Okumura, K., Ogawa, H., Ra, C., Fukuda, T. & Nakao, A. (2002).<br />

Activation of TGF-beta/Smad2 signaling is associated with airway remodeling in asthma.<br />

The Journal of allergy and clinical immunology. 110 (2). pp. 249–254.<br />

Saito, A., Okazaki, H., Sugawara, I., Yamamoto, K. & Takizawa, H. (2003). Potential action<br />

of IL-4 and IL-13 as fibrogenic factors on lung fibroblasts in vitro. International<br />

archives of allergy and immunology. 132 (2). pp. 168–176.<br />

Sakamoto, H., Zhao, L.-H., Jain, F. & Kradin, R. (2002). IL-12p40(-/-) mice treated with<br />

intratracheal bleomycin exhibit decreased pulmonary inflammation and increased<br />

fibrosis. Experimental and molecular pathology. 72 (1). pp. 1–9.<br />

Santana, A., Saxena, B., Noble, N.A., Gold, L.I. & Marshall, B.C. (1995). Increased<br />

expression of transforming growth factor beta isoforms (beta 1, beta 2, beta 3) in<br />

bleomycin-induced pulmonary fibrosis. American journal of respiratory cell and<br />

molecular biology. 13 (1). pp. 34–44.<br />

Savino, W. (2002). The thymus gland is a target in malnutrition. European journal of clinical<br />

nutrition. 56 (3). pp. S46–S49.<br />

Schelegle, E.S., Mansoor, J.K. & Giri, S. (1997). Pirfenidone attenuates bleomycin-induced<br />

changes in pulmonary functions in hamsters. Proceedings of the Society for<br />

© 2017-2018 All Rights Reserved, No part of this document should be modified/used without prior consent<br />

<strong>Tutors</strong> <strong>India</strong> - Your trusted mentor since 2001<br />

www.tutorindia.com I UK # +44-1143520021, info@tutorsindia.com<br />

Page 158 of 172

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