References

Natural History of IPF and Potential Risk Factors

  1. 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.
  2. Kim DS, Collard HR, King TE, Jr. Classification and natural history of the idiopathic interstitial pneumonias. Proc Am Thorac Soc. 2006;3:285-292.
  3. 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;188:733-748.
  4. King TE, Jr., Pardo A, Selman M. Idiopathic pulmonary fibrosis. Lancet 2011;378:1949-1961.
  5. Flaherty KR, Toews GB, Travis WD, et al. Clinical significance of histological classification of idiopathic interstitial pneumonia. Eur Respir J. 2002;19:275-283.
  6. Nicholson AG, Colby TV, du Bois RM, Hansell DM, Wells AU. 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;162:2213-2217.
  7. Rudd RM, Prescott RJ, Chalmers JC, Johnston ID. British Thoracic Society Study on cryptogenic fibrosing alveolitis: Response to treatment and survival. Thorax. 2007;62:62-66.
  8. Bjoraker JA, Ryu JH, Edwin MK, et al. Prognostic significance of histopathologic subsets in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 1998;157:199-203.
  9. King TE, Jr., Tooze JA, Schwarz MI, Brown KR, Cherniack RM. Predicting survival in idiopathic pulmonary fibrosis: scoring system and survival model. Am J Respir Crit Care Med. 2001;164:1171-1181.
  10. Jegal Y, Kim DS, Shim TS, et al. Physiology is a stronger predictor of survival than pathology in fibrotic interstitial pneumonia. Am J Respir Crit Care Med. 2005;171:639-644.
  11. Nagai S, Kitaichi M, Hamada K, et al. Hospital-based historical cohort study of 234 histologically proven Japanese patients with IPF. Sarcoidosis Vasc Diffuse Lung Dis. 1999;16:209-214.
  12. American Cancer Society. Cancer Facts and Figures 2010. Atlanta: American Cancer Society, 2010.
  13. Benza RL, Miller DP, Barst RJ, Badesch DB, Frost AE, McGoon MD. An evaluation of long-term survival from time of diagnosis in pulmonary arterial hypertension from the REVEAL Registry. Chest. 2012;142:448-456.
  14. Olson AL, Swigris JJ, Lezotte DC, Norris JM, Wilson CG, Brown KK. Mortality from pulmonary fibrosis increased in the United States from 1992 to 2003. Am J Respir Crit Care Med. 2007;176:277-284.
  15. Ley B, Collard HR, King TE, Jr. Clinical course and prediction of survival in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 2011;183:431-440.
  16. 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;171:1040-1047.
  17. Daniels CE, Lasky JA, Limper AH, Mieras K, Gabor E, Schroeder DR. Imatinib treatment for idiopathic pulmonary fibrosis: Randomized placebo-controlled trial results. Am J Respir Crit Care Med. 2010;181:604-610.
  18. Demedts M, Behr J, Buhl R, et al. High-dose acetylcysteine in idiopathic pulmonary fibrosis. N Engl J Med. 2005;353:2229-2242.
  19. Raghu G, Brown KK, Bradford WZ, et al. A placebo-controlled trial of interferon gamma-1b in patients with idiopathic pulmonary fibrosis. N Engl J Med. 2004;350:125-133.
  20. Raghu G, Brown KK, Costabel U, et al. Treatment of idiopathic pulmonary fibrosis with etanercept: an exploratory, placebo-controlled trial. Am J Respir Crit Care Med. 2008;178:948-955.
  21. Taniguchi H, Ebina M, Kondoh Y, et al. Pirfenidone in idiopathic pulmonary fibrosis. Eur Respir J. 2010;35:821-829.
  22. Selman M, Carrillo G, Estrada A, et al. Accelerated variant of idiopathic pulmonary fibrosis: clinical behavior and gene expression pattern. PLoS One. 2007;2(5):e482.
  23. Collard HR, Moore BB, Flaherty KR, et al. Acute exacerbations of idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 2007;176:636-643.
  24. Kim DS. Acute exacerbation of idiopathic pulmonary fibrosis. Clin Chest Med. 2012;33:59-68.
  25. Wootton SC, Kim DS, Kondoh Y, et al. Viral infection in acute exacerbation of idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 2011;183:1698-1702.
  26. Collard HR, Calfee CS, Wolters PJ, et al. Plasma biomarker profiles in acute exacerbation of idiopathic pulmonary fibrosis. Am J Physiol Lung Cell Mol Physiol. 2010;299:L3-L7.
  27. Song JW, Hong SB, Lim CM, Koh Y, Kim DS. Acute exacerbation of idiopathic pulmonary fibrosis: incidence, risk factors and outcome. Eur Respir J. 2011;37:356-363.
  28. Kondoh Y, Taniguchi H, Katsuta T, et al. Risk factors of acute exacerbation of idiopathic pulmonary fibrosis. Sarcoidosis Vasc Diffuse Lung Dis. 2010;27:103-110.
  29. Raghu G, Weycker D, Edelsberg J, et al. Incidence and prevalence of idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 2006;174:810-816.
  30. Baumgartner KB, Samet JM, Stidley CA, et al. Cigarette smoking: a risk factor for idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 1997;155:242-248.
  31. Steele MP, Speer MC, Loyd JE, et al. Clinical and pathologic features of familial interstitial pneumonia. Am J Respir Crit Care Med. 2005;172:1146-1152.
  32. Taskar VS, Coultas DB. Is idiopathic pulmonary fibrosis an environmental disease? Proc Am Thorac Soc. 2006;3:293-298.
  33. Baumgartner KB, Samet JM, Coultas DB, et al. Occupational and environmental risk factors for idiopathic pulmonary fibrosis: a multicenter case-control study. Collaborating Centers. Am J Epidemiol. 2000;152:307-315.
  34. Hubbard R, Cooper M, Antoniak M, et al. Risk of cryptogenic fibrosing alveolitis in metal workers. Lancet. 2000;355:466-467.
  35. Gustafson T, Dahlman-Hoglund A, Nilsson K, Strom K, Tornling G, Toren K. Occupational exposure and severe pulmonary fibrosis. Respir Med. 2007;101:2207-2212.
  36. Pinheiro GA, Antao VC, Wood JM, Wassell JT. Occupational risks for idiopathic pulmonary fibrosis mortality in the United States. Int J Occup Environ Health. 2008;14:117-123.
