Idiopathic pulmonary fibrosis

References

Key articles

Collard HR, Moore BB, Flaherty KR, et al; Idiopathic Pulmonary Fibrosis Clinical Research Network Investigators. Acute exacerbations of idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 2007 Oct 1;176(7):636-43.Full text  Abstract

Raghu G, Remy-Jardin M, Richeldi L, et al; American Thoracic Society, European Respiratory Society, Japanese Respiratory Society, and Latin American Thoracic Society. Idiopathic pulmonary fibrosis (an update) and progressive pulmonary fibrosis in adults: an official ATS/ERS/JRS/ALAT clinical practice guideline. Am J Respir Crit Care Med. 2022 May 1;205(9):e18-e47.Full text  Abstract

Raghu G, Mageto YN, Lockhart D, et al. The accuracy of the clinical diagnosis of new-onset idiopathic pulmonary fibrosis and other interstitial lung disease: a prospective study. Chest. 1999 Nov;116(5):1168-74. Abstract

American Thoracic Society (ATS), European Respiratory Society (ERS). American Thoracic Society/European Respiratory Society international multidisciplinary consensus classification of the idiopathic interstitial pneumonias. Am J Respir Crit Care Med. 2002 Jan 15;165(2):277-304.Full text  Abstract

Raghu G, Rochwerg B, Zhang Y, et al. An official ATS/ERS/JRS/ALAT clinical practice guideline: treatment of idiopathic pulmonary fibrosis - an update of the 2011 clinical practice guideline. Am J Respir Crit Care Med. 2015 Jul 15;192(2):e3-19.Full text  Abstract

Richeldi L, du Bois RM, Raghu G, et al; INPULSIS Trial Investigators. Efficacy and safety of nintedanib in idiopathic pulmonary fibrosis. N Engl J Med. 2014 May 29;370(22):2071-82.Full text  Abstract

Martinez FJ, Safrin S, Weycker D, et al. The clinical course of patients with idiopathic pulmonary fibrosis. Ann Intern Med. 2005 Jun 21;142(12 Pt 1):963-7. Abstract

Kotloff RM, Thabut G. Lung transplantation. Am J Resp Crit Care Med. 2011 Jul 15;184(2):159-71. Abstract

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 Mar 15;183(6):788-824.Full text  Abstract

Reference articles

1. American Thoracic Society; European Respiratory Society. Idiopathic pulmonary fibrosis: diagnosis and treatment. International consensus statement. Am J Respir Crit Care Med. 2000 Feb;161(2 Pt 1):646-64.Full text  Abstract

2. Raghu G, Weycker D, Edelsberg J, et al. Incidence and prevalence of idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 2006 Oct 1;174(7):810-6.Full text  Abstract

3. Coultas DB, Zumwalt RE, Black WC, et al. The epidemiology of interstitial lung diseases. Am J Respir Crit Care Med. 1994 Oct;150(4):967-72. Abstract

4. 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 Apr;57(4):338-42. Abstract

5. Gribbin J, Hubbard RB, Le Jeune I, et al. Incidence and mortality of idiopathic pulmonary fibrosis and sarcoidosis in the UK. Thorax. 2006 Nov;61(11):980-5. Abstract

6. Navaratnam V, Fleming KM, West J, et al. The rising incidence of idiopathic pulmonary fibrosis in the U.K. Thorax. 2011 Jun;66(6):462-7. Abstract

7. Meltzer EB, Noble PW. Idiopathic pulmonary fibrosis. Orphanet J Rare Dis. 2008 Mar 26;3:8.Full text  Abstract

8. Marshall RP, Puddicombe A, Cookson WO, et al. Adult familial cryptogenic fibrosing alveolitis in the United Kingdom. Thorax. 2000 Feb;55(2):143-6. Abstract

9. Lee HL, Ryu JH, Wittmer MH, et al. Familial idiopathic pulmonary fibrosis: clinical features and outcome. Chest. 2005 Jun;127(6):2034-41. Abstract

10. Loyd JE. Pulmonary fibrosis in families. Am J Respir Cell Mol Biol. 2003 Sep;29(3 Suppl):S47-50. Abstract

11. Baumgartner KB, Samet JM, Stidley CA, et al. Cigarette smoking: a risk factor for idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 1997 Jan;155(1):242-8. Abstract

