Idiopatic pulmonary fibrosis: A new paradigm


Cite item

Full Text

Abstract

Idiopathic pulmonary fibrosis ((IPF) is the most common disease from a group of interstitial lung diseases, which occurs mainly in middle-aged and elderly people. Over the past decade, there have been considerable changes in approaches to diagnosing and treating IPF. The paper presents an update on the epidemiology of IPF, the results of new studies of its pathogenesis, and main approaches to diagnosing the disease. In addition, there is new evidence on therapy for IPF.

About the authors

S N Avdeev

НИИ пульмонологии ФМБА России

Москва, Россия

References

  1. Ryu JH, Moua T, Daniels CE, Hartman TE, Yi ES, Utz JP, and Limper AH. Idiopathic Pulmonary Fibrosis: Evolving Concepts. Mayo Clinic Proceedings. 2014;89(8):1130-1142. doi: 10.1016/j.mayocp.2014.03.016
  2. Raghu G, Collard HR, Egan JJ, Martinez FJ, Behr J, Brown KK, … Schünemann HJ. An Official ATS/ERS/JRS/ALAT Statement: Idiopathic Pulmonary Fibrosis: Evidence-based Guidelines for Diagnosis and Management. American Journal of Respiratory and Critical Care Medicine. 2011;183(6):788-824. doi: 10.1164/rccm.2009-040gl
  3. King TE, Pardo A, and Selman M. Idiopathic pulmonary fibrosis. Lancet. 2011;378(9807):1949-1961. doi: 10.1016/s0140-6736(11)60052-4
  4. Travis WD, Costabel U, Hansell DM, King TE, Lynch DA, Nicholson AG, … Valeyre D. An Official American Thoracic Society/European Respiratory Society Statement: Update of the International Multidisciplinary Classification of the Idiopathic Interstitial Pneumonias. American Journal of Respiratory and Critical Care Medicine. 2013;188(6):733-748. doi: 10.1164/rccm.201308-1483st
  5. Navaratnam V, Fleming KM, West J, Smith CJP, Jenkins RG, Fogarty A, and Hubbard RB. The rising incidence of idiopathic pulmonary fibrosis in the UK. Thorax. 2011;66(6):462-467. doi: 10.1136/thx.2010.148031
  6. Vancheri C, Failla M, Crimi N, and Raghu G. Idiopathic pulmonary fibrosis: a disease with similarities and links to cancer biology. European Respiratory Journal. 2010;35(3):496-504. doi: 10.1183/09031936.00077309
  7. Collard HR, Chen S-Y, Yeh W-S, Li Q, Lee Y-C, Wang A, and Raghu G. Health Care Utilization and Costs of Idiopathic Pulmonary Fibrosis in U.S. Medicare Beneficiaries Aged 65 Years and Older. Annals of the American Thoracic Society. 2015;12(7):981-987. doi: 10.1513/annalsats.201412-553oc
  8. Thomeer M, Demedts M, and Vandeurzen K. Registration of interstitial lung diseases by 20 centres of respiratory medicine in Flanders. Acta Clinica Belgica. 2001;56(3):163-172. doi: 10.1179/acb.2001.026
  9. Tinelli C, De Silvestri A, Richeldi L, Oggionni T. The Italian register for diffuse infiltrative lung disorders (RIPID): a four-year report. Sarcoidosis Vasculitis and Diffuse Lung Diseases. 2005;22(Suppl 1):4-8.
  10. Idiopathic Pulmonary Fibrosis: Diagnosis and Treatment. American Journal of Respiratory and Critical Care Medicine. 2000;161(2):646-664. doi: 10.1164/ajrccm.161.2.ats3-00
  11. Raghu G, Chen S-Y, Yeh W-S, Maroni B, Li Q, Lee Y-C, and Collard HR. Idiopathic pulmonary fibrosis in US Medicare beneficiaries aged 65 years and older: incidence, prevalence, and survival, 2001-11. Lancet Respiratory Medicine. 2014;2(7):566-572. doi: 10.1016/s2213-2600(14)70101-8
