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Recognizing and Treating Comorbidities of IPF

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Idiopathic Pulmonary Fibrosis

Part of the book series: Respiratory Medicine ((RM,volume 9))

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Abstract

Idiopathic pulmonary fibrosis (IPF) is a progressive and unrelenting disease whose natural history may be complicated by multiple comorbidities. Among these, this chapter will highlight the prevalence, clinical approach, and management of the following: acute exacerbation (AE), combined pulmonary fibrosis and emphysema (CPFE), pulmonary hypertension (PH), gastroesophageal reflux disease (GERD), thromboembolic disease, cardiovascular disease, lung cancer, and end-of-life care.

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References

  1. Daniels CE, Yi ES, Ryu JH. Autopsy findings in 42 consecutive patients with idiopathic pulmonary fibrosis. Eur Respir J. 2008;32(1):170–4.

    PubMed  CAS  Google Scholar 

  2. Panos RJ, Mortenson RL, Niccoli SA, King Jr TE. Clinical deterioration in patients with idiopathic pulmonary fibrosis: causes and assessment. Am J Med. 1990;88(4):396–404.

    PubMed  CAS  Google Scholar 

  3. Ambrosini V, Cancellieri A, Chilosi M, Zompatori M, Trisolini R, Saragoni L, et al. Acute exacerbation of idiopathic pulmonary fibrosis: report of a series. Eur Respir J. 2003;22(5):821–6.

    PubMed  CAS  Google Scholar 

  4. 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):143–50.

    PubMed  CAS  Google Scholar 

  5. Akira M, Kozuka T, Yamamoto S, Sakatani M. Computed tomography findings in acute exacerbation of idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 2008;178(4):372–8.

    PubMed  Google Scholar 

  6. 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(6):1808–12.

    PubMed  CAS  Google Scholar 

  7. Collard HR, Moore BB, Flaherty KR, Brown KK, Kaner RJ, King TE, et al. Acute exacerbations of idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 2007;176(7):636–43.

    PubMed  Google Scholar 

  8. Kubo H, Nakayama K, Yanai M, Suzuki T, Yamaya M, Watanabe M, et al. Anticoagulant therapy for idiopathic pulmonary fibrosis. Chest. 2005;128(3):1475–82.

    PubMed  CAS  Google Scholar 

  9. Azuma A, Nukiwa T, Tsuboi E, Suga M, Abe S, Nakata K, et al. Double-blind, placebo-controlled trial of pirfenidone in patients with idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 2005;171(9):1040–7.

    PubMed  Google Scholar 

  10. Agarwal R, Jindal SK. Acute exacerbation of idiopathic pulmonary fibrosis: a systematic review. Eur J Intern Med. 2008;19(4):227–35.

    PubMed  Google Scholar 

  11. Noble PW, Albera C, Bradford WZ, Costabel U, Glassberg MK, Kardatzke D, et al. Pirfenidone in patients with idiopathic pulmonary fibrosis (CAPACITY): two randomised trials. Lancet. 2011;377(9779):1760–9.

    PubMed  CAS  Google Scholar 

  12. 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(2):356–63.

    PubMed  CAS  Google Scholar 

  13. Sakamoto K, Taniguchi H, Kondoh Y, Ono K, Hasegawa Y, Kitaichi M. Acute exacerbation of idiopathic pulmonary fibrosis as the initial presentation of the disease. Eur Respir Rev. 2009;18(112):129–32.

    PubMed  CAS  Google Scholar 

  14. Parambil JG, Myers JL, Ryu JH. Histopathologic features and outcome of patients with acute exacerbation of idiopathic pulmonary fibrosis undergoing surgical lung biopsy. Chest. 2005;128(5):3310–5.

    PubMed  Google Scholar 

  15. Silva CI, Müller NL, Fujimoto K, Kato S, Ichikado K, Taniguchi H, et al. Acute exacerbation of chronic interstitial pneumonia: high-resolution computed tomography and pathologic findings. J Thorac Imaging. 2007;22(3):221–9.

    PubMed  Google Scholar 

  16. Park IN, Kim DS, Shim TS, Lim CM, Lee SD, Koh Y, et al. Acute exacerbation of interstitial pneumonia other than idiopathic pulmonary fibrosis. Chest. 2007;132(1):214–20.

    PubMed  Google Scholar 

  17. Hyzy R, Huang S, Myers J, Flaherty K, Martinez F. Acute exacerbation of idiopathic pulmonary fibrosis. Chest. 2007;132(5):1652–8.

    PubMed  Google Scholar 

  18. Collard HR, Calfee CS, Wolters PJ, Song JW, Hong SB, Brady S, et al. Plasma biomarker profiles in acute exacerbation of idiopathic pulmonary fibrosis. Am J Physiol Lung Cell Mol Physiol. 2010;299(1):L3–7.

    PubMed  CAS  Google Scholar 

  19. Selman M, Pardo A. Role of epithelial cells in idiopathic pulmonary fibrosis: from innocent targets to serial killers. Proc Am Thorac Soc. 2006;3(4):364–72.

    PubMed  CAS  Google Scholar 

  20. Wootton SC, Kim DS, Kondoh Y, Chen E, Lee JS, Song JW, et al. Viral infection in acute exacerbation of idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 2011;183(12):1698–702.