12. Hubbard R, Lewis S, Richards K, et al. Occupational exposure to metal or wood dust and aetiology of cryptogenic fibrosing alveolitis. Lancet. 1996 Feb 3;347(8997):284-9. Abstract

13. Iwai K, Mori T, Yamada N, et al. Idiopathic pulmonary fibrosis. Epidemiologic approaches to occupational exposure. Am J Respir Crit Care Med. 1994 Sep;150(3):670-5. Abstract

14. Baumgartner KB, Samet JM, Coultas DB, et al. Occupational and environmental risk factors for idiopathic pulmonary fibrosis: a multicenter case-control study. Am J Epidemiol. 2000 Aug 15;152(4):307-15.Full text  Abstract

15. Vannella KM, Moore BB. Viruses as co-factors for the initiation or exacerbation of lung fibrosis. Fibrogenesis Tissue Repair. 2008 Oct 13;1(1):2.Full text  Abstract

16. Taskar VS, Coultas DB. Is idiopathic pulmonary fibrosis an environmental disease? Proc Am Thorac Soc. 2006 Jun;3(4):293-8. Abstract

17. Steele MP, Speer MC, Loyd JE, et al. Clinical and pathologic features of familial interstitial pneumonia. Am J Respir Crit Care Med. 2005 Nov 1;172(9):1146-52.Full text  Abstract

18. Scott J, Johnston I, Britton J. What causes cryptogenic fibrosing alveolitis? A case-control study of environmental exposure to dust. BMJ. 1990 Nov 3;301(6759):1015-7.Full text  Abstract

19. Tobin RW, Pope CE 2nd, Pellegrini CA, et al. Increased prevalence of gastroesophageal reflux in patients with idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 1998 Dec;158(6):1804-8.Full text  Abstract

20. Raghu G, Freudenberger TD, Yang S, et al. High prevalence of abnormal acid gastro-oesophageal reflux in idiopathic pulmonary fibrosis. Eur Respir J. 2006 Jan;27(1):136-42.Full text  Abstract

21. Gribbin J, Hubbard R, Smith C. Role of diabetes mellitus and gastro-oesophageal reflux in the aetiology of idiopathic pulmonary fibrosis. Respir Med. 2009 Jun;103(6):927-31. Abstract

22. Lee JS, Ryu JH, Elicker BM , et al. Gastroesophageal reflux therapy is associated with longer survival time in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 2011 Dec 15;184(12):1390-4. Abstract

23. Ueda T, Ohta K, Suzuki N, et al. Idiopathic pulmonary fibrosis and high prevalence of serum antibodies to hepatitis C virus. Am Rev Respir Dis. 1992 Jul;146(1):266-8. Abstract

24. Irving WL, Day S, Johnston ID. Idiopathic pulmonary fibrosis and hepatitis C virus infection. Am Rev Respir Dis. 1993 Dec;148(6 Pt 1):1683-4. Abstract

25. Meliconi R, Andreone P, Fasano L, et al. Incidence of hepatitis C virus infection in Italian patients with idiopathic pulmonary fibrosis. Thorax. 1996 Mar;51(3):315-7. Abstract

26. Kuwano K, Nomoto Y, Kunitake R, et al. Detection of adenovirus E1A DNA in pulmonary fibrosis using nested polymerase chain reaction. Eur Respir J. 1997 Jul;10(7):1445-9.Full text  Abstract

27. Turner-Warwick M. In search of a cause of cryptogenic fibrosing alveolitis (CFA): one initiating factor or many? Thorax. 1998 Aug;53 Suppl 2:S3-9. Abstract

28. Yonemaru M, Kasuga I, Kusumoto H, et al. Elevation of antibodies to cytomegalovirus and other herpes viruses in pulmonary fibrosis. Eur Respir J. 1997 Sep;10(9):2040-5.Full text  Abstract

29. 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 Jun;41(6):2633-40.Full text  Abstract

30. Dworniczak S, Ziora D, Kapral M, et al. Human cytomegalovirus DNA level in patients with idiopathic pulmonary fibrosis. J Physiol Pharmacol. 2004 Sep;55 Suppl 3:67-75.Full text  Abstract

31. Vergnon JM, Vincent M, de The G, et al. Cryptogenic fibrosing alveolitis and Epstein-Barr virus: an association? Lancet. 1984 Oct 6;2(8406):768-71. Abstract

