Idiopathic Pulmonary Fibrosis (IPF) Questions & Answers

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2. Frankel SK, Schwarz MI. Update in idiopathic pulmonary fibrosis. Curr Opin Pulm Med. 2009 Sep. 15(5):463-9. [QxMD MEDLINE Link].

3. [Guideline] Raghu G, Remy-Jardin M, Myers JL, et al. 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-e68. [QxMD MEDLINE Link].

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7. Flaherty KR, Andrei AC, Murray S, et al. Idiopathic pulmonary fibrosis: prognostic value of changes in physiology and six-minute-walk test. Am J Respir Crit Care Med. 2006 Oct 1. 174(7):803-9. [QxMD MEDLINE Link]. [Full Text].

8. Misumi S, Lynch DA. Idiopathic pulmonary fibrosis/usual interstitial pneumonia: imaging diagnosis, spectrum of abnormalities, and temporal progression. Proc Am Thorac Soc. 2006 Jun. 3(4):307-14. [QxMD MEDLINE Link].

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10. American Thoracic Society/European Respiratory Society International Multidisciplinary Consensus Classification of the Idiopathic Interstitial Pneumonias. This joint statement of the American Thoracic Society (ATS), and the European Respiratory Society (ERS) was adopted by the ATS board of directors, June 2001 and by the ERS Executive Committee, June 2001. Am J Respir Crit Care Med. 2002 Jan 15. 165(2):277-304. [QxMD MEDLINE Link].

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12. 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. [QxMD MEDLINE Link]. [Full Text].

13. Visscher DW, Myers JL. Histologic spectrum of idiopathic interstitial pneumonias. Proc Am Thorac Soc. 2006 Jun. 3(4):322-9. [QxMD MEDLINE Link].

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16. Chen H, Chen Q, Jiang CM, Shi GY, Sui BW, Zhang W, et al. Triptolide suppresses paraquat induced idiopathic pulmonary fibrosis by inhibiting TGFB1-dependent epithelial mesenchymal transition. Toxicol Lett. 2017 Nov 28. 284:1-9. [QxMD MEDLINE Link].

17. Giménez A, Duch P, Puig M, Gabasa M, Xaubet A, Alcaraz J. Dysregulated Collagen Homeostasis by Matrix Stiffening and TGF-β1 in Fibroblasts from Idiopathic Pulmonary Fibrosis Patients: Role of FAK/Akt. Int J Mol Sci. 2017 Nov 16. 18 (11):[QxMD MEDLINE Link].

18. Thannickal VJ, Horowitz JC. Evolving concepts of apoptosis in idiopathic pulmonary fibrosis. Proc Am Thorac Soc. 2006 Jun. 3(4):350-6. [QxMD MEDLINE Link]. [Full Text].

19. Maher TM, Evans IC, Bottoms SE, Mercer PF, Thorley AJ, Nicholson AG. Diminished prostaglandin E2 contributes to the apoptosis paradox in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 2010 Jul 1. 182(1):73-82. [QxMD MEDLINE Link].

20. 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. [QxMD MEDLINE Link].

21. Seibold MA, Wise AL, Speer MC, Steele MP, Brown KK, Loyd JE. A common MUC5B promoter polymorphism and pulmonary fibrosis. N Engl J Med. 2011 Apr 21. 364(16):1503-12. [QxMD MEDLINE Link].

22. Wang XM, Zhang Y, Kim HP, et al. Caveolin-1: a critical regulator of lung fibrosis in idiopathic pulmonary fibrosis. J Exp Med. 2006 Dec 25. 203(13):2895-906. [QxMD MEDLINE Link]. [Full Text].

23. 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. [QxMD MEDLINE Link]. [Full Text].

24. Wootton SC, Kim DS, Kondoh Y, et al. Viral infection in acute exacerbation of idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 2011 Jun 15. 183(12):1698-702. [QxMD MEDLINE Link]. [Full Text].

25. Raghu G, Chen SY, Hou Q, Yeh WS, Collard HR. Incidence and prevalence of idiopathic pulmonary fibrosis in US adults 18-64 years old. Eur Respir J. 2016 Jul. 48 (1):179-86. [QxMD MEDLINE Link].

26. Fernandez Perez ER, Daniels CE, Schroeder DR, et al. Incidence, prevalence, and clinical course of idiopathic pulmonary fibrosis: a population-based study. Chest. 2010 Jan. 137(1):129-37. [QxMD MEDLINE Link]. [Full Text].

27. Raghu G, Weycker D, Edelsberg J, Bradford WZ, Oster G. Incidence and prevalence of idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 2006 Oct 1. 174(7):810-6. [QxMD MEDLINE Link].

28. 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 Aug 1. 176(3):277-84. [QxMD MEDLINE Link].