  12. Natsuizaka M, Chiba H, Kuronuma K, Otsuka M, Kudo K, Mori M, … Takahashi H. Epidemiologic Survey of Japanese Patients with Idiopathic Pulmonary Fibrosis and Investigation of Ethnic Differences. American Journal of Respiratory and Critical Care Medicine. 2014;190(7):773-779. doi: 10.1164/rccm.201403-0566oc
  13. Coultas DB, Zumwalt RE, Black WC, and Sobonya RE. The epidemiology of interstitial lung diseases. American Journal of Respiratory and Critical Care Medicine. 1994;150(4):967-972. doi: 10.1164/ajrccm.150.4.7921471
  14. Mannino DM, Etzel RA, and Parrish RG. Pulmonary fibrosis deaths in the United States, 1979-1991. An analysis of multiple-cause mortality data. American Journal of Respiratory and Critical Care Medicine. 1996;153(5):1548-1552. doi: 10.1164/ajrccm.153.5.8630600
  15. Johnston I, Britton J, Kinnear W, and Logan R. Rising mortality from cryptogenic fibrosing alveolitis. BMJ. 1990;301(6759):1017-1021. doi: 10.1136/bmj.301.6759.1017
  16. Olson AL, Swigris JJ, Lezotte DC, Norris JM, Wilson CG, and Brown KK. Mortality from Pulmonary Fibrosis Increased in the United States from 1992 to 2003. American Journal of Respiratory and Critical Care Medicine. 2007;176(3):277-284. doi: 10.1164/rccm.200701-044oc
  17. Maher TM, Wells AU, and Laurent GJ. Idiopathic pulmonary fibrosis: multiple causes and multiple mechanisms? European Respiratory Journal. 2007;30(5):835-839. doi: 10.1183/09031936.00069307
  18. Daccord C, Maher TM. Recent advances in understanding idiopathic pulmonary fibrosis. F1000Res. 2016;5:1046. doi: 10.12688/f1000research.8209.1
  19. Wuyts WA, Antoniou KM, Borensztajn K, Costabel U, Cottin V, Crestani B, … Wells AU. Combination therapy: the future of management for idiopathic pulmonary fibrosis? Lancet Respiratory Medicine. 2014;2(11):933-942. doi: 10.1016/s2213-2600(14)70232-2
  20. Ahluwalia N, Shea BS, Tager AM. New Therapeutic Targets in Idiopathic Pulmonary Fibrosis. Aiming to Rein in Runaway Wound-Healing Responses. American Journal of Respiratory and Critical Care Medicine. 2014;190(8):867-878. doi: 10.1164/rccm.201403-0509pp
  21. Bagnato G, Harari S. Cellular interactions in the pathogenesis of interstitial lung diseases. European Respiratory Review. 2015;24(135):102-114. doi: 10.1183/09059180.00003214
  22. Wolters PJ, Collard HR, Jones KD. Pathogenesis of Idiopathic Pulmonary Fibrosis. Annual Review of Pathology: Mechanisms of Disease. 2014;9(1):157-179. doi: 10.1146/annurev-pathol-012513-104706
  23. Selman M, Pardo A. Revealing the Pathogenic and Aging-related Mechanisms of the Enigmatic Idiopathic Pulmonary Fibrosis. An Integral Model. American Journal of Respiratory and Critical Care Medicine. 2014;189(10):1161-1172. doi: 10.1164/rccm.201312-2221pp
  24. Wuyts WA, Agostini C, Antoniou KM, Bouros D, Chambers RC, Cottin V, … Verleden GM. The pathogenesis of pulmonary fibrosis: a moving target. European Respiratory Journal. 2012;41(5):1207-1218. doi: 10.1183/09031936.00073012
  25. Fingerlin TE, Murphy E, Zhang W, Peljto AL, Brown KK, Steele MP, … Schwartz DA. Genome-wide association study identifies multiple susceptibility loci for pulmonary fibrosis. Nature Genetics. 2013;45(6):613-620. doi: 10.1038/ng.2609