32. Stewart JP, Egan JJ, Ross AJ, et al. The detection of Epstein-Barr virus DNA in lung tissue from patients with idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 1999 Apr;159(4 Pt 1):1336-41.Full text  Abstract

33. Kelly BG, Lok SS, Hasleton PS, et al. A rearranged form of Epstein-Barr virus DNA is associated with idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 2002 Aug 15;166(4):510-3. Abstract

34. Wangoo A, Shaw RJ, Diss TC, et al. Cryptogenic fibrosing alveolitis: lack of association with Epstein-Barr virus infection. Thorax. 1997 Oct;52(10):888-91. Abstract

35. Zamo A, Poletti V, Reghellin D, et al. HHV-8 and EBV are not commonly found in idiopathic pulmonary fibrosis. Sarcoidosis Vasc Diffuse Lung Dis. 2005 Jun;22(2):123-8. Abstract

36. Kaufman JM, Cuvelier CA, Van der Straeten M. Mycoplasma pneumonia with fulminant evolution into diffuse interstitial fibrosis. Thorax. 1980 Feb;35(2):140-4. Abstract

37. Chastre J, Raghu G, Soler P, et al. Pulmonary fibrosis following pneumonia due to acute Legionnaires' disease. Clinical, ultrastructural, and immunofluorescent study. Chest. 1987 Jan;91(1):57-62. Abstract

38. Lawson WE, Loyd JE. The genetic approach in pulmonary fibrosis: can it provide clues to this complex disease? Proc Am Thorac Soc. 2006 Jun;3(4):345-9.Full text  Abstract

39. Thomas AQ, Lane K, Phillips J 3rd, 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 May 1;165(9):1322-8.Full text  Abstract

40. Armanios MY, Chen JJ, Cogan JD, et al. Telomerase mutations in families with idiopathic pulmonary fibrosis. N Engl J Med. 2007 Mar 29;356(13):1317-26.Full text  Abstract

41. Siebold MA, Wise AL, Speer MC, et al. A common MUC5B promotor polymorphism and pulmonary fibrosis. N Engl J Med. 2011 Apr 21;364(16):1503-12. Abstract

42. Peljto AL, Zhang Y, Fingerlin TE, et al. Association between the MUC5B promoter polymorphism and survival in patients with idiopathic pulmonary fibrosis. JAMA. 2013 Jun 5;309(21):2232-9. Abstract

43. Korthagen NM, van Moorsel CH, Kazemier KM, et al. IL1RN genetic variations and risk of IPF: a meta-analysis and mRNA expression study. Immunogenetics. 2012 May;64(5):371-7.Full text  Abstract

44. Streiter RM. Pathogenesis and natural history of usual interstitial pneumonia: the whole story or the last chapter of a long novel. Chest. 2005 Nov;128(5 suppl 1):526-32S. Abstract

45. Cronkhite JT, Xing C, Raghu G, et al. Telomere shortening in familial and sporadic pulmonary fibrosis. Am J Respir Crit Care Med. 2008 Oct 1;178(7):729-37. Abstract

46. Collard HR, Moore BB, Flaherty KR, et al; Idiopathic Pulmonary Fibrosis Clinical Research Network Investigators. Acute exacerbations of idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 2007 Oct 1;176(7):636-43.Full text  Abstract

47. Song JW, Hong SB, Lim CM, et al. Acute exacerbation of idiopathic pulmonary fibrosis: incidence, risk factors, and outcome. Eur Respir J. 2011 Feb;37(2):356-63. Abstract

48. Raghu G, Remy-Jardin M, Richeldi L, et al; American Thoracic Society, European Respiratory Society, Japanese Respiratory Society, and Latin American Thoracic Society. Idiopathic pulmonary fibrosis (an update) and progressive pulmonary fibrosis in adults: an official ATS/ERS/JRS/ALAT clinical practice guideline. Am J Respir Crit Care Med. 2022 May 1;205(9):e18-e47.Full text  Abstract

49. Raghu G, Remy-Jardin M, Myers JL, et al; American Thoracic Society, European Respiratory Society, Japanese Respiratory Society, and Latin American Thoracic Society. Diagnosis of idiopathic pulmonary fibrosis. An official ATS/ERS/JRS/ALAT clinical practice guideline. Am J Respir Crit Care Med. 2018 Sep 1;198(5):e44-68.Full text  Abstract