29. du Bois RM, Weycker D, Albera C, Bradford WZ, Costabel U, Kartashov A, et al. Ascertainment of individual risk of mortality for patients with idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 2011 Aug 15. 184(4):459-66. [QxMD MEDLINE Link].

30. Ley B, Ryerson CJ, Vittinghoff E, et al. A Multidimensional Index and Staging System for Idiopathic Pulmonary Fibrosis. Annals of Internal Medicine. 2012/05. 156:684-691.

31. Fang A, Studer S, Kawut SM, Ahya VN, Lee J, Wille K. Elevated Pulmonary Artery Pressure Is a Risk Factor for Primary Graft Dysfunction Following Lung Transplantation for Idiopathic Pulmonary Fibrosis. Chest. 2011 Apr. 139(4):782-787. [QxMD MEDLINE Link].

32. 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. [QxMD MEDLINE Link]. [Full Text].

33. Tzilas V, Koti A, Papandrinopoulou D, Tsoukalas G. Prognostic factors in idiopathic pulmonary fibrosis. Am J Med Sci. 2009 Dec. 338(6):481-5. [QxMD MEDLINE Link].

34. Kinder BW, Brown KK, Schwarz MI, Ix JH, Kervitsky A, King TE Jr. Baseline BAL neutrophilia predicts early mortality in idiopathic pulmonary fibrosis. Chest. 2008 Jan. 133(1):226-32. [QxMD MEDLINE Link].

35. Kinder BW, Brown KK, McCormack FX, et al. Serum surfactant protein-A is a strong predictor of early mortality in idiopathic pulmonary fibrosis. Chest. 2009 Jun. 135(6):1557-63. [QxMD MEDLINE Link]. [Full Text].

36. Antoniou KM, Hansell DM, Rubens MB, et al. Idiopathic pulmonary fibrosis: outcome in relation to smoking status. Am J Respir Crit Care Med. 2008 Jan 15. 177(2):190-4. [QxMD MEDLINE Link].

37. Lancaster LH, Mason WR, Parnell JA, et al. Obstructive sleep apnea is common in idiopathic pulmonary fibrosis. Chest. 2009 Sep. 136(3):772-8. [QxMD MEDLINE Link]. [Full Text].

38. Dalleywater W, Powell HA, Fogarty AW, Hubbard RB, Navaratnam V. Venous thromboembolism in people with idiopathic pulmonary fibrosis: a population-based study. Eur Respir J. 2014 Dec. 44 (6):1714-5. [QxMD MEDLINE Link].

39. 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 Dec. 101 (12):2534-40. [QxMD MEDLINE Link].

40. Raghu G, Amatto VC, Behr J, Stowasser S. Comorbidities in idiopathic pulmonary fibrosis patients: a systematic literature review. Eur Respir J. 2015 Oct. 46 (4):1113-30. [QxMD MEDLINE Link].

41. Lynch DA, Sverzellati N, Travis WD, Brown KK, Colby TV, Galvin JR, et al. Diagnostic criteria for idiopathic pulmonary fibrosis: a Fleischner Society White Paper. Lancet Respir Med. 2017 Nov 15. [QxMD MEDLINE Link].

42. Lynch DA, Sverzellati N, Travis WD, Brown KK, Colby TV, Galvin JR, et al. Diagnostic criteria for idiopathic pulmonary fibrosis: a Fleischner Society White Paper. Lancet Respir Med. 2018 Feb. 6 (2):138-153. [QxMD MEDLINE Link].

43. du Bois RM, Weycker D, Albera C, Bradford WZ, Costabel U, Kartashov A, 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 Dec 15. 184(12):1382-9. [QxMD MEDLINE Link].

44. 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. [QxMD MEDLINE Link]. [Full Text].

45. Bois RM, Weycker D, Albera C, et al. Six-minute-walk test in idiopathic pulmonary fibrosis: test validation and minimal clinically important difference. Am J Respir Crit Care Med. 2011 May 1. 183(9):1231-7. [QxMD MEDLINE Link].

46. Ohshimo S, Bonella F, Cui A, et al. Significance of bronchoalveolar lavage for the diagnosis of idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 2009 Jun 1. 179(11):1043-7. [QxMD MEDLINE Link].

47. Sharp C, McCabe M, Adamali H, Medford AR. Use of transbronchial cryobiopsy in the diagnosis of interstitial lung disease-a systematic review and cost analysis. QJM. 2017 Apr 1. 110 (4):207-214. [QxMD MEDLINE Link].

48. Ganganah O, Guo SL, Chiniah M, Li YS. Efficacy and safety of cryobiopsy versus forceps biopsy for interstitial lung diseases and lung tumours: A systematic review and meta-analysis. Respirology. 2016 Jul. 21 (5):834-41. [QxMD MEDLINE Link].

