Skip to main content
SpringerLink
Log in

Lung Transplantation for Interstitial Lung Disease

  • Chapter
  • First Online:
Essentials in Lung Transplantation

  • 788 Accesses

Abstract

This chapter aims to review the need for lung transplantation for interstitial lung diseases in particular for idiopathic pulmonary fibrosis (IPF), which often fails to respond to medical therapy. The American Thoracic Society recognizes that supplemental oxygen and transplantation are the only suggested treatments for IPF. Prognosis of IPF is generally poor, retrospective cohort studies indicate a median survival of 2–3 years from diagnosis and only 20–30% survive 5 years after diagnosis. Consequently patients with this condition are at increased risk of mortality and lung transplantation offers hope to patients, who are found to be suitable after careful selection for this life saving procedure. Over the past 2–3 decades significant improvements in long term survival and quality of life have been demonstrated. However survival after lung transplantation remains significantly shorter than survival reported in other solid organ transplants (SOT) due to chronic lung allograft dysfunction (CLAD) and infection.

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

Log in via an institution
Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 79.99
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD 99.99
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info
Hardcover Book
USD 139.99
Price excludes VAT (USA)
  • Durable hardcover edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

References

  1. Leibow AA, Carrington CB. The interstitial pneumonia. In: Simon M, Potchen EJ, LeMay M, editors. Frontiers of pulmonary radiology. 1st ed. New York: Grune & Stratton; 1969. p. 102–41.

    Google Scholar 

  2. Travis WD, Costabel U, Hansell DM, et al. American Thoracic Society/European Respiratory Society: international multidisciplinary consensus classification of the idiopathic interstitial pneumonias. Am J Respir Crit Care Med. 2002;165(2):277–304.

    Article  Google Scholar 

  3. Troy L, Corte T. Interstitial lung disease in 2015: where are we now? Aust Fam Physician. 2015;44(8):546–52.

    PubMed  Google Scholar 

  4. Raghu G, Collard HR. 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. https://doi.org/10.1164/rccm.2009.

    Article  PubMed  PubMed Central  Google Scholar 

  5. Selman M, Pardo A. Idiopathic pulmonary fibrosis: an epithelial fibroblast cross talk disorder. Respir Res. 2002;3:3.

    Article  PubMed  Google Scholar 

  6. Gross TJ, Hunninghake GW. Idiopathic pulmonary fibrosis. N Engl J Med. 2001;345:517–25. https://doi.org/10.1056/NEJMra003200.

    Article  PubMed  CAS  Google Scholar 

  7. Carmelo A, Dunmore R, Sleeman MA, et al. The epithelium in idiopathic pulmonary fibrosis: breaking the barrier. Front Pharmacol. 2014;4:173.

    Google Scholar 

  8. Cooper JA Jr, White DA, Matthay RA. Drug induced pulmonary disease. Part 1: cytoxic drugs. Am Rev Respir Dis. 1986;133:321–40.

    PubMed  CAS  Google Scholar 

  9. Bradley B, Branley HM, Egan JJ, et al. Interstitial lung disease guideline. Thorax. 2008;63(Suppl 5):v1–v58.

    PubMed  Google Scholar 

  10. Raghu C, Mageto YN, Lockhart D, et al. The accuracy of the clinical diagnosis of new onset idiopathic pulmonary fibrosis. Chest. 1999;116:1168–74.

    Article  CAS  PubMed  Google Scholar 

  11. King TE Jr, Tooze JA, Schwatz MI, et al. Predicting survival in idiopathic pulmonary fibrosis: scoring system and survival model. Am J Respir Crit Care Med. 2001;164:171–1181.

    Google Scholar 

  12. Gribbin J, Hubbard RB, Le Jeune I, et al. Incidence and mortality of idiopathic pulmonary fibrosis and sarcoidosis in the UK. Thorax. 2006;61:908–85.

    Article  Google Scholar 

  13. Flaherty KR, King TE Jr, Raghu G, et al. Idiopathic interstitial pneumonia: what is the effect of a multidisciplinary approach to diagnosis? Am J Respir Crit Care Med. 2004;170:904–10.

    Article  PubMed  Google Scholar 

  14. Yang S, Raghu G, et al. Clinical evidence in diffuse parenchymal lung disease. Basel: Karger; 2007. p. 22–8.

    Google Scholar 

  15. Weill D, Benden C, Corris PA, et al. A consensus document for the selection of lung transplant candidates: 2014—an update from the Pulmonary Transplantation Council of the International Society for Heart and Lung Transplantation. J Heart Lung Transplant. 2015;34(1):1–15.

    Article  PubMed  Google Scholar 

  16. Richeldi L, Costabel U, et al. Efficacy of a tyrosine kinase inhibitor in idiopathic pulmonary fibrosis. N Engl J Med. 2011;365:1079–87.