  26. Noth I, Zhang Y, Ma S-F, Flores C, Barber M, Huang Y, … Garcia JG. Genetic variants associated with idiopathic pulmonary fibrosis susceptibility and mortality: a genome-wide association study. Lancet Respiratory Medicine. 2013;1(4):309-317. doi: 10.1016/s2213-2600(13)70045-6
  27. Roy MG, Livraghi-Butrico A, Fletcher AA, McElwee MM, Evans SE, Boerner RM, … Evans CM. Muc5b is required for airway defence. Nature. 2013;505(7483):412-416. doi: 10.1038/nature12807
  28. Seibold MA, Wise AL, Speer MC, Steele MP, Brown KK, Loyd JE, … Schwartz DA. A Common MUC5B Promoter Polymorphism and Pulmonary Fibrosis. New England Journal of Medicine. 2011;364(16):1503-1512. doi: 10.1056/nejmoa1013660
  29. Peljto AL, Zhang Y, Fingerlin TE, Ma S-F, Garcia JGN, Richards TJ, … Schwartz DA. Association Between the MUC5B Promoter Polymorphism and Survival in Patients With Idiopathic Pulmonary Fibrosis. JAMA. 2013;309(21):2232. doi: 10.1001/jama.2013.5827
  30. Stock CJ, Sato H, Fonseca C, Banya WAS, Molyneaux PL, Adamali H, … Renzoni EA. 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. doi: 10.1136/thoraxjnl-2012-201786
  31. Kropski JA, Blackwell TS, Loyd JE. The genetic basis of idiopathic pulmonary fibrosis. European Respiratory Journal. 2015;45(6):1717-1727. doi: 10.1183/09031936.00163814
  32. Kropski JA, Pritchett JM, Zoz DF, Crossno PF, Markin C, Garnett ET, … Blackwell TS. Extensive Phenotyping of Individuals at Risk for Familial Interstitial Pneumonia Reveals Clues to the Pathogenesis of Interstitial Lung Disease. American Journal of Respiratory and Critical Care Medicine. 2015;191(4):417-426. doi: 10.1164/rccm.201406-1162oc
  33. Stuart BD, Lee JS, Kozlitina J, Noth I, Devine MS, Glazer CS, … Garcia CK. Effect of telomere length on survival in patients with idiopathic pulmonary fibrosis: an observational cohort study with independent validation. Lancet Respiratory Medicine. 2014;2(7):557-565. doi: 10.1016/s2213-2600(14)70124-9
  34. Silva CIS, Müller NL. Idiopathic Interstitial Pneumonias. Journal of Thoracic Imaging. 2009;24(4):260-273. doi: 10.1097/rti.0b013e3181c1a9eb
  35. Lynch DA, Godwin JD, Safrin S, Starko KM, Hormel P, Brown KK, … Webb WR. High-Resolution Computed Tomography in Idiopathic Pulmonary Fibrosis. American Journal of Respiratory and Critical Care Medicine. 2005;172(4):488-493. doi: 10.1164/rccm.200412-1756oc
  36. Arakawa H, Honma K. Honeycomb Lung: History and Current Concepts. American Journal of Roentgenology. 2011;196(4):773-782. doi: 10.2214/ajr.10.4873
  37. Hutchinson JP, Fogarty AW, McKeever TM, Hubbard RB. In-Hospital Mortality after Surgical Lung Biopsy for Interstitial Lung Disease in the United States. 2000 to 2011. American Journal of Respiratory and Critical Care Medicine. 2016;193(10):1161-1167. doi: 10.1164/rccm.201508-1632oc
  38. Ryerson CJ, Urbania TH, Richeldi L, Mooney JJ, Lee JS, Jones KD, … Collard HR. Prevalence and prognosis of unclassifiable interstitial lung disease. European Respiratory Journal. 2012;42(3):750-757. doi: 10.1183/09031936.00131912
  39. Tomassetti S, Wells AU, Costabel U, Cavazza A, Colby TV, Rossi G, … Poletti V. Bronchoscopic Lung Cryobiopsy Increases Diagnostic Confidence in the Multidisciplinary Diagnosis of Idiopathic Pulmonary Fibrosis. American Journal of Respiratory and Critical Care Medicine. 2016;193(7):745-752. doi: 10.1164/rccm.201504-0711oc