49. Hetzel J, Maldonado F, Ravaglia C, et al. Transbronchial Cryobiopsies for the Diagnosis of Diffuse Parenchymal Lung Diseases: Expert Statement from the Cryobiopsy Working Group on Safety and Utility and a Call for Standardization of the Procedure. Respiration. 2018. 95 (3):188-200. [QxMD MEDLINE Link].

50. 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 Dec 15. 184(12):1390-4. [QxMD MEDLINE Link].

51. Collard HR, Ryerson CJ, Corte TJ, et al. Acute Exacerbation of Idiopathic Pulmonary Fibrosis. An International Working Group Report. Am J Respir Crit Care Med. 2016 Aug 1. 194 (3):265-75. [QxMD MEDLINE Link].

52. Kozower BD, Meyers BF, Smith MA, et al. The impact of the lung allocation score on short-term transplantation outcomes: a multicenter study. J Thorac Cardiovasc Surg. 2008 Jan. 135(1):166-71. [QxMD MEDLINE Link].

53. Weiss ES, Allen JG, Merlo CA, Conte JV, Shah AS. Survival after single versus bilateral lung transplantation for high-risk patients with pulmonary fibrosis. Ann Thorac Surg. 2009 Nov. 88(5):1616-25; discussion 1625-6. [QxMD MEDLINE Link].

54. Schaffer JM, Singh SK, Reitz BA, Zamanian RT, Mallidi HR. Single- vs double-lung transplantation in patients with chronic obstructive pulmonary disease and idiopathic pulmonary fibrosis since the implementation of lung allocation based on medical need. JAMA. 2015 Mar 3. 313 (9):936-48. [QxMD MEDLINE Link].

55. Atsumi K, Saito Y, Kuse N, Kobayashi K, Tanaka T, Kashiwada T, et al. Prognostic Factors in the Acute Exacerbation of Idiopathic Pulmonary Fibrosis: A Retrospective Single-center Study. Intern Med. 2017 Nov 20. [QxMD MEDLINE Link].

56. Hyzy R, Huang S, Myers J, Flaherty K, Martinez F. Acute exacerbation of idiopathic pulmonary fibrosis. Chest. 2007 Nov. 132(5):1652-8. [QxMD MEDLINE Link].

57. 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 Feb. 37(2):356-63. [QxMD MEDLINE Link].

58. Farrand E, Vittinghoff E, Ley B, Butte AJ, Collard HR. Corticosteroid use is not associated with improved outcomes in acute exacerbation of IPF. Respirology. 2020 Jun. 25 (6):629-635. [QxMD MEDLINE Link].

59. Mallick S. Outcome of patients with idiopathic pulmonary fibrosis (IPF) ventilated in intensive care unit. Respir Med. 2008 Oct. 102 (10):1355-9. [QxMD MEDLINE Link].

60. Horton MR, Santopietro V, Mathew L, Horton KM, Polito AJ, Liu MC, et al. Thalidomide for the treatment of cough in idiopathic pulmonary fibrosis: a randomized trial. Ann Intern Med. 2012 Sep 18. 157 (6):398-406. [QxMD MEDLINE Link].

61. Birring SS, Wijsenbeek MS, Agrawal S, van den Berg JWK, Stone H, Maher TM, et al. A novel formulation of inhaled sodium cromoglicate (PA101) in idiopathic pulmonary fibrosis and chronic cough: a randomised, double-blind, proof-of-concept, phase 2 trial. Lancet Respir Med. 2017 Oct. 5 (10):806-815. [QxMD MEDLINE Link].

62. Flaherty KR, King TE Jr, Raghu G, Lynch JP 3rd, Colby TV, Travis WD, et al. Idiopathic interstitial pneumonia: what is the effect of a multidisciplinary approach to diagnosis?. Am J Respir Crit Care Med. 2004 Oct 15. 170 (8):904-10. [QxMD MEDLINE Link].

63. Flaherty KR, Andrei AC, King TE Jr, et al. Idiopathic interstitial pneumonia: do community and academic physicians agree on diagnosis?. Am J Respir Crit Care Med. 2007 May 15. 175 (10):1054-60. [QxMD MEDLINE Link].

64. Magnani D, Lenoci G, Balduzzi S, Artioli G, Ferri P. Effectiveness of support groups to improve the quality of life of people with idiopathic pulmonary fibrosis a pre-post test pilot study. Acta Biomed. 2017 Nov 30. 88 (5-S):5-12. [QxMD MEDLINE Link].

65. Root ED, Graney B, Baird S, Churney T, Fier K, Korn M, et al. Physical activity and activity space in patients with pulmonary fibrosis not prescribed supplemental oxygen. BMC Pulm Med. 2017 Nov 23. 17 (1):154. [QxMD MEDLINE Link].