    PubMed  Google Scholar 

  21. Lee JS, Song JW, Wolters PJ, Elicker BM, King TE, Kim DS, et al. Bronchoalveolar lavage pepsin in acute exacerbation of idiopathic pulmonary fibrosis. Eur Respir J. 2012;39(2):352–8.

    PubMed  CAS  Google Scholar 

  22. Churg A, Muller NL, Silva CI, Wright JL. Acute exacerbation (acute lung injury of unknown cause) in UIP and other forms of fibrotic interstitial pneumonias. Am J Surg Pathol. 2007;31(2):277–84.

    PubMed  Google Scholar 

  23. Suda T, Kaida Y, Nakamura Y, Enomoto N, Fujisawa T, Imokawa S, et al. Acute exacerbation of interstitial pneumonia associated with collagen vascular diseases. Respir Med. 2009;103(6):846–53.

    PubMed  Google Scholar 

  24. Dallari R, Foglia M, Paci M, Cavazza A. Acute exacerbation of idiopathic pulmonary fibrosis. Eur Respir J. 2004;23(5):792.

    PubMed  CAS  Google Scholar 

  25. Judge EP, Fabre A, Adamali HI, Egan JJ. Acute exacerbations and pulmonary hypertension in advanced idiopathic pulmonary fibrosis. Eur Respir J. 2012;40:93–100.

    PubMed  Google Scholar 

  26. Kondoh Y, Taniguchi H, Kitaichi M, Yokoi T, Johkoh T, Oishi T, et al. Acute exacerbation of interstitial pneumonia following surgical lung biopsy. Respir Med. 2006;100(10):1753–9.

    PubMed  Google Scholar 

  27. Hoshikawa Y, Kondo T. [Perioperative lung injury: acute exacerbation of idiopathic pulmonary fibrosis and acute interstitial pneumonia after pulmonary resection]. Nihon Geka Gakkai Zasshi. 2004;105(12):757–62.

    PubMed  Google Scholar 

  28. Enomoto T, Kawamoto M, Kunugi S, Hiramatsu K, Sakakibara K, Usuki J, et al. [Clinicopathological analysis of patients with idiopathic pulmonary fibrosis which became acutely exacerbated after video-assisted thoracoscopic surgical lung biopsy]. Nihon Kokyuki Gakkai Zasshi. 2002;40(10):806–11.

    PubMed  Google Scholar 

  29. Hiwatari N, Shimura S, Takishima T, Shirato K. Bronchoalveolar lavage as a possible cause of acute exacerbation in idiopathic pulmonary fibrosis patients. Tohoku J Exp Med. 1994;174(4):379–86.

    PubMed  CAS  Google Scholar 

  30. Sakamoto K, Taniguchi H, Kondoh Y, Wakai K, Kimura T, Kataoka K, et al. Acute exacerbation of IPF following diagnostic bronchoalveolar lavage procedures. Respir Med. 2012;106(3):436–42.

    PubMed  Google Scholar 

  31. Fujimoto K, Taniguchi H, Johkoh T, Kondoh Y, Ichikado K, Sumikawa H, et al. Acute exacerbation of idiopathic pulmonary fibrosis: high-resolution CT scores predict mortality. Eur Radiol. 2012;22(1):83–92.

    PubMed  Google Scholar 

  32. Horita N, Akahane M, Okada Y, Kobayashi Y, Arai T, Amano I, et al. Tacrolimus and steroid treatment for acute exacerbation of idiopathic pulmonary fibrosis. Intern Med. 2011;50(3):189–95.

    PubMed  CAS  Google Scholar 

  33. Inase N, Sawada M, Ohtani Y, Miyake S, Isogai S, Sakashita H, et al. Cyclosporin A followed by the treatment of acute exacerbation of idiopathic pulmonary fibrosis with corticosteroid. Intern Med. 2003;42(7):565–70.

    PubMed  CAS  Google Scholar 

  34. Blivet S, Philit F, Sab JM, Langevin B, Paret M, Guérin C, et al. Outcome of patients with idiopathic pulmonary fibrosis admitted to the ICU for respiratory failure. Chest. 2001;120(1):209–12.

    PubMed  CAS  Google Scholar 

  35. Fumeaux T, Rothmeier C, Jolliet P. Outcome of mechanical ventilation for acute respiratory failure in patients with pulmonary fibrosis. Intensive Care Med. 2001;27(12):1868–74.

    PubMed  CAS  Google Scholar 

  36. Mallick S. Outcome of patients with idiopathic pulmonary fibrosis (IPF) ventilated in intensive care unit. Respir Med. 2008;102(10):1355–9.

    PubMed  Google Scholar 

  37. Mollica C, Paone G, Conti V, Ceccarelli D, Schmid G, Mattia P, et al. Mechanical ventilation in patients with end-stage idiopathic pulmonary fibrosis. Respiration. 2010;79(3):209–15.

    PubMed  Google Scholar 

  38. Mejía M, Carrillo G, Rojas-Serrano J, Estrada A, Suárez T, Alonso D, et al. Idiopathic pulmonary fibrosis and emphysema: decreased survival associated with severe pulmonary arterial hypertension. Chest. 2009;136(1):10–5.