50. Raghu G, Mageto YN, Lockhart D, et al. The accuracy of the clinical diagnosis of new-onset idiopathic pulmonary fibrosis and other interstitial lung disease: a prospective study. Chest. 1999 Nov;116(5):1168-74. Abstract

51. Hunninghake GW, Lynch DA, Galvin JR, et al. Radiologic findings are strongly associated with a pathologic diagnosis of usual interstitial pneumonia. Chest. 2003 Oct;124(4):1215-23. Abstract

52. Johkoh T, Muller NL, Cartier Y, et al. Idiopathic interstitial pneumonias: diagnostic accuracy of thin-section CT in 129 patients. Radiology. 1999 May;211(2):555-60. Abstract

53. Hunninghake GW, Zimmerman MB, Schwartz DA, et al. Utility of a lung biopsy for the diagnosis of idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 2001 Jul 15;164(2):193-6.Full text  Abstract

54. Lynch DA, Sverzellati N, Travis WD, et al. Diagnostic criteria for idiopathic pulmonary fibrosis: a Fleischner Society White Paper. Lancet Respir Med. 2017 Nov 15;6(2):138-53.Full text  Abstract

55. Meyer KC, Raghu G, Baughman RP, et al. An official American Thoracic Society clinical practice guideline: the clinical utility of bronchoalveolar lavage cellular analysis in interstitial lung disease. Am J Respir Crit Care Med. 2012 May 1;185(9):1004-14. Abstract

56. Berbescu EA, Katzenstein AL, Snow JL, et al. Transbronchial biopsy in usual interstitial pneumonia. Chest. 2006 May;129(5):1126-31.Full text  Abstract

57. Katzenstein AL, Myers JL. Idiopathic pulmonary fibrosis: clinical relevance of pathologic classification. Am J Respir Crit Care Med. 1998 Apr;157(4 Pt 1):1301-15.Full text  Abstract

58. American Thoracic Society (ATS), European Respiratory Society (ERS). American Thoracic Society/European Respiratory Society international multidisciplinary consensus classification of the idiopathic interstitial pneumonias. Am J Respir Crit Care Med. 2002 Jan 15;165(2):277-304.Full text  Abstract

59. Travis WD, Costabel U, Hansell DM, et al; ATS/ERS Committee on Idiopathic Interstitial Pneumonias. 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 Sep 15;188(6):733-48.Full text  Abstract

60. Cordier JF. Cryptogenic organising pneumonia. Eur Respir J. 2006 Aug;28(2):422-46.Full text  Abstract

61. Chan-Yeung M, Müller NL. Cryptogenic fibrosing alveolitis. Lancet. 1997 Aug 30;350(9078):651-6. Abstract

62. Raghu G, Rochwerg B, Zhang Y, et al. An official ATS/ERS/JRS/ALAT clinical practice guideline: treatment of idiopathic pulmonary fibrosis - an update of the 2011 clinical practice guideline. Am J Respir Crit Care Med. 2015 Jul 15;192(2):e3-19.Full text  Abstract

63. Raghu G, Rochwerg B, Zhang Y, et al. An official ATS/ERS/JRS/ALAT clinical practice guideline: treatment of idiopathic pulmonary fibrosis - an update of the 2011 clinical practice guideline. Am J Respir Crit Care Med. 2015 Jul 15;192(2):e3-19.Full text  Abstract

64. Flaherty KR, Fell CD, Huggins JT, et al. Safety of nintedanib added to pirfenidone treatment for idiopathic pulmonary fibrosis. Eur Respir J. 2018 Aug 2;52(2):1800230. [Erratum in: Eur Respir J. 2018 Oct 4;52(4):1850230.]Full text  Abstract

65. 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 May 1;171(9):1040-7.Full text  Abstract

66. Taniguchi H, Ebina M, Kondoh Y, et al; Pirfenidone Clinical Study Group in Japan. Pirfenidone in idiopathic pulmonary fibrosis. Eur Respir J. 2010 Apr;35(4):821-9. Abstract

67. Noble PW, Albera C, Bradford WZ, et al; CAPACITY Study Group. Pirfenidone in patients with idiopathic pulmonary fibrosis (CAPACITY): two randomised trials. Lancet. 2011 May 21;377(9779):1760-9. Abstract

68. Spagnolo P, Del Giovane C, Luppi F, et al. Non-steroid agents for idiopathic pulmonary fibrosis. Cochrane Database Syst Rev. 2010 Sep 8;(9):CD003134. Abstract