  37. Hubbard R, Lewis S, Richards K, Johnston I, Britton J. Occupational exposure to metal or wood dust and aetiology of cryptogenic fibrosing alveolitis. Lancet. 1996;347:284-289.
  38. Olson AL, Swigris JJ. Idiopathic pulmonary fibrosis: diagnosis and epidemiology. Clin Chest Med. 2012;33:41-50.
  39. Scott J, Johnston I, Britton J. What causes cryptogenic fibrosing alveolitis? A case-control study of environmental exposure to dust. BMJ. 1990;301:1015-1017.
  40. Tang YW, Johnson JE, Browning PJ, et al. Herpesvirus DNA is consistently detected in lungs of patients with idiopathic pulmonary fibrosis. J Clin Microbiol. 2003;41:2633-2640.
  41. Arase Y, Suzuki F, Suzuki Y et al. Hepatitis C virus enhances incidence of idiopathic pulmonary fibrosis. World J Gastroenterol. 2008;14:5880-5886.
  42. Raghu G, Freudenberger TD, Yang S et al. High prevalence of abnormal acid gastro-oesophageal reflux in idiopathic pulmonary fibrosis. Eur Respir J. 2006;27:136-142.
  43. el-Serag HB, Sonnenberg A. Comorbid occurrence of laryngeal or pulmonary disease with esophagitis in United States military veterans. Gastroenterology. 1997;113:755-760.
  44. Tobin RW, Pope CE, Pellegrini CA, Emond MJ, Sillery J, Raghu G. Increased prevalence of gastroesophageal reflux in patients with idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 1998;158:1804-1808.
  45. Lee JS, Collard HR, Raghu G, et al. Does chronic microaspiration cause idiopathic pulmonary fibrosis? Am J Med. 2010;123:304-311.
  46. Garcia-Sancho C, Buendia-Roldan I, Fernandez-Plata MR, et al. Familial pulmonary fibrosis is the strongest risk factor for idiopathic pulmonary fibrosis. Respir Med. 2011;105:1902-1907.
  47. Hodgson U, Laitinen T, Tukiainen P. Nationwide prevalence of sporadic and familial idiopathic pulmonary fibrosis: evidence of founder effect among multiplex families in Finland. Thorax. 2002;57:338-342.
  48. Marshall RP, Puddicombe A, Cookson WO, Laurent GJ. Adult familial cryptogenic fibrosing alveolitis in the United Kingdom. Thorax. 2000;55:143-146.
  49. Fernandez BA, Fox G, Bhatia R, et al. A Newfoundland cohort of familial and sporadic idiopathic pulmonary fibrosis patients: clinical and genetic features. Respir Res. 2012;13:64.
  50. Loyd JE. Pulmonary fibrosis in families. Am J Respir Cell Mol Biol. 2003;29:S47-S50.
  51. 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;165:1322-1328.
  52. van Moorsel CH, van Oosterhout MF, Barlo NP, et al. Surfactant protein C mutations are the basis of a significant portion of adult familial pulmonary fibrosis in a dutch cohort. Am J Respir Crit Care Med. 2010;182:1419-1425.
  53. 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;84:52-59.
  54. Armanios MY, Chen JJ, Cogan JD, et al. Telomerase mutations in families with idiopathic pulmonary fibrosis. N Engl J Med. 2007;356:1317-1326.
  55. Kropski JA, Mitchell DB, Markin C, et al. A novel dyskerin (DKC1) mutation is associated with Familial Interstitial Pneumonia. Chest. 2014.
  56. 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;104:7552-7557.
  57. Seibold MA, Wise AL, Speer MC, et al. A common MUC5B promoter polymorphism and pulmonary fibrosis. N Engl J Med. 2011;364:1503-1512.
  58. Zhang Y, Noth I, Garcia JG, Kaminski N. A variant in the promoter of MUC5B and idiopathic pulmonary fibrosis. N Engl J Med. 2011;364:1576-1577.
  59. Fingerlin TE, Murphy E, Zhang W, et al. Genome-wide association study identifies multiple susceptibility loci for pulmonary fibrosis. Nat Genet. 2013;45:613-620.
  60. Noth I, Zhang Y, Ma SF, et al. Genetic variants associated with idiopathic pulmonary fibrosis susceptibility and mortality: a genome-wide association study. Lancet Respir Med. 2013;1:309-317.
  61. Peljto AL, Steele MP, Fingerlin TE, et al. The pulmonary fibrosis-associated MUC5B promoter polymorphism does not influence the development of interstitial pneumonia in systemic sclerosis. Chest. 2012;142:1584-1588.
  62. O'Dwyer DN, Armstrong ME, Trujillo G, et al. The Toll-like receptor 3 L412F polymorphism and disease progression in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 2013;188:1442-1450.

The Pathogenesis of IPF

  1. Homolka J. Idiopathic pulmonary fibrosis: a historical review. CMAJ. 1987;137(11):1003-1005.
  2. Liebow AA, Carrington CB. The interstitial pneumonias. Frontiers of Pulmonary Radiology: Pathophysiologic, Roentgenographic and Radioisotopic Considerations. Grune & Stratton; 1969. 102-141.
  3. Noble PW, Homer RJ. Back to the future: historical perspective on the pathogenesis of idiopathic pulmonary fibrosis. Am J Respir Cell Mol Biol. 2005;33(2):113-120.
  4. King TE, Jr., Pardo A, Selman M. Idiopathic pulmonary fibrosis. Lancet. 2011;378(9807):1949-1961.
  5. Wolters PJ, Collard HR, Jones KD. Pathogenesis of idiopathic pulmonary Fibrosis. Annu Rev Pathol. 2014;9:157-179.
  6. Keogh BA, Crystal RG. Alveolitis: the key to the interstitial lung disorders. Thorax. 1982;37(1):1-10.
  7. Reynolds HY, Fulmer JD, Kazmierowski JA, Roberts WC, Frank MM, Crystal RG. Analysis of cellular and protein content of broncho-alveolar lavage fluid from patients with idiopathic pulmonary fibrosis and chronic hypersensitivity pneumonitis. J Clin Invest. 1977;59(1):165-175.
  8. Katzenstein AL, Myers JL. Idiopathic pulmonary fibrosis: clinical relevance of pathologic classification. Am J Respir Crit Care Med. 1998;157(4 Pt 1):1301-1315.