    PubMed  Google Scholar 

  39. Cottin V, Nunes H, Brillet PY, Delaval P, Devouassoux G, Tillie-Leblond I, et al. Combined pulmonary fibrosis and emphysema: a distinct underrecognised entity. Eur Respir J. 2005;26(4):586–93.

    PubMed  CAS  Google Scholar 

  40. Brillet PY, Cottin V, Letoumelin P, Landino F, Brauner MW, Valeyre D, et al. [Combined apical emphysema and basal fibrosis syndrome (emphysema/fibrosis syndrome): CT imaging features and pulmonary function tests]. J Radiol. 2009;90(1 Pt 1):43–51.

    PubMed  CAS  Google Scholar 

  41. Grubstein A, Bendayan D, Schactman I, Cohen M, Shitrit D, Kramer MR. Concomitant upper-lobe bullous emphysema, lower-lobe interstitial fibrosis and pulmonary hypertension in heavy smokers: report of eight cases and review of the literature. Respir Med. 2005;99(8):948–54.

    PubMed  Google Scholar 

  42. Cottin V, Le Pavec J, Prévot G, Mal H, Humbert M, Simonneau G, et al. Pulmonary hypertension in patients with combined pulmonary fibrosis and emphysema syndrome. Eur Respir J. 2010;35(1):105–11.

    PubMed  CAS  Google Scholar 

  43. Cottin V, Cordier JF. The syndrome of combined pulmonary fibrosis and emphysema. Chest. 2009;136(1):1–2.

    PubMed  Google Scholar 

  44. Yousem SA. Respiratory bronchiolitis-associated interstitial lung disease with fibrosis is a lesion distinct from fibrotic nonspecific interstitial pneumonia: a proposal. Mod Pathol. 2006;19(11):1474–9.

    PubMed  Google Scholar 

  45. Katzenstein AL, Mukhopadhyay S, Zanardi C, Dexter E. Clinically occult interstitial fibrosis in smokers: classification and significance of a surprisingly common finding in lobectomy specimens. Hum Pathol. 2010;41(3):316–25.

    PubMed  Google Scholar 

  46. Rogliani P, Mura M, Mattia P, Ferlosio A, Farinelli G, Mariotta S, et al. HRCT and histopathological evaluation of fibrosis and tissue destruction in IPF associated with pulmonary emphysema. Respir Med. 2008;102(12):1753–61.

    PubMed  Google Scholar 

  47. Schmidt SL, Nambiar AM, Tayob N, Sundaram B, Han MK, Gross BH, et al. Pulmonary function measures predict mortality differently in IPF versus combined pulmonary fibrosis and emphysema. Eur Respir J. 2011;38(1):176–83.

    PubMed  CAS  Google Scholar 

  48. Simonneau G, Robbins IM, Beghetti M, Channick RN, Delcroix M, Denton CP, et al. Updated clinical classification of pulmonary hypertension. J Am Coll Cardiol. 2009;54(1 Suppl): S43–54.

    PubMed  Google Scholar 

  49. Lettieri CJ, Nathan SD, Barnett SD, Ahmad S, Shorr AF. Prevalence and outcomes of pulmonary arterial hypertension in advanced idiopathic pulmonary fibrosis. Chest. 2006;129(3):746–52.

    PubMed  Google Scholar 

  50. Nadrous HF, Pellikka PA, Krowka MJ, Swanson KL, Chaowalit N, Decker PA, et al. Pulmonary hypertension in patients with idiopathic pulmonary fibrosis. Chest. 2005;128(4):2393–9.

    PubMed  Google Scholar 

  51. Zisman DA, Karlamangla AS, Kawut SM, Shlobin OA, Saggar R, Ross DJ, et al. Validation of a method to screen for pulmonary hypertension in advanced idiopathic pulmonary fibrosis. Chest. 2008;133(3):640–5.

    PubMed  Google Scholar 

  52. Swigris JJ, Olson AL, Shlobin OA, Ahmad S, Brown KK, Nathan SD. Heart rate recovery after six-minute walk test predicts pulmonary hypertension in patients with idiopathic pulmonary fibrosis. Respirology. 2011;16(3):439–45.

    PubMed  Google Scholar 

  53. Zisman DA, Ross DJ, Belperio JA, Saggar R, Lynch JP, Ardehali A, et al. Prediction of pulmonary hypertension in idiopathic pulmonary fibrosis. Respir Med. 2007;101(10):2153–9.

    PubMed  Google Scholar 

  54. Zisman DA, Karlamangla AS, Ross DJ, Keane MP, Belperio JA, Saggar R, et al. High-resolution chest CT findings do not predict the presence of pulmonary hypertension in advanced idiopathic pulmonary fibrosis. Chest. 2007;132(3):773–9.

    PubMed  Google Scholar 

  55. Nathan SD, Shlobin OA, Ahmad S, Koch J, Barnett SD, Ad N, et al. Serial development of pulmonary hypertension in patients with idiopathic pulmonary fibrosis. Respiration. 2008;76(3):288–94.