66. Lee JS, Collard HR, Anstrom KJ, Martinez FJ, Noth I, Roberts RS, 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 Jul. 1 (5):369-76. [QxMD MEDLINE Link].

67. 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 Dec 15. 184 (12):1390-4. [QxMD MEDLINE Link].

68. Tran T, Suissa S. The effect of anti-acid therapy on survival in idiopathic pulmonary fibrosis: a methodological review of observational studies. Eur Respir J. 2018 Jun. 51 (6):[QxMD MEDLINE Link].

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70. Gorina E, Richeldi L, Raghu G, et al. PRAISE, a randomized, placebo-controlled, double-blind phase 2 clinical trial of pamrevlumab (FG-3019) in IPF patients. Eur Respir J. 2017. 50:Suppl 61:OA3400.

71. Maher TM, van der Aar EM, Van de Steen O, Allamassey L, Desrivot J, Dupont S, et al. Safety, tolerability, pharmacokinetics, and pharmacodynamics of GLPG1690, a novel autotaxin inhibitor, to treat idiopathic pulmonary fibrosis (FLORA): a phase 2a randomised placebo-controlled trial. Lancet Respir Med. 2018 Aug. 6 (8):627-635. [QxMD MEDLINE Link].

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Alaa Abu Sayf, MD Senior Staff Physician, Division of Pulmonary and Critical Care Medicine, Henry Ford Hospital, Wayne State University School of Medicine

Alaa Abu Sayf, MD is a member of the following medical societies: American College of Chest Physicians, American Thoracic Society, Society of Critical Care Medicine

Disclosure: Nothing to disclose.

Daniel R Ouellette, MD, FCCP Associate Professor of Medicine, Wayne State University School of Medicine; Medical Director, Pulmonary Medicine General Practice Unit (F2), Senior Staff and Attending Physician, Division of Pulmonary and Critical Care Medicine, Henry Ford Hospital

Daniel R Ouellette, MD, FCCP is a member of the following medical societies: American College of Chest Physicians, American Thoracic Society, Society of Critical Care Medicine

Disclosure: Received research grant from: Sanofi Pharmaceutical.

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Guy W Soo Hoo, MD, MPH Professor of Clinical Medicine, University of California, Los Angeles, David Geffen School of Medicine; Director, Medical Intensive Care Unit, Chief, Pulmonary, Critical Care and Sleep Section, West Los Angeles VA Healthcare Center, Veteran Affairs Greater Los Angeles Healthcare System

Guy W Soo Hoo, MD, MPH is a member of the following medical societies: American Association for Respiratory Care, American College of Chest Physicians, American College of Physicians, American Thoracic Society, California Thoracic Society, Society of Critical Care Medicine

Disclosure: Nothing to disclose.

Stephen P Peters, MD, PhD, FACP, FAAAAI, FCCP, FCPP Thomas H Davis Chair in Pulmonary Medicine, Chief, Section on Pulmonary, Critical Care, Allergy and Immunologic Diseases, Professor of Internal Medicine, Pediatrics, and Translational Science, Associate Director, Center for Genomics and Personalized Medicine Research, Wake Forest University School of Medicine; Executive Director of the Respiratory Service Line, Wake Forest Baptist Medical Center

Stephen P Peters, MD, PhD, FACP, FAAAAI, FCCP, FCPP is a member of the following medical societies: American Academy of Allergy Asthma and Immunology, American Association of Immunologists, American College of Chest Physicians, American College of Physicians, American Federation for Medical Research, American Thoracic Society, Sigma Xi, The Scientific Research Honor Society

Disclosure: Serve(d) as a speaker or a member of a speakers bureau for: Integrity CE, Merck<br/>Received income in an amount equal to or greater than $250 from: – Array Biopharma, AstraZeneca, Aerocrine, Airsonett AB, Boehringer-Ingelheim, Experts in Asthma, Gilead, GlaxoSmithKline, Merck, Novartis, Ono Pharmaceuticals, Pfizer, PPD Development, Quintiles, Sunovion, Saatchi & Saatichi, Targacept, TEVA, Theron.

Amanda M K Godfrey, MD Associate Staff, Department of Internal Medicine, IHA Pulmonary, Critical Care, and Sleep Consultants; Associate Staff, Department of Internal Medicine, Division of Pulmonary and Critical Care Medicine, St Joseph Mercy Ann Arbor

Amanda M K Godfrey, MD is a member of the following medical societies: American College of Chest Physicians, American College of Physicians, American Medical Association, American Thoracic Society, Michigan State Medical Society

Disclosure: Nothing to disclose.

The authors and editors of Medscape Reference gratefully acknowledge the contributions of previous authors, Rajesh G. Patel, MD, and Javier I. Diaz, MD, to the development and writing of this article.

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