  9. Raghu G, Anstrom KJ, King TE, Jr., Lasky JA, Martinez FJ. Prednisone, azathioprine, and N-acetylcysteine for pulmonary fibrosis. N Engl J Med. 2012;366(21):1968-1977.
  10. 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):136-151.
  11. 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):1322-1328.
  12. van Moorsel CH, van Oosterhout MF, Barlo NP, et al. Surfactant protein C mutations are the basis of a significant portion of adult familial pulmonary fibrosis in a dutch cohort. Am J Respir Crit Care Med. 2010;182(11):1419-1425.
  13. 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):52-59.
  14. Sime PJ, Xing Z, Graham FL, Csaky KG, Gauldie J. Adenovector-mediated gene transfer of active transforming growth factor-beta1 induces prolonged severe fibrosis in rat lung. J Clin Invest. 1997;100(4):768-776.
  15. Mulugeta S, Nguyen V, Russo SJ, Muniswamy M, Beers MF. 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):521-530.
  16. Armanios MY, Chen JJ, Cogan JD, et al. Telomerase mutations in families with idiopathic pulmonary fibrosis. N Engl J Med. 2007;356(13):1317-1326.
  17. 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(18):7552-7557.
  18. Tobin RW, Pope CE, Pellegrini CA, Emond MJ, Sillery J, Raghu G. Increased prevalence of gastroesophageal reflux in patients with idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 1998;158(6):1804-1808.
  19. Baumgartner KB, Samet JM, Stidley CA, Colby TV, Waldron JA. Cigarette smoking: a risk factor for idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 1997;155(1):242-248.
  20. Tang YW, Johnson JE, Browning PJ, et al. Herpesvirus DNA is consistently detected in lungs of patients with idiopathic pulmonary fibrosis. J Clin Microbiol. 2003;41(6):2633-2640.
  21. Raghu G, Freudenberger TD, Yang S, et al. High prevalence of abnormal acid gastro-oesophageal reflux in idiopathic pulmonary fibrosis. Eur Respir J. 2006;27(1):136-142.
  22. Maher TM. Idiopathic pulmonary fibrosis: pathobiology of novel approaches to treatment. Clin Chest Med. 2012;33(1):69-83.
  23. Uhal BD, Joshi I, True AL, et al. Fibroblasts isolated after fibrotic lung injury induce apoptosis of alveolar epithelial cells in vitro. Am J Physiol. 1995;269(6 Pt 1):L819-L828.
  24. Wang L, Taneja R, Wang W, et al. Human alveolar epithelial cells attenuate pulmonary microvascular endothelial cell permeability under septic conditions. PLoS One. 2013;8(2):e55311.
  25. Nogee LM, Garnier G, Dietz HC, et al. A mutation in the surfactant protein B gene responsible for fatal neonatal respiratory disease in multiple kindreds. J Clin Invest. 1994;93(4):1860-1863.
  26. Shulenin S, Nogee LM, Annilo T, Wert SE, Whitsett JA, Dean M. ABCA3 gene mutations in newborns with fatal surfactant deficiency. N Engl J Med. 2004;350(13):1296-1303.
  27. Desai TJ, Brownfield DG, Krasnow MA. Alveolar progenitor and stem cells in lung development, renewal and cancer. Nature. 2014;507(7491):190-194.
  28. Barkauskas CE, Cronce MJ, Rackley CR, et al. Type 2 alveolar cells are stem cells in adult lung. J Clin Invest. 2013;123(7):3025-3036.
  29. Rosas IO, Ren P, Avila NA, et al. Early interstitial lung disease in familial pulmonary fibrosis. Am J Respir Crit Care Med. 2007;176(7):698-705.
  30. Fingerlin TE, Murphy E, Zhang W, et al. Genome-wide association study identifies multiple susceptibility loci for pulmonary fibrosis. Nat Genet. 2013;45(6):613-620.
  31. Noth I, Zhang Y, Ma SF, et al. Genetic variants associated with idiopathic pulmonary fibrosis susceptibility and mortality: a genome-wide association study. Lancet Respir Med. 2013;1(4):309-317.
  32. Seibold MA, Wise AL, Speer MC, et al. A common MUC5B promoter polymorphism and pulmonary fibrosis. N Engl J Med. 2011;364(16):1503-1512.
  33. 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.
  34. 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):436-441.
  35. Zhang Y, Noth I, Garcia JG, Kaminski N. A variant in the promoter of MUC5B and idiopathic pulmonary fibrosis. N Engl J Med. 2011;364(16):1576-1577.
  36. Hunninghake GM, Hatabu H, Okajima Y, et al. MUC5B promoter polymorphism and interstitial lung abnormalities. N Engl J Med. 2013;368(23):2192-2200.
  37. Roy MG, Livraghi-Butrico A, Fletcher AA, et al. Muc5b is required for airway defence. Nature. 2014;505(7483):412-416.
  38. Kropski JA, Mitchell DB, Markin C, et al. A novel dyskerin (DKC1) mutation is associated with Familial Interstitial Pneumonia. Chest. 2014.
  39. 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(35):13051-13056.
  40. Cronkhite JT, Xing C, Raghu G, et al. Telomere shortening in familial and sporadic pulmonary fibrosis. Am J Respir Crit Care Med. 2008;178(7):729-737.
  41. Rahman I, Skwarska E, Henry M, et al. Systemic and pulmonary oxidative stress in idiopathic pulmonary fibrosis. Free Radic Biol Med. 1999;27(1-2):60-68.
  42. Waghray M, Cui Z, Horowitz JC, et al. Hydrogen peroxide is a diffusible paracrine signal for the induction of epithelial cell death by activated myofibroblasts. FASEB J. 2005;19(7):854-856.
  43. Kliment CR, Englert JM, Gochuico BR, et al. Oxidative stress alters syndecan-1 distribution in lungs with pulmonary fibrosis. J Biol Chem. 2009;284(6):3537-3545.
  44. Bocchino M, Agnese S, Fagone E, et al. Reactive oxygen species are required for maintenance and differentiation of primary lung fibroblasts in idiopathic pulmonary fibrosis. PLoS One. 2010;5(11):e14003.
  45. Hecker L, Vittal R, Jones T, et al. NADPH oxidase-4 mediates myofibroblast activation and fibrogenic responses to lung injury. Nat Med. 2009;15(9):1077-1081.