    PubMed  CAS  Google Scholar 

  56. Papakosta D, Pitsiou G, Daniil Z, Dimadi M, Stagaki E, Rapti A, et al. Prevalence of pulmonary hypertension in patients with idiopathic pulmonary fibrosis: correlation with physiological parameters. Lung. 2011;189(5):391–9.

    PubMed  CAS  Google Scholar 

  57. Nathan SD, Shlobin OA, Ahmad S, Urbanek S, Barnett SD. Pulmonary hypertension and pulmonary function testing in idiopathic pulmonary fibrosis. Chest. 2007;131(3):657–63.

    PubMed  Google Scholar 

  58. Nathan SD, Shlobin OA, Barnett SD, Saggar R, Belperio JA, Ross DJ, et al. Right ventricular systolic pressure by echocardiography as a predictor of pulmonary hypertension in idiopathic pulmonary fibrosis. Respir Med. 2008;102(9):1305–10.

    PubMed  Google Scholar 

  59. Gagermeier J, Dauber J, Yousem S, Gibson K, Kaminski N. Abnormal vascular phenotypes in patients with idiopathic pulmonary fibrosis and secondary pulmonary hypertension. Chest. 2005;128(6 Suppl):601S.

    PubMed  Google Scholar 

  60. Farkas L, Gauldie J, Voelkel NF, Kolb M. Pulmonary hypertension and idiopathic pulmonary fibrosis: a tale of angiogenesis, apoptosis, and growth factors. Am J Respir Cell Mol Biol. 2011;45(1):1–15.

    PubMed  CAS  Google Scholar 

  61. Parra ER, David YR, da Costa LR, Ab’Saber A, Sousa R, Kairalla RA, et al. Heterogeneous remodeling of lung vessels in idiopathic pulmonary fibrosis. Lung. 2005;183(4):291–300.

    PubMed  Google Scholar 

  62. King TE, Behr J, Brown KK, du Bois RM, Lancaster L, de Andrade JA, et al. BUILD-1: a randomized placebo-controlled trial of bosentan in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 2008;177(1):75–81.

    PubMed  CAS  Google Scholar 

  63. King Jr TE, Brown KK, Raghu G, du Bois RM, Lynch DA, Martinez F, et al. BUILD-3: a randomized, controlled trial of bosentan in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 2011;184(1):92–9.

    PubMed  Google Scholar 

  64. Collard HR, Anstrom KJ, Schwarz MI, Zisman DA. Sildenafil improves walk distance in idiopathic pulmonary fibrosis. Chest. 2007;131(3):897–9.

    PubMed  Google Scholar 

  65. Ghofrani HA, Wiedemann R, Rose F, Schermuly RT, Olschewski H, Weissmann N, et al. Sildenafil for treatment of lung fibrosis and pulmonary hypertension: a randomised controlled trial. Lancet. 2002;360(9337):895–900.

    PubMed  CAS  Google Scholar 

  66. Olschewski H, Ghofrani HA, Walmrath D, Schermuly R, Temmesfeld-Wollbruck B, Grimminger F, et al. Inhaled prostacyclin and iloprost in severe pulmonary hypertension secondary to lung fibrosis. Am J Respir Crit Care Med. 1999;160(2):600–7.

    PubMed  CAS  Google Scholar 

  67. Saggar R, Shapiro SS, Ross DJ, Fishbein MC, Zisman DA, Lynch JP, et al. Treprostinil to reverse pulmonary hypertension associated with idiopathic pulmonary fibrosis as a bridge to single-lung transplantation. J Heart Lung Transplant. 2009;28(9):964–7.

    PubMed  Google Scholar 

  68. Raghu G, Yang ST, Spada C, Hayes J, Pellegrini CA. Sole treatment of acid gastroesophageal reflux in idiopathic pulmonary fibrosis: a case series. Chest. 2006;129(3):794–800.

    PubMed  Google Scholar 

  69. 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):1804–8.

    PubMed  CAS  Google Scholar 

  70. Sweet MP, Patti MG, Leard LE, Golden JA, Hays SR, Hoopes C, et al. Gastroesophageal reflux in patients with idiopathic pulmonary fibrosis referred for lung transplantation. J Thorac Cardiovasc Surg. 2007;133(4):1078–84.

    PubMed  Google Scholar 

  71. Jamieson JR, Stein HJ, DeMeester TR, Bonavina L, Schwizer W, Hinder RA, et al. Ambulatory 24-h esophageal pH monitoring: normal values, optimal thresholds, specificity, sensitivity, and reproducibility. Am J Gastroenterol. 1992;87(9):1102–11.

    PubMed  CAS  Google Scholar 

  72. Raghu G, Freudenberger TD, Yang S, Curtis JR, Spada C, Hayes J, et al. High prevalence of abnormal acid gastro-oesophageal reflux in idiopathic pulmonary fibrosis. Eur Respir J. 2006;27(1):136–42.

    PubMed  CAS  Google Scholar 

  73. el-Serag HB, Sonnenberg A. Comorbid occurrence of laryngeal or pulmonary disease with esophagitis in United States military veterans. Gastroenterology. 1997;113(3):755–60.

    PubMed  CAS  Google Scholar 

  74. Raiha I, Manner R, Hietanen E, Hartiala J, Sourander L. Radiographic pulmonary changes of gastro-oesophageal reflux disease in elderly patients. Age Ageing. 1992;21(4):250–5.

