Idiopathic pulmonary fibrosis

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Abstract

Idiopathic pulmonary fibrosis (IPF) is the most common disease from the group interstitial lung disease and occurs predominantly in persons of middle and old age. Over the past decade there have been significant changes in approaches to diagnosis and treatment of IPF. This article contains modern data on the epidemiology of IPF, the results of new research on the pathogenesis, basic approaches to the diagnosis of the disease. In addition, presented new data on the therapy of IPF.

About the authors

S. N Avdeev

Research Institute of Pulmonology of FMBA of Russia

Email: serg_avdeev@list.ru
чл-кор. РАН, д-р. мед. наук, проф., рук. клин. отд. ФГБУ «НИИ пульмонологии» 105077, Russian Federation, Moscow, ul. 11-ia Parkovaia, d. 32, korp. 4

References

  1. Чучалин А.Г., Авдеев С.Н., Айсанов З.Р. и др. Диагностика и лечение идиопатического легочного фиброза. Федеральные клинические рекомендации. Пульмонология. 2016; 26 (4): 399-419.
  2. Интерстициальные и орфанные заболевания легких. Библиотека врача - специалиста. Под ред. М.М.Ильковича. М.: ГЭОТАР-Медиа, 2016.
  3. Авдеев С.Н. Идиопатический легочный фиброз. Пульмонология. 2015; 25 (5): 600-12.
  4. Raghu G, Collard H.R, Egan J.J et al. and ATS/ERS/JRS/ALAT Committee on Idiopathic Pulmonary Fibrosis. An official ATS/ERS/JRS/ALAT statement: idiopathic pulmonary fibrosis; evidence - based guidelines for diagnosis and management. Am J Respir Crit Care Med 2011; 183: 788-824.
  5. Travis W.D, Costabel U, Hansell D.M and 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; 188: 733-48.
  6. Ryu J.H, Moua T, Daniels C.E et al. Idiopathic pulmonary fibrosis: Evolving concepts. Mayo Clin Proc 2014; 89: 1130-42.
  7. King T.E, Pardo A, Selman M. Idiopathic pulmonary fibrosis. Lancet 2011; 378: 1949-61.
  8. Navaratnam V.F.K, West J, Smith C.J et al. The rising incidence of idiopathic pulmonary fibrosis in the UK. Thorax 2011; 66: 462-7.
  9. 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: 496-504.
  10. Collard H.R, Chen S-Y, Yeh W-S et al. Health care utilization and costs in idiopathic pulmonary fibrosis in United States medicare beneficiaries aged 65 years and older. Ann Am Thorac Soc 2015; 12: 981-7.
  11. Thomeer M, Demedts M, Vandeurzen K. Registration of interstitial lung diseases by 20 centres of respiratory medicine in Flanders. Acta Clin Belg 2001; 56: 163-72.
  12. Tinelli C, de Silvestri A, Richeldi L, Oggionni T. The Italian register for diffuse infiltrative lung disorders (RIPID): a four - year report. Sarcoidosis Vasc. Diffuse Lung Dis 2005; 22: S4-8.
  13. American Thoracic Society. Idiopathic pulmonary fibrosis: diagnosis and treatment; international consensus statement. Am J Respir Crit Care Med 2000; 161: 646-64.
  14. Raghu G, Chen S-Y, Yeh W-S et al. Idiopathic pulmonary fibrosis in US Medicare beneficiaries aged 65 years and older: incidence, prevalence and survival, 2001-2011. Lancet Respir Med 2014; 2: 566-72.
  15. Natsuizaka M, Chiba H, Kuronuma K et al. Epidemiologic survey of Japanese patients with idiopathic pulmonary fibrosis and investigation of ethnic differences. Am J Respir Crit Care Med 2014; 190: 773-9.
  16. Coultas D.B, Zumwalt R.E, Black W.C, Sobonya R.E. The epidemiology of interstitial lung diseases. Am J Respir Crit Care Med 1994; 150: 967-72.
  17. Mannino D.M, Etzel R, Parrish R.G. Pulmonary fibrosis deaths in the United States, 1979-1991. An analysis of multiple - cause mortality data. Am J Respir Crit Care Med 1996; 153: 1548-52.
  18. Johnston I, Britton J, Kinnear W, Logan R. Rising mortality from cryptogenic fibrosing alveolitis. BMJ 1990; 301: 1017-21.
  19. Olson A.L, Swigris J, Lezotte D.C et al. Mortality from pulmonary fibrosis increased in the United States from1992 to 2003. Am J Respir Crit Care Med 2007; 176: 277-84.
  20. Maher T.M, Wells A.U, Laurent G.J. Idiopathic pulmonary fibrosis: multiple causes and multiple mechanisms? Eur Respir J 2007; 30: 835-9.