  40. Spagnolo P, Wells AU, Collard HR. Pharmacological treatment of idiopathic pulmonary fibrosis: an update. Drug Discovery Today. 2015;20(5):514-524. doi: 10.1016/j.drudis.2015.01.001
  41. Prednisone, Azathioprine, N-Acetylcysteine for Pulmonary Fibrosis. New England Journal of Medicine. 2012;366(21):1968-1977. doi: 10.1056/nejmoa1113354
  42. Raghu G, Rochwerg B, Zhang Y, Garcia CAC, Azuma A, Behr J, … Schünemann HJ. An Official ATS/ERS/JRS/ALAT Clinical Practice Guideline: Treatment of Idiopathic Pulmonary Fibrosis. An Update of the 2011 Clinical Practice Guideline. American Journal of Respiratory and Critical Care Medicine. 2015;192(2):e3-e19. doi: 10.1164/rccm.201506-1063st
  43. Richeldi L, Costabel U, Selman M, Kim DS, Hansell DM, Nicholson AG, … du Bois RM. Efficacy of a Tyrosine Kinase Inhibitor in Idiopathic Pulmonary Fibrosis. New England Journal of Medicine. 2011;365(12):1079-1087. doi: 10.1056/nejmoa1103690
  44. Richeldi L, du Bois RM, Raghu G, Azuma A, Brown KK, Costabel U, … Collard HR. Efficacy and Safety of Nintedanib in Idiopathic Pulmonary Fibrosis. New England Journal of Medicine. 2014;370(22):2071-2082. doi: 10.1056/nejmoa1402584
  45. Richeldi L, Cottin V, du Bois RM, Selman M, Kimura T, Bailes Z, … Brown KK. Nintedanib in patients with idiopathic pulmonary fibrosis: Combined evidence from the TOMORROW and INPULSIS trials. Respiratory Medicine. 2016;113:74-79. doi: 10.1016/j.rmed.2016.02.001
  46. Oku H, Shimizu T, Kawabata T, Nagira M, Hikita I, Ueyama A, … Arimura A. Antifibrotic action of pirfenidone and prednisolone: Different effects on pulmonary cytokines and growth factors in bleomycin-induced murine pulmonary fibrosis. European Journal of Pharmacology. 2008;590(1-3):400-408. doi: 10.1016/j.ejphar.2008.06.046
  47. King TE, Bradford WZ, Castro-Bernardini S, Fagan EA, Glaspole I, Glassberg MK, … Noble PW. A Phase 3 Trial of Pirfenidone in Patients with Idiopathic Pulmonary Fibrosis. New England Journal of Medicine. 2014;370(22):2083-2092. doi: 10.1056/nejmoa1402582
  48. Noble PW, Albera C, Bradford WZ, Costabel U, Glassberg MK, Kardatzke D, … du Bois RM. Pirfenidone in patients with idiopathic pulmonary fibrosis (CAPACITY): two randomised trials. Lancet. 2011;377(9779):1760-1769. doi: 10.1016/s0140-6736(11)60405-4
  49. Demedts M, Behr J, Buhl R, Costabel U, Dekhuijzen R, Jansen HM, … Montanari M. High-Dose Acetylcysteine in Idiopathic Pulmonary Fibrosis. New England Journal of Medicine. 2005;353(21):2229-2242. doi: 10.1056/nejmoa042976
  50. Martinez FJ, de Andrade JA, Anstrom KJ, King TE Jr, Raghu G, and Idiopathic Pulmonary Fibrosis Clinical Research Network. Randomized Trial of Acetylcysteine in Idiopathic Pulmonary Fibrosis. New England Journal of Medicine. 2014;370(22):2093-2101. doi: 10.1056/nejmoa1401739
  51. Oldham JM, Ma S-F, Martinez FJ, Anstrom KJ, Raghu G, Schwartz DA, … Noth I. TOLLIP, MUC5B, and the Response to N-Acetylcysteine among Individuals with Idiopathic Pulmonary Fibrosis. American Journal of Respiratory and Critical Care Medicine. 2015;192(12):1475-1482. doi: 10.1164/rccm.201505-1010oc
  52. Tobin RW, Pope CE, Pellegrini CA, Emond MJ, Sillery J, and Raghu G. Increased Prevalence of Gastroesophageal Reflux in Patients with Idiopathic Pulmonary Fibrosis. American Journal of Respiratory and Critical Care Medicine. 1998;158(6):1804-1808. doi: 10.1164/ajrccm.158.6.9804105