    PubMed  CAS  Google Scholar 

  75. D’Ovidio F, Singer LG, Hadjiliadis D, Pierre A, Waddell TK, de Perrot M, et al. Prevalence of gastroesophageal reflux in end-stage lung disease candidates for lung transplant. Ann Thorac Surg. 2005;80(4):1254–60.

    PubMed  Google Scholar 

  76. Lee JS, Ryu JH, Elicker BM, Lydell CP, Jones KD, Wolters PJ, et al. Gastroesophageal reflux therapy is associated with longer survival in patients with idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 2011;184(12):1390–4.

    PubMed  Google Scholar 

  77. Popper H, Juettner F, Pinter J. The gastric juice aspiration syndrome (Mendelson syndrome). Aspects of pathogenesis and treatment in the pig. Virchows Arch A Pathol Anat Histopathol. 1986;409(1):105–17.

    PubMed  CAS  Google Scholar 

  78. Raghu G. Idiopathic pulmonary fibrosis: increased survival with “gastroesophageal reflux therapy”: fact or fallacy? Am J Respir Crit Care Med. 2011;184(12):1330–2.

    PubMed  Google Scholar 

  79. Raghu G, Meyer KC. Silent gastro-oesophageal reflux and microaspiration in IPF: mounting evidence for anti-reflux therapy? Eur Respir J. 2012;39(2):242–5.

    PubMed  CAS  Google Scholar 

  80. Linden PA, Gilbert RJ, Yeap BY, Boyle K, Deykin A, Jaklitsch MT, et al. Laparoscopic fundoplication in patients with end-stage lung disease awaiting transplantation. J Thorac Cardiovasc Surg. 2006;131(2):438–46.

    PubMed  Google Scholar 

  81. Davis Jr RD, Lau CL, Eubanks S, Messier RH, Hadjiliadis D, Steele MP, et al. Improved lung allograft function after fundoplication in patients with gastroesophageal reflux disease undergoing lung transplantation. J Thorac Cardiovasc Surg. 2003;125(3):533–42.

    PubMed  Google Scholar 

  82. Cantu 3rd E, Appel 3rd JZ, Hartwig MG, Woreta H, Green C, Messier R, et al. J. Maxwell Chamberlain Memorial Paper. Early fundoplication prevents chronic allograft dysfunction in patients with gastroesophageal reflux disease. Ann Thorac Surg. 2004;78(4):1142–51. discussion −51.

    PubMed  Google Scholar 

  83. Hubbard RB, Smith C, Le Jeune I, Gribbin J, Fogarty AW. The association between idiopathic pulmonary fibrosis and vascular disease: a population-based study. Am J Respir Crit Care Med. 2008;178(12):1257–61.

    PubMed  Google Scholar 

  84. Sode BF, Dahl M, Nielsen SF, Nordestgaard BG. Venous thromboembolism and risk of idiopathic interstitial pneumonia: a nationwide study. Am J Respir Crit Care Med. 2010;181(10):1085–92.

    PubMed  Google Scholar 

  85. Sprunger DB, Olson AL, Huie TJ, Fernandez-Perez ER, Fischer A, Solomon JJ, et al. Pulmonary fibrosis is associated with an elevated risk of thromboembolic disease. Eur Respir J. 2012;39(1):125–32.

    PubMed  CAS  Google Scholar 

  86. Saydain G, Islam A, Afessa B, Ryu JH, Scott JP, Peters SG. Outcome of patients with idiopathic pulmonary fibrosis admitted to the intensive care unit. Am J Respir Crit Care Med. 2002;166(6):839–42.

    PubMed  Google Scholar 

  87. Wygrecka M, Jablonska E, Guenther A, Preissner KT, Markart P. Current view on alveolar coagulation and fibrinolysis in acute inflammatory and chronic interstitial lung diseases. Thromb Haemost. 2008;99(3):494–501.

    PubMed  CAS  Google Scholar 

  88. Chambers RC, Leoni P, Blanc-Brude OP, Wembridge DE, Laurent GJ. Thrombin is a potent inducer of connective tissue growth factor production via proteolytic activation of protease-activated receptor-1. J Biol Chem. 2000;275(45):35584–91.

    PubMed  CAS  Google Scholar 

  89. Rizkallah J, Man SF, Sin DD. Prevalence of pulmonary embolism in acute exacerbations of COPD: a systematic review and metaanalysis. Chest. 2009;135(3):786–93.

    PubMed  Google Scholar 

  90. Tillie-Leblond I, Marquette CH, Perez T, Scherpereel A, Zanetti C, Tonnel AB, et al. Pulmonary embolism in patients with unexplained exacerbation of chronic obstructive pulmonary disease: prevalence and risk factors. Ann Intern Med. 2006;144(6):390–6.

    PubMed  Google Scholar 

  91. Noth I, Anstrom KJ, Calvert SB, de Andrade J, Flaherty KR, Glazer C, et al. A placebo-controlled randomized trial of warfarin in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 2012;186:88–95.