  21. Daccord C, Maher T.M. Recent advances in understanding idiopathic pulmonary fibrosis. F1000Research 2016, 5(F1000 Faculty Rev): 1046. doi: 10.12688/f1000research.8209.1.
  22. Wuyts W.A, Antoniou K.M, Borensztajn K et al. Combination therapy: the future of management for idiopathic pulmonary fibrosis? Lancet Respir Med 2014; 2 (11): 933-42.
  23. Ahluwalia N, Shea B.S, Tager A.M. New therapeutic targets in idiopathic pulmonary fibrosis. Aiming to rein in runaway wound - healing responses. Am J Respir Crit Care Med 2014; 190: 867-78.
  24. Selman M, Pardo A. Revealing the pathogenic and aging - related mechanisms of the enigmatic idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 2014; 189 (10): 1161-72.
  25. Wuyts W.A, Agostini C, Antoniou K.M et al. The pathogenesis of pulmonary fibrosis: a moving target. Eur Respir J 2013; 41: 1207-18.
  26. Fingerlin T.E, Murphy E, Zhang W et al. Genome - wide association study identifies multiple susceptibility loci for pulmonary fibrosis. Nat Genet 2013; 45: 613-20.
  27. Noth I, Zhang Y, Ma S.F et al. Genetic variants associated with idiopathic pulmonary fibrosis susceptibility and mortality: a genome - wide association study. Lancet Respir Med 2013; 1: 309-17.
  28. Roy M.G, Livraghi-Butrico A, Fletcher A.A et al. Muc5b is required for airway defence. Nature 2014; 505: 412-6.
  29. Seibold M.A, Wise A.L, Speer M.C et al. A common MUC5B promoter polymorphism and pulmonary fibrosis. N Engl J Med 2011; 364: 1503-12.
  30. Peljto A.L, Zhang Y, Fingerlin T.E et al. Association between the MUC5B promoter polymorphism and survival in patients with idiopathic pulmonary fibrosis. JAMA 2013; 309: 2232-9.
  31. Kropski J.A, Blackwell T.S, Loyd J.E. The genetic basis of idiopathic pulmonary fibrosis. Eur Respir J 2015; 45: 1717-27.
  32. Kropski J.A, Pritchett J.M, Zoz D.F et al. Extensive phenotyping of individuals at risk for familial interstitial pneumonia reveals clues to the pathogenesis of interstitial lung disease. Am J Respir Crit Care Med 2015; 191: 417-26.
  33. Stuart B.D, Lee J.S, Kozlitina J et al. Effect of telomere length on survival in patients with idiopathic pulmonary fibrosis: an observational cohort study with independent validation. Lancet Respir Med 2014; 2: 557-65.
  34. Richeldi L, Rubin A, Avdeev S et al. Idiopathic pulmonary fibrosis in BRIC countries: the cases of Brazil, Russia, India, and China. BMC Medicine 2015; 13: 237.
  35. Behr J, Ryu J.H. Pulmonary hypertension in interstitial lung disease. Eur Respir J 2008; 31: 1357-67.
  36. Nathan S.D, Noble P.W, Tuder R.M. Idiopathic pulmonary fibrosis and pulmonary hypertension: connecting the dots. Am J Respir Crit Care Med 2007; 175: 875-80.
  37. Silva C.I, Müller N.L. Idiopathic interstitial pneumonias. J Thorac Imaging 2009; 24: 260-73.
  38. Lynch D.A, Godwin J.D, Safrin S and Idiopathic Pulmonary Fibrosis Study Group. High - resolution computed tomography in idiopathic pulmonary fibrosis: diagnosis and prognosis. Am J Respir Crit Care Med 2005; 172: 488-93.
  39. Arakawa H, Honma K. Honeycomb lung: history and current concepts. AJR Am J Roentgenol 2011; 196: 773-82.
  40. Cottin V, Nunes H, Brillet P.Y and Groupe d'Etude et de Recherche sur les Maladies Orphelines Pulmonaires (GERM O P). Combined pulmonary fibrosis and emphysema: a distinct underrecognised entity. Eur Respir J 2005; 26: 586-93.
  41. Jankowich M.D, Rounds S.I. Combined pulmonary fibrosis and emphysema syndrome: a review. Chest 2012; 141: 222-31.
  42. Ryerson C.J, Hartman T, Elicker B.M et al. Clinical features and outcomes in combined pulmonary fibrosis and emphysema in idiopathic pulmonary fibrosis. Chest 2013; 144: 234-40.
  43. Zhang Y, Kaminski N. Biomarkers in idiopathic pulmonary fibrosis. Curr Opin Pulm Med 2012; 18: 441-6.
  44. Vij R, Noth I. Peripheral blood biomarkers in idiopathic pulmonary fibrosis. Transl Res 2012; 159: 218-27.
  45. Tzouvelekis A, Kouliatsis G, Anevlavis S, Bouros D. Serum biomarkers in interstitial lung diseases. Respir Res 2005; 6: 78.
  46. Prasse A, Müller-Quernheim J. Non - invasive biomarkers in pulmonary fibrosis. Respirology 2009; 14: 788-95.