  53. Lee JS, Collard HR, Raghu G, Sweet MP, Hays SR, Campos GM, … King TE. Does Chronic Microaspiration Cause Idiopathic Pulmonary Fibrosis? American Journal of Medicine. 2010;123(4):304-311. doi: 10.1016/j.amjmed.2009.07.033
  54. Lee JS, Ryu JH, Elicker BM, Lydell CP, Jones KD, Wolters PJ, … Collard HR. Gastroesophageal Reflux Therapy Is Associated with Longer Survival in Patients with Idiopathic Pulmonary Fibrosis. American Journal of Respiratory and Critical Care Medicine. 2011;184(12):1390-1394. doi: 10.1164/rccm.201101-0138oc
  55. Lee JS, Collard HR, Anstrom KJ, Martinez FJ, Noth I, Roberts RS, … Raghu G. Anti-acid treatment and disease progression in idiopathic pulmonary fibrosis: an analysis of data from three randomised controlled trials. Lancet Respiratory Medicine. 2013;1(5):369-376. doi: 10.1016/s2213-2600(13)70105-x
  56. Raghu G, and Meyer KC. Silent gastro-oesophageal reflux and microaspiration in IPF: mounting evidence for anti-reflux therapy? European Respiratory Journal. 2012;39(2):242-245. doi: 10.1183/09031936.00211311
  57. Dallemagne B, Weerts J, Markiewicz S, Dewandre J-M, Wahlen C, Monami B, and Jehaes C. Clinical results of laparoscopic fundoplication at ten years after surgery. Surgical Endoscopy. 2005;20(1):159-165. doi: 10.1007/s00464-005-0174-x
  58. Hoppo T., Jarido V, Pennathur A, Morrell M, Crespo M, Shigemura N, Bermudez C, Hunter JG, Toyoda Y, Pilewski J, Luketich JD, Jobe BA. Antireflux Surgery Preserves Lung Function in Patients With Gastroesophageal Reflux Disease and End-stage Lung Disease Before and After Lung Transplantation. Archives of Surgery. 2011;146(9):1041. doi: 10.1001/archsurg.2011.216
  59. Linden PA, Gilbert RJ, Yeap BY, Boyle K, Deykin A, Jaklitsch MT, … Bueno R. Laparoscopic fundoplication in patients with end-stage lung disease awaiting transplantation. Journal of Thoracic and Cardiovascular Surgery. 2006;131(2):438-446. doi: 10.1016/j.jtcvs.2005.10.014
  60. Raghu G, Morrow E, Collins BF, Ho LAT, Hinojosa MW, Hayes JM, … Pellegrini CA. Laparoscopic anti-reflux surgery for idiopathic pulmonary fibrosis at a single centre. European Respiratory Journal. 2016;48(3):826-832. doi: 10.1183/13993003.00488-2016
  61. Collard HR, Ryerson CJ, Corte TJ, Jenkins G, Kondoh Y, Lederer DJ, … Martinez FJ. Acute Exacerbation of Idiopathic Pulmonary Fibrosis. An International Working Group Report. American Journal of Respiratory and Critical Care Medicine. 2016;194(3):265-275. doi: 10.1164/rccm.201604-0801ci
  62. Kim DS, Park JH, Park BK, Lee JS, Nicholson AG, Colby T. Acute exacerbation of idiopathic pulmonary fibrosis: frequency and clinical features. European Respiratory Journal. 2006;27(1):143-150. doi: 10.1183/09031936.06.00114004
  63. Ambrosini V, Cancellieri A, Chilosi M, Zompatori M, Trisolini R, Saragoni L, and Poletti V. Acute exacerbation of idiopathic pulmonary fibrosis: report of a series. European Respiratory Journal. 2003;22(5):821-826. doi: 10.1183/09031936.03.00022703
  64. Kondoh Y, Taniguchi H, Kitaichi M, Yokoi T, Johkoh T, Oishi T, … du Bois RM. Acute exacerbation of interstitial pneumonia following surgical lung biopsy. Respiratory Medicine. 2006;100(10):1753-1759. doi: 10.1016/j.rmed.2006.02.002