    PubMed  CAS  Google Scholar 

  92. Izbicki G, Ben-Dor I, Shitrit D, Bendayan D, Aldrich TK, Kornowski R, et al. The prevalence of coronary artery disease in end-stage pulmonary disease: is pulmonary fibrosis a risk factor? Respir Med. 2009;103(9):1346–9.

    PubMed  Google Scholar 

  93. Kizer JR, Zisman DA, Blumenthal NP, Kotloff RM, Kimmel SE, Strieter RM, et al. Association between pulmonary fibrosis and coronary artery disease. Arch Intern Med. 2004;164(5):551–6.

    PubMed  Google Scholar 

  94. Nathan SD, Basavaraj A, Reichner C, Shlobin OA, Ahmad S, Kiernan J, et al. Prevalence and impact of coronary artery disease in idiopathic pulmonary fibrosis. Respir Med. 2010;104(7): 1035–41.

    PubMed  Google Scholar 

  95. Ponnuswamy A, Manikandan R, Sabetpour A, Keeping IM, Finnerty JP. Association between ischaemic heart disease and interstitial lung disease: a case–control study. Respir Med. 2009;103(4):503–7.

    PubMed  CAS  Google Scholar 

  96. 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(3):277–84.

    PubMed  Google Scholar 

  97. Enomoto T, Usuki J, Azuma A, Nakagawa T, Kudoh S. Diabetes mellitus may increase risk for idiopathic pulmonary fibrosis. Chest. 2003;123(6):2007–11.

    PubMed  Google Scholar 

  98. Ross R. Atherosclerosis – an inflammatory disease. N Engl J Med [Research Support, US Gov’t, PHS Review]. 1999;340(2):115–26.

    CAS  Google Scholar 

  99. Kelly M, Kolb M, Bonniaud P, Gauldie J. Re-evaluation of fibrogenic cytokines in lung fibrosis. Curr Pharm Des [Review]. 2003;9(1):39–49.

    CAS  Google Scholar 

  100. Gifford AH, Matsuoka M, Ghoda LY, Homer RJ, Enelow RI. Chronic inflammation and lung fibrosis: pleotropic syndromes but limited distinct phenotypes. Mucosal Immunol. 2012;5:480–4.

    PubMed  CAS  Google Scholar 

  101. Nathan SD, Weir N, Shlobin OA, Urban BA, Curry CA, Basavaraj A, et al. The value of computed tomography scanning for the detection of coronary artery disease in patients with idiopathic pulmonary fibrosis. Respirology. 2011;16(3):481–6.

    PubMed  Google Scholar 

  102. Wells C, Mannino DM. Pulmonary fibrosis and lung cancer in the United States: analysis of the multiple cause of death mortality data, 1979 through 1991. South Med J. 1996;89(5):505–10.

    PubMed  CAS  Google Scholar 

  103. Matsushita H, Tanaka S, Saiki Y, Hara M, Nakata K, Tanimura S, et al. Lung cancer associated with usual interstitial pneumonia. Pathol Int. 1995;45(12):925–32.

    PubMed  CAS  Google Scholar 

  104. Kawai T, Yakumaru K, Suzuki M, Kageyama K. Diffuse interstitial pulmonary fibrosis and lung cancer. Acta Pathol Jpn. 1987;37(1):11–9.

    PubMed  CAS  Google Scholar 

  105. Le Jeune I, Gribbin J, West J, Smith C, Cullinan P, Hubbard R. The incidence of cancer in patients with idiopathic pulmonary fibrosis and sarcoidosis in the UK. Respir Med. 2007;101(12):2534–40.

    PubMed  Google Scholar 

  106. 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(1):5–8.

    PubMed  CAS  Google Scholar 

  107. Park J, Kim DS, Shim TS, Lim CM, Koh Y, Lee SD, et al. Lung cancer in patients with idiopathic pulmonary fibrosis. Eur Respir J. 2001;17(6):1216–9.

    PubMed  CAS  Google Scholar 

  108. Lee HJ, Im JG, Ahn JM, Yeon KM. Lung cancer in patients with idiopathic pulmonary fibrosis: CT findings. J Comp Assist Tomogr. 1996;20(6):979–82.

    CAS  Google Scholar 

  109. Watanabe A, Higami T, Ohori S, Koyanagi T, Nakashima S, Mawatari T. Is lung cancer resection indicated in patients with idiopathic pulmonary fibrosis? J Thorac Cardiovasc Surg. 2008;136(5):1357–63. 63.e1-2.

    PubMed  Google Scholar 

  110. Mizushima Y, Kobayashi M. Clinical characteristics of synchronous multiple lung cancer associated with idiopathic pulmonary fibrosis. A review of Japanese cases. Chest. 1995;108(5):1272–7.

    PubMed  CAS  Google Scholar 

  111. Hironaka M, Fukayama M. Pulmonary fibrosis and lung carcinoma: a comparative study of metaplastic epithelia in honeycombed areas of usual interstitial pneumonia with or without lung carcinoma. Pathol Int. 1999;49(12):1060–6.

    PubMed  CAS  Google Scholar 

  112. Goto T, Maeshima A, Akanabe K, Oyamada Y, Kato R. Acute exacerbation of idiopathic pulmonary fibrosis of microscopic usual interstitial pneumonia pattern after lung cancer surgery. Ann Thorac Cardiovasc Surg. 2011;17(6):573–6.