  46. Sueblinvong V, Neujahr DC, Mills ST, et al. Predisposition for disrepair in the aged lung. Am J Med. Sci 2012;344(1):41-51.
  47. Araya J, Kojima J, Takasaka N, et al. Insufficient autophagy in idiopathic pulmonary fibrosis. Am J Physiol Lung Cell Mol Physiol. 2013;304(1):L56-L69.
  48. Torres-Gonzalez E, Bueno M, Tanaka A, et al. Role of endoplasmic reticulum stress in age-related susceptibility to lung fibrosis. Am J Respir Cell Mol Biol. 2012;46(6):748-756.
  49. Heise RL, Stober V, Cheluvaraju C, Hollingsworth JW, Garantziotis S. Mechanical stretch induces epithelial-mesenchymal transition in alveolar epithelia via hyaluronan activation of innate immunity. J Biol Chem. 2011;286(20):17435-17444.
  50. Yamamoto H, Teramoto H, Uetani K, Igawa K, Shimizu E. Stretch induces a growth factor in alveolar cells via protein kinase. Respir Physiol. 2001;127(2-3):105-111.
  51. Cabrera-Benitez NE, Parotto M, Post M, et al. Mechanical stress induces lung fibrosis by epithelial-mesenchymal transition. Crit Care Med. 2012;40(2):510-517.
  52. Nakstad B, Lyberg T, Skjonsberg OH, Boye NP. Local activation of the coagulation and fibrinolysis systems in lung disease. Thromb Res. 1990;57(6):827-838.
  53. Gunther A, Mosavi P, Ruppert C, et al. Enhanced tissue factor pathway activity and fibrin turnover in the alveolar compartment of patients with interstitial lung disease. Thromb Haemost 2000;83(6):853-860.
  54. Chapman HA, Allen CL, Stone OL. Abnormalities in pathways of alveolar fibrin turnover among patients with interstitial lung disease. Am Rev Respir Dis. 1986;133(3):437-443.
  55. Scotton CJ, Krupiczojc MA, Konigshoff M, et al. Increased local expression of coagulation factor X contributes to the fibrotic response in human and murine lung injury. J Clin Invest. 2009;119(9):2550-2563.
  56. Mercer PF, Johns RH, Scotton CJ, et al. Pulmonary epithelium is a prominent source of proteinase-activated receptor-1-inducible CCL2 in pulmonary fibrosis. Am J Respir Crit Care Med. 2009;179(5):414-425.
  57. Borensztajn K, Bresser P, van der Loos C, et al. Protease-activated receptor-2 induces myofibroblast differentiation and tissue factor up-regulation during bleomycin-induced lung injury: potential role in pulmonary fibrosis. Am J Pathol. 2010;177(6):2753-2764.
  58. Lee JS, Collard HR, Anstrom KJ, et al. Anti-acid treatment and disease progression in idiopathic pulmonary fibrosis: an analysis of data from three randomised controlled trials. Lancet Respir Med. 2013;1(5):369-376.
  59. Xu YD, Hua J, Mui A, O'Connor R, Grotendorst G, Khalil N. Release of biologically active TGF-β1 by alveolar epithelial cells results in pulmonary fibrosis. Am J Physiol Lung Cell Mol Physiol. 2003;285(3):L527-L539.
  60. Khalil N, Parekh TV, O'Connor R, et al. Regulation of the effects of TGF-β1 by activation of latent TGF-β1 and differential expression of TGF-βreceptors (TβR-I and TβR-II) in idiopathic pulmonary fibrosis. Thorax. 2001;56(12):907-915.
  61. Munger JS, Huang X, Kawakatsu, H, et al. The integrin αvβ6 binds and activates latent TGFβ1: a mechanism for regulating pulmonary inflammation and fibrosis. Cell. 1999;96(3):319-328.
  62. Pandit KV, Corcoran D, Yousef H, et al. Inhibition and role of let-7d in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 2010;182:220-229.
  63. Lee CG, Cho SJ, Kang MJ, et al. Early growth response gene 1-mediated apoptosis is essential for transforming growth factor beta1-induced pulmonary fibrosis. J Exp Med. 2004;200(3):377-389.
  64. Xu SW, Howat SL, Renzoni EA, et al. Endothelin-1 induces expression of matrix-associated genes in lung fibroblasts through MEK/ERK. J Biol Chem. 2004;279(22):23098-23103.
  65. Bogatkevich GS, Ludwicka-Bradley A, Singleton CB, Bethard JR, Silver RM. Proteomic analysis of CTGF-activated lung fibroblasts: identification of IQGAP1 as a key player in lung fibroblast migration. Am J Physiol Lung Cell Mol Physiol. 2008;295(4):L603-L611.
  66. Martinet Y, Bitterman PB, Mornex JF, Grotendorst GR, Martin GR, Crystal RG. Activated human monocytes express the c-sis proto-oncogene and release a mediator showing PDGF-like activity. Nature 1986;319(6049):158-160.
  67. Phillips RJ, Burdick MD, Hong K, et al. Circulating fibrocytes traffic to the lungs in response to CXCL12 and mediate fibrosis. J Clin Invest. 2004;114(3):438-446.
  68. Tager AM, LaCamera P, Shea BS, et al. The lysophosphatidic acid receptor LPA1 links pulmonary fibrosis to lung injury by mediating fibroblast recruitment and vascular leak. Nat Med. 2008;14(1):45-54.
  69. Andersson-Sjoland A, de Alba CG, Nihlberg K, et al. Fibrocytes are a potential source of lung fibroblasts in idiopathic pulmonary fibrosis. Int J Biochem Cell Biol. 2008;40(10):2129-2140.
  70. Chilosi M, Poletti V, Zamo A, et al. Aberrant Wnt/β-catenin pathway activation in idiopathic pulmonary fibrosis. Am J Pathol. 2003;162(5):1495-1502.
  71. Konigshoff M, Balsara N, Pfaff EM, et al. Functional Wnt signaling is increased in idiopathic pulmonary fibrosis. PLoS One. 2008;3(5):e2142.
  72. Tanjore H, Degryse AL, Crossno PF, et al. β-catenin in the alveolar epithelium protects from lung fibrosis after intratracheal bleomycin. Am J Respir Crit Care Med. 2013;187(6):630-639.