69. King TE Jr, Bradford WZ, Castro-Bernardini S, et al; ASCEND Study Group. A phase 3 trial of pirfenidone in patients with idiopathic pulmonary fibrosis. N Engl J Med. 2014 May 29;370(22):2083-92. Abstract

70. Richeldi L, Costabel U, Selman M, et al. Efficacy of a tyrosine kinase inhibitor in idiopathic pulmonary fibrosis. N Engl J Med. 2011 Sep 22;365(12):1079-87.Full text  Abstract

71. Richeldi L, du Bois RM, Raghu G, et al; INPULSIS Trial Investigators. Efficacy and safety of nintedanib in idiopathic pulmonary fibrosis. N Engl J Med. 2014 May 29;370(22):2071-82.Full text  Abstract

72. Costabel U, Inoue Y, Richeldi L, et al. Efficacy of nintedanib in idiopathic pulmonary fibrosis across prespecified subgroups in INPULSIS. Am J Respir Crit Care Med. 2016 Jan 15;193(2):178-85.Full text  Abstract

73. Crestani B, Huggins JT, Kaye M, et al. Long-term safety and tolerability of nintedanib in patients with idiopathic pulmonary fibrosis: results from the open-label extension study, INPULSIS-ON. Lancet Respir Med. 2018 Sep 14;7(1):60-8. Abstract

74. Kolb M, Raghu G, Wells AU, et al. Nintedanib plus sildenafil in patients with idiopathic pulmonary fibrosis. N Engl J Med. 2018 Sep 15;379(18):1722-31.Full text  Abstract

75. Richeldi L, Davies HR, Ferrara G, et al. Corticosteroids for idiopathic pulmonary fibrosis. Cochrane Database Syst Rev. 2003 Jul 21;(3):CD002880. Abstract

76. King TE Jr, Albera C, Bradford WZ, et al. Effect of interferon gamma-1b on survival in patients with idiopathic pulmonary fibrosis (INSPIRE): a multicentre, randomised, placebo-controlled trial. Lancet. 2009 Jul 18;374(9685):222-8. Abstract

77. Daniels CE, Lasky JA, Limper AH, et al. Imatinib treatment for idiopathic pulmonary fibrosis: randomized placebo-controlled trial results. Am J Respir Crit Care Med. 2010 Mar 15;181(6):604-10. Abstract

78. King TE, Brown KK, Raghu G. BUILD-3: a randomized, controlled trial of bosentan in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 2011 Jul 1;184(1):92-9. Abstract

79. Raghu G, Behr J, Brown KK, et al; ARTEMIS-IPF Investigators. Treatment of idiopathic pulmonary fibrosis with ambrisentan: a parallel, randomized trial. Ann Intern Med. 2013 May 7;158(9):641-9. Abstract

80. Raghu G, Million-Rousseau R, Morganti A, et al; MUSIC Study Group. Macitentan for the treatment of idiopathic pulmonary fibrosis: the randomised controlled MUSIC trial. Eur Respir J. 2013 Dec;42(6):1622-32. Abstract

81. 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 Mar 15;183(6):788-824.Full text  Abstract

82. Raghu G, Depaso WJ, Cain K, et al. Azathioprine combined with prednisone in the treatment of idiopathic pulmonary fibrosis: a prospective double-blind, randomized, placebo-controlled clinical trial. Am Rev Respir Dis. 1991 Aug;144(2):291-6. Abstract

83. Demedts M, Behr J, Buhl R, et al; IFIGENIA Study Group. High-dose acetylcysteine in idiopathic pulmonary fibrosis. N Engl J Med. 2005 Nov 24;353(21):2229-42.Full text  Abstract

84. Raghu G, Anstrom KJ, King TE Jr, et al; Idiopathic Pulmonary Fibrosis Clinical Research Network. Prednisone, azathioprine, and N-acetylcysteine for pulmonary fibrosis. N Engl J Med. 2012 May 24;366(21):1968-77.Full text  Abstract

85. Martinez FJ, de Andrade JA, Anstrom KJ, et al; Idiopathic Pulmonary Fibrosis Clinical Research Network. Randomized trial of acetylcysteine in idiopathic pulmonary fibrosis. N Engl J Med. 2014 May 29;370(22):2093-101. Abstract