  65. Hiwatari N, Shimura S, Takishima T, and Shirato K. Bronchoalveolar Lavage as a Possible Cause of Acute Exacerbation in Idiopathic Pulmonary Fibrosis Patients. Tohoku Journal of Experimental Medicine. 1994;174(4):379-386. doi: 10.1620/tjem.174.379
  66. Parambil JG, Myers JL, and Ryu JH. Histopathologic Features and Outcome of Patients With Acute Exacerbation of Idiopathic Pulmonary Fibrosis Undergoing Surgical Lung Biopsy. Chest. 2005;128(5):3310-3315. doi: 10.1378/chest.128.5.3310
  67. Ranieri VM, Rubenfeld GD, Thompson BT, Ferguson ND, Caldwell E, Fan E, Camporota L, Slutsky AS; ARDS Definition Task Force. Acute Respiratory Distress Syndrome. The Berlin Definition. JAMA. 2012;307(23):2526-2533. doi: 10.1001/jama.2012.5669
  68. Rush B, Wiskar K, Berger L, and Griesdale D. The use of mechanical ventilation in patients with idiopathic pulmonary fibrosis in the United States: A nationwide retrospective cohort analysis. Respiratory Medicine. 2016;111:72-76. doi: 10.1016/j.rmed.2015.12.005
  69. Inase N, Sawada M, Ohtani Y, Miyake S, Isogai S, Sakashita H, … Yoshizawa Y. Cyclosporin A Followed by the Treatment of Acute Exacerbation of Idiopathic Pulmonary Fibrosis with Corticosteroid. Internal Medicine. 2003;42(7):565-570. doi: 10.2169/internalmedicine.42.565
  70. Homma S, Sakamoto S, Kawabata M, Kishi K, Tsuboi E, Motoi N, and Yoshimura K. Cyclosporin Treatment in Steroid-resistant and Acutely Exacerbated Interstitial Pneumonia. Internal Medicine. 2005;44(11):1144-1150. doi: 10.2169/internalmedicine.44.1144
  71. Sakamoto S, Homma S, Miyamoto A, Kurosaki A, Fujii T, and Yoshimura K. Cyclosporin A in the Treatment of Acute Exacerbation of Idiopathic Pulmonary Fibrosis. Internal Medicine. 2010;49(2):109-115. doi: 10.2169/internalmedicine.49.2359
  72. Donahoe M, Valentine VG, Chien N, Gibson KF, Raval JS, Saul M, … Duncan SR. Autoantibody-Targeted Treatments for Acute Exacerbations of Idiopathic Pulmonary Fibrosis. PLoS ONE. 2015;10(6):e0127771. doi: 10.1371/journal.pone.0127771
  73. Horita N, Akahane M, Okada Y, Kobayashi Y, Arai T, Amano I, Takezawa T, To M, To Y. Tacrolimus and Steroid Treatment for Acute Exacerbation of Idiopathic Pulmonary Fibrosis. Internal Medicine. 2011;50(3):189-195. doi: 10.2169/internalmedicine.50.4327
  74. Isshiki T, Sakamoto S, Kinoshita A, Sugino K, Kurosaki A, and Homma S. Recombinant Human Soluble Thrombomodulin Treatment for Acute Exacerbation of Idiopathic Pulmonary Fibrosis: A Retrospective Study. Respiration. 2015;89(3):201-207. doi: 10.1159/000369828
  75. Tsushima K, Yamaguchi K, Kono Y, Yokoyama T, Kubo K, Matsumura T, Ichimura Y, Abe M, Terada J, Tatsumi K. Thrombomodulin for acute exacerbations of idiopathic pulmonary fibrosis: A proof of concept study. Pulmonary Pharmacology & Therapeutics. 2014;29(2):233-240. doi: 10.1016/j.pupt.2014.04.008
  76. Kataoka K, Taniguchi H, Kondoh Y, Nishiyama O, Kimura T, Matsuda T, Yokoyama T, Sakamoto K, Ando M. Recombinant Human Thrombomodulin in Acute Exacerbation of Idiopathic Pulmonary Fibrosis. Chest. 2015;148(2):436-443. doi: 10.1378/chest.14-2746.

Copyright (c) 2017 Consilium Medicum

Creative Commons License
This work is licensed under a Creative Commons Attribution-NonCommercial-ShareAlike 4.0 International License.
 
 


This website uses cookies

You consent to our cookies if you continue to use our website.

About Cookies