  73. Milosevic J, Pandit K, Magister M, et al. Profibrotic role of miR-154 in pulmonary fibrosis. Am J Respir Cell Mol Biol. 2012;47(6):879-887.
  74. Bolanos AL, Milla CM, Lira JC, et al. Role of Sonic Hedgehog in idiopathic pulmonary fibrosis. Am J Physiol Lung Cell Mol Physiol. 2012;303(11):L978-L990.
  75. Zuo F, Kaminski N, Eugui E, et al. 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(9):6292-6297.
  76. Pardo A, Selman M. Role of matrix metaloproteases in idiopathic pulmonary fibrosis. Fibrogenesis & Tissue Repair. 2012;5(Suppl 1):59.
  77. Hayashi T, Stetler-Stevenson WG, Fleming MV, et al. Immunohistochemical study of metalloproteinases and their tissue inhibitors in the lungs of patients with diffuse alveolar damage and idiopathic pulmonary fibrosis. Am J Pathol. 1996;149(4):1241-1256.
  78. Swiderski RE, Dencoff JE, Floerchinger CS, Shapiro SD, Hunninghake GW. Differential expression of extracellular matrix remodeling genes in a murine model of bleomycin-induced pulmonary fibrosis. Am J Pathol. 1998;152(3):821-828.
  79. Ramos C, Montano M, Garcia-Alvarez J, et al. Fibroblasts from idiopathic pulmonary fibrosis and normal lungs differ in growth rate, apoptosis, and tissue inhibitor of metalloproteinases expression. Am J Respir Cell Mol Biol. 2001;24(5):591-598.
  80. Zhang HY, Gharaee-Kermani M, Zhang K, Karmiol S, Phan SH. Lung fibroblast α-smooth muscle actin expression and contractile phenotype in bleomycin-induced pulmonary fibrosis. Am J Pathol. 1996;148(2):527-537.
  81. Bostrom H, Willetts K, Pekny M, et al. PDGF-A signaling is a critical event in lung alveolar myofibroblast development and alveogenesis. Cell. 1996;85(6):863-873.
  82. Tai IT, Dai M, Chen LB. Periostin induction in tumor cell line explants and inhibition of in vitro cell growth by anti-periostin antibodies. Carcinogenesis. 2005;26(5):908-915.
  83. Naik PK, Bozyk PD, Bentley JK, et al. Periostin promotes fibrosis and predicts progression in patients with idiopathic pulmonary fibrosis. Am J Physiol Lung Cell Mol Physiol. 2012;303(12):L1046-L1056.
  84. Horowitz JC, Lee DY, Waghray M, et al. Activation of the pro-survival phosphatidylinositol 3-kinase/AKT pathway by transforming growth factor-β1 in mesenchymal cells is mediated by p38 MAPK-dependent induction of an autocrine growth factor. J Biol Chem. 2004;279(2):1359-1367.
  85. Booth AJ, Hadley R, Cornett AM, et al. Acellular normal and fibrotic human lung matrices as a culture system for in vitro investigation. Am J Respir Crit Care Med. 2012;186(9):866-876.
  86. Henderson NC, Arnold TD, Katamura Y, et al. Targeting of αv integrin identifies a core molecular pathway that regulates fibrosis in several organs. Nat Med. 2013;19(12):1617-1624.
  87. 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):525-535.
  88. Rabinovich EI, Kapetanaki MG, Steinfeld I, et al. Global methylation patterns in idiopathic pulmonary fibrosis. PLoS One. 2012;7(4):e33770.
  89. Nance T, Smith KS, Anaya V, et al. Transcriptome analysis reveals differential splicing events in IPF lung tissue. PLoS One. 2014;9(3):e92111.
  90. 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):1589-1597.
  91. Lino Cardenas CL, Henaoui IS, Courcot E, et al. miR-199a-5p Is upregulated during fibrogenic response to tissue injury and mediates TGFbeta-induced lung fibroblast activation by targeting caveolin-1. PLoS Genet. 2013;9(2):e1003291.
  92. Kim DS, Park JH, Park BK, Lee JS, Nicholson AG, Colby T. Acute exacerbation of idiopathic pulmonary fibrosis: frequency and clinical features. Eur Respir J. 2006;27(1):143-150.
  93. Lee JS, Song JW, Wolters PJ, et al. Bronchoalveolar lavage pepsin in acute exacerbation of idiopathic pulmonary fibrosis. Eur Respir J. 2012;39(2):352-358.
  94. Konishi K, Gibson KF, Lindell KO, et al. Gene expression profiles of acute exacerbations of Idiopathic Pulmonary Fibrosis. Am J Respir Crit Care Med. 2009;180:167-175.
  95. Wootton SC, Kim DS, Kondoh Y, et al. Viral infection in acute exacerbation of idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 2011;183(12):1698-1702.
  96. 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.

Diagnosis of IPF

  1. Ley B, Collard HR, King TE, Jr. Clinical course and prediction of survival in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 2011;183:431-440.
  2. Raghu G. Idiopathic pulmonary fibrosis: guidelines for diagnosis and clinical management have advanced from consensus-based in 2000 to evidence-based in 2011. Eur Respir J. 2011;37:743-746.
  3. Bjoraker JA, Ryu JH, Edwin MK, et al. Prognostic significance of histopathologic subsets in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 1998;157:199-203.
  4. American Cancer Society. Cancer Facts and Figures 2010. Atlanta: American Cancer Society, 2010.
  5. King TE, Jr., Tooze JA, Schwarz MI, Brown KR, Cherniack RM. Predicting survival in idiopathic pulmonary fibrosis: scoring system and survival model. Am J Respir Crit Care Med. 2001;164:1171-1181.
  6. Wells AU. Managing diagnostic procedures in idiopathic pulmonary fibrosis. Eur Respir Rev. 2013;22:158-162.
  7. American Thoracic Society. Idiopathic pulmonary fibrosis: diagnosis and treatment. International consensus statement. American Thoracic Society (ATS), and the European Respiratory Society (ERS). Am J Respir Crit Care Med. 2000;161:646-664.
  8. 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.
  9. du Bois RM. An earlier and more confident diagnosis of idiopathic pulmonary fibrosis. Eur Respir Rev. 2012;21:141-146.
  10. Kim DS, Collard HR, King TE, Jr. Classification and natural history of the idiopathic interstitial pneumonias. Proc Am Thorac Soc. 2006;3:285-292.