86. Fujimoto H, Gabazza EC, Hataji O, et al. Thrombin-activatable fibrinolysis inhibitor and protein C inhibitor in interstitial lung disease. Am J Respir Crit Care Med. 2003 Jun 15;167(12):1687-94.Full text  Abstract

87. Kubo H, Nakayama K, Yanai M, et al. Anticoagulant therapy for idiopathic pulmonary fibrosis. Chest. 2005 Sep;128(3):1475-82. Abstract

88. Noth I, Anstrom KJ, Calvert SB, et al. A placebo-controlled randomized trial of warfarin in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 2012 Jul 1;186(1):88-95. Abstract

89. Dowman L, Hill CJ, Holland AE. Pulmonary rehabilitation for interstitial lung disease. Cochrane Database Syst Rev. 2014 Oct 6;(10):CD006322.Full text  Abstract

90. Jacobs SS, Krishnan JA, Lederer DJ, et al. Home oxygen therapy for adults with chronic lung disease. An official American Thoracic Society clinical practice guideline. Am J Respir Crit Care Med. 2020 Nov 15;202(10):e121-41.Full text  Abstract

91. Sharp C, Adamali H, Millar AB. Ambulatory and short-burst oxygen for interstitial lung disease. Cochrane Database Syst Rev. 2016 Jul 6;(7):CD011716.Full text  Abstract

92. Barnes H, McDonald J, Smallwood N, et al. Opioids for the palliation of refractory breathlessness in adults with advanced disease and terminal illness. Cochrane Database Syst Rev. 2016 Mar 31;(3):CD011008.Full text  Abstract

93. Bajwah S, Ross JR, Wells AU, et al. Palliative care for patients with advanced fibrotic lung disease: a randomised controlled phase II and feasibility trial of a community case conference intervention. Thorax. 2015 Sep;70(9):830-9. Abstract

94. Martinez FJ, Safrin S, Weycker D, et al. The clinical course of patients with idiopathic pulmonary fibrosis. Ann Intern Med. 2005 Jun 21;142(12 Pt 1):963-7. Abstract

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

96. Kotloff RM, Thabut G. Lung transplantation. Am J Resp Crit Care Med. 2011 Jul 15;184(2):159-71. Abstract

97. Orens JB, Estenne M, Arcasoy S, et al. International guidelines for the selection of lung transplant candidates: 2006 update - a consensus report from the Pulmonary Scientific Council of the International Society for Heart and Lung Transplantation. J Heart Lung Transplant. 2006 Jul;25(7):745-55. Abstract

98. Thabut G, Mal H, Castier Y, et al. Survival benefit of lung transplantation for patients with idiopathic pulmonary fibrosis. J Thorac Cardiovasc Surg. 2003 Aug;126(2):469-75. Abstract

99. Waxman A, Restrepo-Jaramillo R, Thenappan T, et al. Inhaled treprostinil in pulmonary hypertension due to interstitial lung disease. N Engl J Med. 2021 Jan 28;384(4):325-34.Full text  Abstract

100. ClinicalTrials.gov. Safety and tolerability study in subjects with idiopathic pulmonary fibrosis (IPF). Jul 2015 [internet publication].Full text

101. Raghu G, van den Blink B, Hamblin MJ, et al. Effect of recombinant human pentraxin 2 vs placebo on change in forced vital capacity in patients with idiopathic pulmonary fibrosis: a randomized clinical trial. JAMA. 2018 Jun 12;319(22):2299-307.Full text  Abstract

102. Raghu G, Rochwerg B, Zhang Y, et al. An official ATS/ERS/JRS/ALAT clinical practice guideline: treatment of idiopathic pulmonary fibrosis - an update of the 2011 clinical practice guideline. Am J Respir Crit Care Med. 2015 Jul 15;192(2):e3-19.Full text  Abstract

103. Jacobs SS, Krishnan JA, Lederer DJ, et al. Home oxygen therapy for adults with chronic lung disease. An official American Thoracic Society clinical practice guideline. Am J Respir Crit Care Med. 2020 Nov 15;202(10):e121-41.Full text  Abstract

104. Kim DS, Collard HR, King TE Jr. Classification and natural history of the idiopathic interstitial pneumonias. Proc Am Thorac Soc. 2006 Jun;3(4):285-92.Full text  Abstract