    PubMed  Google Scholar 

  113. Sakai S, Ono M, Nishio T, Kawarada Y, Nagashima A, Toyoshima S. Lung cancer associated with diffuse pulmonary fibrosis: CT-pathologic correlation. J Thorac Imaging. 2003;18(2): 67–71.

    PubMed  Google Scholar 

  114. Kishi K, Homma S, Kurosaki A, Motoi N, Yoshimura K. High-resolution computed tomography findings of lung cancer associated with idiopathic pulmonary fibrosis. J Comput Assist Tomogr. 2006;30(1):95–9.

    PubMed  Google Scholar 

  115. Jeon TY, Lee KS, Yi CA, Chung MP, Kwon OJ, Kim BT, et al. Incremental value of PET/CT over CT for mediastinal nodal staging of non-small cell lung cancer: comparison between patients with and without idiopathic pulmonary fibrosis. AJR Am J Roentgenol. 2010;195(2):370–6.

    PubMed  Google Scholar 

  116. Fujimoto T, Okazaki T, Matsukura T, Hanawa T, Yamashita N, Nishimura K, et al. Operation for lung cancer in patients with idiopathic pulmonary fibrosis: surgical contraindication? Ann Thorac Surg. 2003;76(5):1674–8. discussion 9.

    PubMed  Google Scholar 

  117. Kumar P, Goldstraw P, Yamada K, Nicholson AG, Wells AU, Hansell DM, et al. Pulmonary fibrosis and lung cancer: risk and benefit analysis of pulmonary resection. J Thorac Cardiovasc Surg. 2003;125(6):1321–7.

    PubMed  CAS  Google Scholar 

  118. Saito Y, Kawai Y, Takahashi N, Ikeya T, Murai K, Kawabata Y, et al. Survival after surgery for pathologic stage IA non-small cell lung cancer associated with idiopathic pulmonary fibrosis. Ann Thorac Surg. 2011;92(5):1812–7.

    PubMed  Google Scholar 

  119. Kawasaki H, Nagai K, Yoshida J, Nishimura M, Nishiwaki Y. Postoperative morbidity, mortality, and survival in lung cancer associated with idiopathic pulmonary fibrosis. J Surg Oncol. 2002;81(1):33–7.

    PubMed  Google Scholar 

  120. Kushibe K, Kawaguchi T, Takahama M, Kimura M, Tojo T, Taniguchi S. Operative indications for lung cancer with idiopathic pulmonary fibrosis. Thorac Cardiovasc Surg. 2007;55(8):505–8.

    PubMed  CAS  Google Scholar 

  121. Vancheri C, Failla M, Crimi N, Raghu G. Idiopathic pulmonary fibrosis: a disease with similarities and links to cancer biology. Eur Respir J. 2010;35(3):496–504.

    PubMed  CAS  Google Scholar 

  122. Oshikawa K, Sugiyama Y. Serum anti-p53 autoantibodies from patients with idiopathic pulmonary fibrosis associated with lung cancer. Respir Med. 2000;94(11):1085–91.

    PubMed  CAS  Google Scholar 

  123. Ohwada H, Hayashi Y, Seki M. An experimental study on carcinogenesis related to localized fibrosis in the lung. Gann. 1980;71(3):285–91.

    PubMed  CAS  Google Scholar 

  124. Fernández Pérez ER, Daniels CE, Schroeder DR, St Sauver J, Hartman TE, Bartholmai BJ, et al. Incidence, prevalence, and clinical course of idiopathic pulmonary fibrosis: a population-based study. Chest. 2010;137(1):129–37.

    PubMed  Google Scholar 

  125. Martinez FJ, Safrin S, Weycker D, Starko KM, Bradford WZ, King Jr TE, et al. The clinical course of patients with idiopathic pulmonary fibrosis. Ann Intern Med. 2005;142(12 Pt 1): 963–7.

    PubMed  Google Scholar 

  126. Borgsteede SD, Deliens L, Francke AL, Stalman WA, Willems DL, van Eijk JT, et al. Defining the patient population: one of the problems for palliative care research. Palliat Med. 2006;20(2):63–8.

    PubMed  Google Scholar 

  127. Billings JA. What is palliative care? J Palliat Med. 1998;1(1):73–81.

    PubMed  CAS  Google Scholar 

  128. Lanken PN, Terry PB, Delisser HM, Fahy BF, Hansen-Flaschen J, Heffner JE, et al. An official American Thoracic Society clinical policy statement: palliative care for patients with respiratory diseases and critical illnesses. Am J Respir Crit Care Med. 2008;177(8):912–27.

    PubMed  Google Scholar 

  129. De Vries J, Kessels BL, Drent M. Quality of life of idiopathic pulmonary fibrosis patients. Eur Respir J. 2001;17(5):954–61.

    PubMed  Google Scholar 

  130. Tomioka H, Imanaka K, Hashimoto K, Iwasaki H. Health-related quality of life in patients with idiopathic pulmonary fibrosis – cross-sectional and longitudinal study. Intern Med. 2007;46(18):1533–42.

    PubMed  Google Scholar 

  131. 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(7):588–94.