  11. Castriotta RJ, Eldadah BA, Foster WM, et al. Workshop on idiopathic pulmonary fibrosis in older adults. Chest. 2010;138:693-703.
  12. Meltzer EB, Noble PW. Idiopathic pulmonary fibrosis. Orphanet J Rare Dis. 2008;3:1-15.
  13. Fell CD, Martinez FJ, Liu LX, et al. Clinical predictors of a diagnosis of idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 2010;181:832-837.
  14. Douglas WW, Ryu JH, Schroeder DR. Idiopathic pulmonary fibrosis: Impact of oxygen and colchicine, prednisone, or no therapy on survival. Am J Respir Crit Care Med. 2000;161:1172-1178.
  15. Cottin V, Cordier JF. Velcro crackles: the key for early diagnosis of idiopathic pulmonary fibrosis? Eur Respir J. 2012;40:519-521.
  16. Epler GR, Carrington CB, Gaensler EA. Crackles (rales) in the interstitial pulmonary diseases. Chest. 1978;73:333-339.
  17. Kraman SS. Lung sounds for the clinician. Arch Intern Med. 1986;146:1411-1412.
  18. Panos RJ, Mortenson RL, Niccoli SA, King TE, Jr. Clinical deterioration in patients with idiopathic pulmonary fibrosis: causes and assessment. Am J Med. 1990;88:396-404.
  19. Baumgartner KB, Samet JM, Stidley CA, Colby TV, Waldron JA. Cigarette smoking: a risk factor for idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 1997;155:242-248.
  20. Baumgartner KB, Samet JM, Coultas DB et al. Occupational and environmental risk factors for idiopathic pulmonary fibrosis: a multicenter case-control study. Collaborating Centers. Am J Epidemiol 2000;152:307-315.
  21. Selman M, Pardo A, King TE, Jr. Hypersensitivity pneumonitis: insights in diagnosis and pathobiology. Am J Respir Crit Care Med. 2012;186:314-324.
  22. Selman M. Hypersensitivity pneumonitis. In: Schwarz M, King TE, Jr., eds. Interstitial lung disease. 5th ed. Shelton, CT: People's Medical Publishing House; 2011:597-625.
  23. Morell F, Villar A, Montero MA, et al. Chronic hypersensitivity pneumonitis in patients diagnosed with idiopathic pulmonary fibrosis: a prospective case-cohort study. Lancet Respir Med. 2013;1:685-694.
  24. Camus P. The Drug-Induced Respiratory Disease Website. 2012. Available at www.pneumotox.com. Accessed April 4, 2014.
  25. Schwaiblmair M, Behr W, Haeckel T, Markl B, Foerg W, Berghaus T. Drug induced interstitial lung disease. Open Respir Med J. 2012;6:63-74.
  26. Raghu G, Freudenberger TD, Yang S, et al. High prevalence of abnormal acid gastro-oesophageal reflux in idiopathic pulmonary fibrosis. Eur Respir J. 2006;27:136-142.
  27. Nathan SD, Basavaraj A, Reichner C, et al. Prevalence and impact of coronary artery disease in idiopathic pulmonary fibrosis. Respir Med. 2010;104:1035-1041.
  28. Wrobel JP, Thompson BR, Williams TJ. Mechanisms of pulmonary hypertension in chronic obstructive pulmonary disease: a pathophysiologic review. J Heart Lung Transplant. 2012;31:557-564.
  29. Caminati A, Cassandro R, Harari S. Pulmonary hypertension in chronic interstitial lung diseases. Eur Respir Rev. 2013;22:292-301.
  30. Hubbard R, Venn A, Lewis S, Britton J. Lung cancer and cryptogenic fibrosing alveolitis. A population-based cohort study. Am J Respir Crit Care Med. 2000;161:5-8.
  31. Kinder BW, Collard HR, Koth L, et al. Idiopathic nonspecific interstitial pneumonia: lung manifestation of undifferentiated connective tissue disease? Am J Respir Crit Care Med. 2007;176:691-697.
  32. Vij R, Strek ME. Diagnosis and treatment of connective tissue disease-associated interstitial lung disease. Chest. 2013;143:814-824.
  33. Ahmad Y, Bruce I. Connective tissue diseases and the role of the general practitioner. In: Adebajo AO, Dickson DJ, eds. Collected reports on the rheumatic diseases 2005. Series 4 (revised); Arthritis Research Campaign; 2005:85-88.
  34. British Columbia Medical Association. Antinuclear antibody (ANA) testing protocol. Available at www.bcguidelines.ca. Accessed April 24, 2014.
  35. Han MK, McLaughlin VV, Criner GJ, Martinez FJ. Pulmonary diseases and the heart. Circulation. 2007;116:2992-3005.
  36. Portillo CK, Roldan SJ, Morera PJ. [Combined pulmonary fibrosis and emphysema]. Arch Bronconeumol. 2010;46:646-651.
  37. Cottin V, Nunes H, Brillet PY, et al. Combined pulmonary fibrosis and emphysema: a distinct underrecognised entity. Eur Respir J. 2005;26:586-593.
  38. Ryu JH, Daniels CE, Hartman TE, Yi ES. Diagnosis of interstitial lung diseases. Mayo Clin Proc. 2007;82:976-986.
  39. Schmidt SL, Sundaram B, Flaherty KR. Diagnosing fibrotic lung disease: when is high-resolution computed tomography sufficient to make a diagnosis of idiopathic pulmonary fibrosis? Respirology. 2009;14:934-939.
  40. Larsen BT, Colby TV. Update for pathologists on idiopathic interstitial pneumonias. Arch Pathol Lab Med. 2012;136:1234-1241.
  41. Hansell DM, Bankier AA, MacMahon H, McLoud TC, Muller NL, Remy J. Fleischner Society: glossary of terms for thoracic imaging. Radiology. 2008;246:697-722.
  42. Johkoh T, Muller NL, Cartier Y, et al. Idiopathic interstitial pneumonias: diagnostic accuracy of thin-section CT in 129 patients. Radiology. 1999;211:555-560.
  43. Hunninghake GW, Lynch DA, Galvin JR, et al. Radiologic findings are strongly associated with a pathologic diagnosis of usual interstitial pneumonia. Chest. 2003;124:1215-1223.