  47. Авдеева О.Е., Лебедин Ю.С., Авдеев С.Н. и др. Гликозилированный муцин - антиген 3EG5 - сывороточный маркер активности и тяжести при интерстициальных заболеваниях легких. Пульмонология. 1998; 2: 22-7.
  48. Hutchinson J.P, Fogarty A.W, Mc Keever T.M et al. In-Hospital Mortality after Surgical Lung Biopsy for Interstitial Lung Disease in the United States. 2000 to 2011. Am J Respir Crit Care Med 2016; 193: 1161-7.
  49. Ryerson C.J, Urbania T.H, Richeldi L et al. Prevalence and prognosis of unclassifiable interstitial lung disease. Eur Respir J 2013; 42: 750-7.
  50. Tomassetti S, Wells A.U, Costabel U et al. Bronchoscopic Lung Cryobiopsy Increases Diagnostic Confidence in the Multidisciplinary Diagnosis of Idiopathic Pulmonary Fibrosis. Am J Respir Crit Care Med 2016; 193: 745-52.
  51. Spagnolo P, Wells A.U, Collard H.R. Pharmacological treatment of idiopathic pulmonary fibrosis: an update. Drug Discovery Today 2015. http://dx.doi.org/10.1016/j.drudis.2015.01.001
  52. Idiopathic Pulmonary Fibrosis Clinical Research Network. Prednisone, azathioprine, and N-acetylcysteine for pulmonary fibrosis. N Engl J Med 2012; 366: 1968-77.
  53. Raghu G, Rochwerg B, Zhang Y et al. An Official ATS/ERS/JRS/ALAT Clinical Practice Guideline: Treatment of Idiopathic Pulmonary Fibrosis: Executive Summary An Update of the 2011 Clinical Practice Guideline. Am J Respir Crit Care Med 2015; 192: e3-e19.
  54. Richeldi L, Costabel U, Selman M et al. Efficacy of a tyrosine kinase inhibitor in idiopathic pulmonary fibrosis. N Engl J Med 2011; 365: 1079-87.
  55. Richeldi L, du Bois R.M, Raghu G et al. Efficacy and safety of nintedanib in idiopathic pulmonary fibrosis. N Engl J Med 2014; 370: 2071-82.
  56. Richeldi L, Cottin V, du Bois R.M et al. Nintedanib in patients with idiopathic pulmonary fibrosis: Combined evidence from the TOMORROW and INPULSIS® trials. Respir Med 2016; 113: 74-9.
  57. Oku H, Shimizu T, Kawabata T et al. Antifibrotic action of pirfenidone and prednisolone: different effects on pulmonary cytokines and growth factors in bleomycin - induced murine pulmonary fibrosis. Eur J Pharmacol 2008; 590: 400-8.
  58. King T.E, Bradford W.Z, Castro-Bernardini S et al. A phase 3 trial of pirfenidone in patients with idiopathic pulmonary fibrosis. N Engl J Med 2014; 370: 2083-92.
  59. Demedts M, Behr J, Buhl R et al. High - dose acetylcysteine in idiopathic pulmonary fibrosis. N Engl J Med 2005; 353: 2229-42.
  60. Martinez F.J, de Andrade J.A, Anstrom K.J et al. and Idiopathic Pulmonary Fibrosis Clinical Research Network. Randomized trial of N-acetylcysteine in idiopathic pulmonary fibrosis. N Engl J Med 2014; 370: 2093-101.
  61. Oldham J.M, Ma S-F, Martinez F.J et al. TOLLIP, MUC5B, and the Response to N-Acetylcysteine among Individuals with Idiopathic Pulmonary Fibrosis. Am J Respir Crit Care Med 2015; 192: 1475-82.
  62. Tobin R.W, Pope C.E, Pellegrini C.A et al. Increased prevalence of gastroesophageal reflux in patients with idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 1998; 158: 1804-8.
  63. Lee J.S, Collard H.R, Raghu G et al. Does chronic microaspiration cause idiopathic pulmonary fibrosis? Am J Med 2010; 123: 304-11.
  64. Lee J.S, Ryu J.H, Elicker B.M et al. Gastroesophageal reflux therapy is associated with longer survival in patients with idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 2011; 184: 1390-4.
  65. Lee J.S, Collard H.R, Anstrom K.J et al. Anti - acid treatment and disease progression in idiopathic pulmonary fibrosis: an analysis of data from three randomised controlled trials. Lancet Respir Med 2013; 1: 369-76.
  66. Raghu G, Meyer K.C. Silent gastro - oesophageal reflux and microaspiration in IPF: mounting evidence for anti - reflux therapy? Eur Respir J 2012; 39: 242-5.
  67. Dallemagne B, Weerts J, Markiewicz S et al. Clinical results of laparoscopic fundoplication at ten years after surgery. Surg Endosc 2006; 20: 159-65.
  68. Raghu G, Morrow E, Collins B.F et al. Laparoscopic anti - reflux surgery for idiopathic pulmonary fibrosis at a single centre. Eur Respir J 2016; 48: 826-32.

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