105. Ley B, Ryerson CJ, Vittinghoff E, et al. A multidimensional index and staging system for idiopathic pulmonary fibrosis. Ann Intern Med. 2012 May 15;156(10):684-91. Abstract

106. Ryerson CJ, Vittinghoff E, Ley B, et al. Predicting survival across chronic interstitial lung disease: the ILD-GAP model. Chest. 2014 Apr;145(4):723-8. Abstract

107. Flaherty KR, Thwaite EL, Kazerooni EA, et al. Radiological versus histological diagnosis in UIP and NSIP: survival implications. Thorax. 2003 Feb;58(2):143-8.Full text  Abstract

108. Collard HR, King TE Jr, Bartelson BB, et al. Changes in clinical and physiologic variables predict survival in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 2003 Sep 1;168(5):538-42.Full text  Abstract

109. 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 Sep 1;168(5):531-7.Full text  Abstract

110. 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 Sep 1;168(5):543-8.Full text  Abstract

111. King TE Jr, Tooze JA, Schwarz MI, et al. Predicting survival in idiopathic pulmonary fibrosis: scoring system and survival model. Am J Respir Crit Care Med. 2001 Oct 1;164(7):1171-81.Full text  Abstract

112. Antoniou KM, Hansell DM, Rubens MB, et al. Idiopathic pulmonary fibrosis: outcome in relation to smoking status. Am J Resp Crit Care Med. 2008 Jan 15;177(2):190-4. Abstract

113. 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 Apr;35(4):830-6. Abstract

114. 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 Sep;136(3):841-8.Full text  Abstract

115. 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 Nov 1;168(9):1084-90.Full text  Abstract

116. Song JW, Do KH, Jang SJ, et al. Blood biomarkers MMP-7 and SP-A: predictors of outcome in idiopathic pulmonary fibrosis. Chest. 2013 May;143(5):1422-9. Abstract

117. Nathan SD, Reffett T, Brown AW, et al. The red cell distribution width as a prognostic indicator in idiopathic pulmonary fibrosis. Chest. 2013 Jun;143(6):1692-8. Abstract

118. Kahloon RA, Xue J, Bhargava A, et al. Patients with idiopathic pulmonary fibrosis with antibodies to heat shock protein 70 have poor prognoses. Am J Respir Crit Care Med. 2013 Apr 1;187(7):768-75. Abstract

119. Nadrous HR, Pellikka PA, Krowka MJ, et al. Pulmonary hypertension in patients with idiopathic pulmonary fibrosis. Chest. 2005 Oct;128(4):2393-9. Abstract

120. Gagermeier J, Dauber J, Yousem S, et al. Abnormal vascular phenotypes in patients with idiopathic pulmonary fibrosis and secondary pulmonary hypertension. Chest. 2005 Dec;128(6 suppl):601S. Abstract

121. Lettieri CJ, Nathan SD, Barnett SD, et al. Prevalence and outcomes of pulmonary arterial hypertension in advanced idiopathic pulmonary fibrosis. Chest. 2006 Mar;129(3):746-52. Abstract

122. Ryu JH, Krowka MJ, Swanson KL, et al. Pulmonary hypertension in patients with interstitial lung diseases. Mayo Clin Proc. 2007 Mar;82(3):342-50. Abstract

123. 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 Mar 15;183(6):788-824.Full text  Abstract

124. Harris JM, Johnston ID, Rudd R, et al. Cryptogenic fibrosing alveolitis and lung cancer: the BTS study. Thorax. 2010 Jan;65(1):70-6. Abstract

125. Daniels CE, Jett JR. Does interstitial lung disease predispose to lung cancer? Curr Opin Pulm Med. 2005 Sep;11(5):431-7. Abstract

126. Panos RJ, Mortenson R, Niccoli SA, et al. Clinical deterioration in patients with idiopathic pulmonary fibrosis. Causes and assessment. Am J Med. 1990 Apr;88(4):396-404. Abstract

127. Hubbard RB, Smith C, Le Jeune I, et al. The association between idiopathic pulmonary fibrosis and vascular disease: a population-based study. Am J Respir Crit Care Med. 2008 Dec 15;178(12):1257-61. Abstract

128. Nathan SD, Basavaraj A, Reichner C, et al. Prevalence and impact of coronary artery disease in idiopathic pulmonary fibrosis. Respir Med. 2010 Jul;104(7):1035-41. Abstract

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