  44. Katzenstein AL, Myers JL. Idiopathic pulmonary fibrosis: clinical relevance of pathologic classification. Am J Respir Crit Care Med. 1998;157:1301-1315.
  45. Travis WD, Matsui K, Moss J, Ferrans VJ. Idiopathic nonspecific interstitial pneumonia: prognostic significance of cellular and fibrosing patterns: survival comparison with usual interstitial pneumonia and desquamative interstitial pneumonia. Am J Surg Pathol. 2000;24:19-33.
  46. Cool CD, Groshong SD, Rai PR, Henson PM, Stewart JS, Brown KK. Fibroblast foci are not discrete sites of lung injury or repair: the fibroblast reticulum. Am J Respir Crit Care Med. 2006;174:654-658.
  47. Flaherty KR, King TE, Jr., Raghu G, et al. Idiopathic interstitial pneumonia: what is the effect of a multidisciplinary approach to diagnosis? Am J Respir Crit Care Med. 2004;170:904-910.

Challenges in IPF Management

  1. 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.
  2. Jegal Y, Kim DS, Shim TS, et al. Physiology is a stronger predictor of survival than pathology in fibrotic interstitial pneumonia. Am J Respir Crit Care Med. 2005;171:639-644.
  3. Collard HR, King TE, Jr., Bartelson BB, Vourlekis JS, Schwarz MI, Brown KK. Changes in clinical and physiologic variables predict survival in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 2003;168:538-542.
  4. King TE, Jr., Safrin S, Starko KM, et al. Analyses of efficacy end points in a controlled trial of interferon-γ1b for idiopathic pulmonary fibrosis. Chest. 2005;127:171-177.
  5. Latsi PI, du Bois RM, Nicholson AG, et al. Fibrotic idiopathic interstitial pneumonia: the prognostic value of longitudinal functional trends. Am J Respir Crit Care Med. 2003;168:531-537.
  6. Flaherty KR, Mumford JA, Murray S, et al. Prognostic implications of physiologic and radiographic changes in idiopathic interstitial pneumonia. Am J Respir Crit Care Med. 2003;168:543-548.
  7. 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;185:1044-1048.
  8. Song JW, Hong SB, Lim CM, Koh Y, Kim DS. Acute exacerbation of idiopathic pulmonary fibrosis: incidence, risk factors and outcome. Eur Respir J. 2011;37:356-363.
  9. Kondoh Y, Taniguchi H, Katsuta T, et al. Risk factors of acute exacerbation of idiopathic pulmonary fibrosis. Sarcoidosis Vasc Diffuse Lung Dis. 2010;27:103-110.
  10. Lama VN, Flaherty KR, Toews GB, et al. Prognostic value of desaturation during a 6-minute walk test in idiopathic interstitial pneumonia. Am J Respir Crit Care Med. 2003;168:1084-1090.
  11. 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;184:1382-1389.
  12. Zappala CJ, Latsi PI, Nicholson AG, et al. Marginal decline in forced vital capacity is associated with a poor outcome in idiopathic pulmonary fibrosis. Eur Respir J. 2010;35:830-836.
  13. Egan JJ, Martinez FJ, Wells AU, Williams T. Lung function estimates in idiopathic pulmonary fibrosis: the potential for a simple classification. Thorax. 2005;60:270-273.
  14. Swigris JJ, Gould MK, Wilson SR. Health-related quality of life among patients with idiopathic pulmonary fibrosis. Chest. 2005;127:284-294.
  15. Best AC, Meng J, Lynch AM, et al. Idiopathic pulmonary fibrosis: physiologic tests, quantitative CT indexes, and CT visual scores as predictors of mortality. Radiology. 2008;246:935-940.
  16. Shin KM, Lee KS, Chung MP, et al. Prognostic determinants among clinical, thin-section CT, and histopathologic findings for fibrotic idiopathic interstitial pneumonias: tertiary hospital study. Radiology. 2008;249:328-337.
  17. Sumikawa H, Johkoh T, Colby TV, et al. Computed tomography findings in pathological usual interstitial pneumonia: relationship to survival. Am J Respir Crit Care Med. 2008;177:433-439.
  18. Flaherty KR, Thwaite EL, Kazerooni EA, et al. Radiological versus histological diagnosis in UIP and NSIP: survival implications. Thorax. 2003;58:143-148.
  19. Collard HR, Moore BB, Flaherty KR, et al. Acute exacerbations of idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 2007;176:636-643.
  20. du Bois RM, Weycker D, Albera C, et al. Ascertainment of individual risk of mortality for patients with idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 2011;184:459-466.
  21. Kondoh Y, Taniguchi H, Kawabata Y, Yokoi T, Suzuki K, Takagi K. Acute exacerbation in idiopathic pulmonary fibrosis. Analysis of clinical and pathologic findings in three cases. Chest. 1993;103:1808-1812.
  22. Panos RJ, Mortenson RL, Niccoli SA, King TE, Jr. Clinical deterioration in patients with idiopathic pulmonary fibrosis: causes and assessment. Am J Med. 1990;88:396-404.
  23. Hallstrand TS, Boitano LJ, Johnson WC, Spada CA, Hayes JG, Raghu G. The timed walk test as a measure of severity and survival in idiopathic pulmonary fibrosis. Eur Respir J. 2005;25:96-103.
  24. Lederer DJ, Arcasoy SM, Wilt JS, D'Ovidio F, Sonett JR, Kawut SM. Six-minute-walk distance predicts waiting list survival in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 2006;174:659-664.
  25. Swigris JJ, Swick J, Wamboldt FS, et al. Heart rate recovery after 6-min walk test predicts survival in patients with idiopathic pulmonary fibrosis. Chest. 2009;136:841-848.
  26. Caminati A, Bianchi A, Cassandro R, Mirenda MR, Harari S. Walking distance on 6-MWT is a prognostic factor in idiopathic pulmonary fibrosis. Respir Med. 2009;103:117-123.
  27. Lynch DA, Godwin JD, Safrin S, et al. High-resolution computed tomography in idiopathic pulmonary fibrosis: diagnosis and prognosis. Am J Respir Crit Care Med. 2005;172:488-493.
  28. Swigris JJ, Kuschner WG, Jacobs SS, Wilson SR, Gould MK. Health-related quality of life in patients with idiopathic pulmonary fibrosis: a systematic review. Thorax. 2005;60:588-594.