    PubMed  CAS  Google Scholar 

  132. Chang JA, Curtis JR, Patrick DL, Raghu G. Assessment of health-related quality of life in patients with interstitial lung disease. Chest. 1999;116(5):1175–82.

    PubMed  CAS  Google Scholar 

  133. Jastrzebski D, Kozielski J, Banaś A, Cebula T, Gumola A, Ziora D, et al. Quality of life during one-year observation of patients with idiopathic pulmonary fibrosis awaiting lung transplantation. J Physiol Pharmacol. 2005;56 Suppl 4:99–105.

    PubMed  Google Scholar 

  134. 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(3):273–8.

    PubMed  Google Scholar 

  135. Nishiyama O, Taniguchi H, Kondoh Y, Kimura T, Ogawa T, Watanabe F, et al. Health-related quality of life in patients with idiopathic pulmonary fibrosis. What is the main contributing factor? Respir Med. 2005;99(4):408–14.

    PubMed  CAS  Google Scholar 

  136. Swigris JJ, Gould MK, Wilson SR. Health-related quality of life among patients with idiopathic pulmonary fibrosis. Chest. 2005;127(1):284–94.

    PubMed  Google Scholar 

  137. Lindell KO, Olshansky E, Song MK, Zullo TG, Gibson KF, Kaminski N, et al. Impact of a disease-management program on symptom burden and health-related quality of life in patients with idiopathic pulmonary fibrosis and their care partners. Heart Lung. 2010;39(4): 304–13.

    PubMed  Google Scholar 

  138. Ryerson CJ, Donesky D, Pantilat SZ, Collard HR. Dyspnea in idiopathic pulmonary fibrosis: a systematic review. J Pain Symptom Manage. 2012;43(4):771–82.

    PubMed  Google Scholar 

  139. Swigris JJ, Fairclough DL, Morrison M, Make B, Kozora E, Brown KK, et al. Benefits of pulmonary rehabilitation in idiopathic pulmonary fibrosis. Respir Care. 2011;56(6):783–9.

    PubMed  Google Scholar 

  140. Nishiyama O, Kondoh Y, Kimura T, Kato K, Kataoka K, Ogawa T, et al. Effects of pulmonary rehabilitation in patients with idiopathic pulmonary fibrosis. Respirology. 2008;13(3):394–9.

    PubMed  Google Scholar 

  141. 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):549–54.

    PubMed  CAS  Google Scholar 

  142. Ozalevli S, Karaali HK, Ilgin D, Ucan ES. Effect of home-based pulmonary rehabilitation in patients with idiopathic pulmonary fibrosis. Multidiscip Respir Med. 2010;5:31–7.

    PubMed  Google Scholar 

  143. Horton MR, Danoff SK, Lechtzin N. Thalidomide inhibits the intractable cough of idiopathic pulmonary fibrosis. Thorax [Clinical Trial, Phase II Letter Research Support, Non-US Gov’t]. 2008;63(8):749.

    CAS  Google Scholar 

  144. Hope-Gill BD, Hilldrup S, Davies C, Newton RP, Harrison NK. A study of the cough reflex in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 2003;168(8):995–1002.

    PubMed  Google Scholar 

  145. 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):128–30.

    PubMed  Google Scholar 

  146. Ryerson CJ, Berkeley J, Carrieri-Kohlman VL, Pantilat SZ, Landefeld CS, Collard HR. Depression and functional status are strongly associated with dyspnea in interstitial lung disease. Chest. 2011;139(3):609–16.

    PubMed  Google Scholar 

  147. Ley B, Collard HR, King Jr TE. Clinical course and prediction of survival in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 2011;183(4):431–40.

    PubMed  Google Scholar 

  148. Stern JB, Mal H, Groussard O, Brugière O, Marceau A, Jebrak G, et al. Prognosis of patients with advanced idiopathic pulmonary fibrosis requiring mechanical ventilation for acute respiratory failure. Chest. 2001;120(1):213–9.

    PubMed  CAS  Google Scholar 

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Authors and Affiliations

  1. Division of Pulmonary and Critical Care Medicine, Mayo Clinic, Gonda 18 South, 200 First Street, SW, Rochester, MN, 55902, USA

    Teng Moua M.D.

  2. Division of Pulmonary and Critical Care Medicine, Mayo Clinic, Rochester, MN, USA

    Jay H. Ryu M.D.

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  1. Teng Moua M.D.
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  2. Jay H. Ryu M.D.
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Correspondence to Teng Moua M.D. .

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  1. Dept. Internal Medicine, Sect. Allergy Pulmonary & Critical Care Med., University of Wisconsin School of Medicine & Public Health, Madison, Wisconsin, USA

    Keith C. Meyer

  2. Advanced Lung Disease and Lung Transplant Program, Inova Fairfax Hospital, Falls Church, Virginia, USA

    Steven D. Nathan

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Moua, T., Ryu, J.H. (2014). Recognizing and Treating Comorbidities of IPF. In: Meyer, K., Nathan, S. (eds) Idiopathic Pulmonary Fibrosis. Respiratory Medicine, vol 9. Humana Press, Totowa, NJ. https://doi.org/10.1007/978-1-62703-682-5_15

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