  29. De Vries J, Seebregts A, Drent M. Assessing health status and quality of life in idiopathic pulmonary fibrosis: which measure should be used? Respir Med. 2000;94:273-278.
  30. De Vries J, Kessels BL, Drent M. Quality of life of idiopathic pulmonary fibrosis patients. Eur Respir J. 2001;17:954-961.
  31. Baddini Martinez JA, Martinez TY, Lovetro Galhardo FP, de Castro Pereira CA. Dyspnea scales as a measure of health-related quality of life in patients with idiopathic pulmonary fibrosis. Med Sci Monit. 2002;8:CR405-CR410.
  32. Martinez TY, Pereira CA, dos Santos ML, Ciconelli RM, Guimaraes SM, Martinez JA. Evaluation of the short-form 36-item questionnaire to measure health-related quality of life in patients with idiopathic pulmonary fibrosis. Chest. 2000;117:1627-1632.
  33. Nishiyama O, Taniguchi H, Kondoh Y, et al. Health-related quality of life in patients with idiopathic pulmonary fibrosis. What is the main contributing factor? Respir Med. 2005;99:408-414.
  34. Chang JA, Curtis JR, Patrick DL, Raghu G. Assessment of health-related quality of life in patients with interstitial lung disease. Chest. 1999;116:1175-1182.
  35. Swigris JJ, Stewart AL, Gould MK, Wilson SR. Patients' perspectives on how idiopathic pulmonary fibrosis affects the quality of their lives. Health Qual Life Outcomes 2005;3:61.
  36. Lee JS, McLaughlin S, Collard HR. Comprehensive care of the patient with idiopathic pulmonary fibrosis. Curr Opin Pulm Med 2011;17(5):348-354.
  37. Bradley B, Branley HM, Egan JJ et al. Interstitial lung disease guideline: the British Thoracic Society in collaboration with the Thoracic Society of Australia and New Zealand and the Irish Thoracic Society. Thorax 2008;63 Suppl 5:v1-58.
  38. Holland AE, Hill CJ, Conron M, Munro P, McDonald CF. Short term improvement in exercise capacity and symptoms following exercise training in interstitial lung disease. Thorax 2008;63(6):549-554.
  39. Nishiyama O, Kondoh Y, Kimura T et al. Effects of pulmonary rehabilitation in patients with idiopathic pulmonary fibrosis. Respirology 2008;13(3):394-399.
  40. Nici L, Donner C, Wouters E et al. American Thoracic Society/European Respiratory Society statement on pulmonary rehabilitation. Am J Respir Crit Care Med 2006;173(12):1390-1413.
  41. Allen S, Raut S, Woollard J, Vassallo M. Low dose diamorphine reduces breathlessness without causing a fall in oxygen saturation in elderly patients with end-stage idiopathic pulmonary fibrosis. Palliat Med 2005;19(2):128-130.
  42. Brown KK. Chronic cough due to chronic interstitial pulmonary diseases: ACCP evidence-based clinical practice guidelines. Chest 2006;129(1 Suppl):180S-185S.
  43. Swigris JJ, Fairclough DL, Morrison M et al. Benefits of pulmonary rehabilitation in idiopathic pulmonary fibrosis. Respir Care 2011;56(6):783-789.
  44. Akhtar AA, Ali MA, Smith RP. Depression in patients with idiopathic pulmonary fibrosis. Chron Respir Dis 2013;10(3):127-133.
  45. Ferreira A, Garvey C, Connors GL et al. Pulmonary rehabilitation in interstitial lung disease: benefits and predictors of response. Chest 2009;135(2):442-447.
  46. Lee AS, Mira-Avendano I, Ryu JH, Daniels CE. The burden of idiopathic pulmonary fibrosis: an unmet public health need. Respir Med. 2014;108:955-967.
  47. Collard HR, Ward AJ, Lanes S, et al. Burden of illness in idiopathic pulmonary fibrosis. J Med Econ. 2012;15:829-835.
  48. Navaratnam V, Fogarty AW, Glendening R, McKeever T, Hubbard RB. The increasing secondary care burden of idiopathic pulmonary fibrosis: hospital admission trends in England from 1998 to 2010. Chest. 2013;143:1078-1084.
  49. Wu N, Yu YF, Chuang CC, et al. Healthcare resource utilization among patients diagnosed with idiopathic pulmonary fibrosis in the United States. J Med Econ. 2015;18(4):249-257.
  50. Wu N, Yu Y, Chuang CC, et al. Hospitalization and exacerbation patterns and the associated burden among patients newly diagnosed with idiopathic pulmonary fibrosis. Poster presented at the Pulmonary Fibrosis Foundation Summit. 2013. Available at http://www.pffsummit.org/assets/summit13-workbook-pro.pdf. Accessed April 28, 2015.
  51. Yu Y, Wu N, Chuang C-C, et al. Burden of hospital admissions and exacerbations among patients newly diagnosed with idiopathic pulmonary fibrosis. Presented at the American Thoracic Society Annual Meeting. San Diego, CA. 2014.
  52. Annual Hospital Use for Patients with IPF - 2012. Available at http://hcupnet ahrq gov/. Accessed March 27, 2015.

Auscultation of Breath Sounds

  1. Reichert S, Gass R, Brandt C, Andres E. Analysis of respiratory sounds: state of the art. Clin Med Circ Respirat Pulm Med. 2008;2:45-58.
  2. Mikami R, Murao M, Cugell DW, et al. International Symposium on Lung Sounds. Synopsis of proceedings. Chest. 1987;92:342-345.
  3. Cugell DW. Lung sound nomenclature. Am Rev Respir Dis. 1987;136:1016.
  4. Pasterkamp H, Kraman SS, Wodicka GR. Respiratory sounds. Advances beyond the stethoscope. Am J Respir Crit Care Med. 1997;156:974-987.
  5. Epler GR, Carrington CB, Gaensler EA. Crackles (rales) in the interstitial pulmonary diseases. Chest. 1978;73:333-339.
  6. Kraman SS. Lung sounds for the clinician. Arch Intern Med. 1986;146:1411-1412.
  7. Forgacs P. The functional basis of pulmonary sounds. Chest. 1978;73:399-405.
  8. Standards for the diagnosis and care of patients with chronic obstructive pulmonary disease. American Thoracic Society. Am J Respir Crit Care Med. 1995;152:S77-121.