    Article  CAS  PubMed  Google Scholar 

  17. Richeldi L, du Bois RM, Raghu G, et al. Efficacy and safety of nintedanib in idiopathic pulmonary fibrosis. N Engl J Med. 2014;370(22):2071–82.

    Article  CAS  PubMed  Google Scholar 

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

    Article  CAS  PubMed  Google Scholar 

  19. Jo HE. TSANZ position statement: treatment of idiopathic pulmonary fibrosis. Respirology. 2017;22:1436.

    Article  PubMed  Google Scholar 

  20. Ragu G. Idiopathic pulmonary fibrosis: a rational approach. Chest. 1987;92:148–54.

    Article  Google Scholar 

  21. Selman M, Carrilo G, Estrada A, et al. Accelerated variance of idiopathic pulmonary fibrosis: clinical behavior and gene expression pattern. PLoS One. 2007;2:e48.

    Article  CAS  Google Scholar 

  22. Mejia M, Carrilo G, Rojas-Serrano J, et al. Idiopathic pulmonary fibrosis and empysema: decreased survival associated with severe PAH. Chest. 2009;136:10–5.

    Article  PubMed  Google Scholar 

  23. Bongartz T, Nannini C, Medina-Velasquez YF, et al. Incidence and mortality of interstitial lung disease in rheumatoid arthritis:a population- based study. Arthritis Rheum. 2010;62:1583–91.

    Article  PubMed  PubMed Central  Google Scholar 

  24. Bouros D, Wells AU, Nicholson AG, et al. Histopathologic subsets of fibrosing alveolitis in patients with systemic sclerosis and their relationship to outcome. Am J Respir Crit Care Med. 2002;165:1581–6.

    Article  PubMed  Google Scholar 

  25. Group TLT: Toronto Lung Transplant Group. Unilateral lung transplantation for pulmonary fibrosis. N Engl J Med. 1986;314(18):1140–5.

    Article  Google Scholar 

  26. Christie JD, Edwards LB, Aurora P, et al. The registry of the International Society for Heart and Lung Transplantation: thirteenth official pediatric lung and heart-lung transplantation report—2010. The official report ISHLT. J Heart Lung Transplant. 2009;28(10):993–1006.

    Article  PubMed  Google Scholar 

  27. Yusen RD, Shearon TH, Qian Y, et al. Lung transplantation in USA 1999-2006. Am J Transplant. 2010;10(4 Pt 2):1047–68.

    Article  CAS  PubMed  Google Scholar 

  28. Timothy JG, Aranaoutakis GJ, et al. Lung transplant in idiopathic pulmonary fibrosis. Arch Surg. 2011;146(10):1204–9.

    Article  Google Scholar 

  29. Armanios M. Telomeres and age relate disease: how telomere biology informs clinical paradigms. J Clin Invest. 2013;123:996–1002.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  30. Silhan LL, Shah PD, Chambers DC, et al. Lung transplantation in telomerase mutation carriers with pulmonary fibrosis. Eur Respir J. 2014;44:178–87.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  31. Raghu G, Rochwerg B, Zhang Y, et al. An official ATS/ERS/JRS/ALAT clinical practice guidelines: treatment of idiopathic pulmonary fibrosis. An update of the 2011 clinical practice guideline. Am J Respir Crit Care Med. 2015;192(2):e3–e19.

    Article  PubMed  Google Scholar 

  32. ISHLT Registry Report in the JHLT 2017;36(10):1037–79.

    Google Scholar 

  33. Kistler K, Nalysnk L, Rotella P, et al. Lung transplantation in idiopathic pulmonary fibrosis: a systemic review of the literature. BMC Pulm Med. 2014;14:139.

    Article  PubMed  PubMed Central  Google Scholar 

  34. Force SD, Kilgo P, Neujahr DC, et al. Bilateral lung transplantation offers better long term survival, compared with single lung transplantation for younger patients with idiopathic pulmonary fibrosis. Ann Thorac Surg. 2011;91:244–9.

    Article  PubMed  PubMed Central  Google Scholar 

  35. Mason DP, Brizzio ME, Alster JM, et al. Lung transplantation for idiopathic pulmonary fibrosis. Ann Thorac Surg. 2007;84:1121–8.

    Article  PubMed  Google Scholar 

  36. Neurohr C, Huppmann P, Thum D, et al. Potential functional and survival benefit of double over single lung transplantation for selected patients with idiopathic pulmonary fibrosis. Transpl Int. 2010;23:887–96.

    PubMed  Google Scholar 

  37. De Oliveria NC, Osaki S, Maloney J, et al. Lung transplant for interstitial lung disease: outcomes for single versus bilateral lung transplantation. Interact Cardiovasc Thorac Surg. 2012;14:263–7.

    Article  Google Scholar 

  38. Yusen RD, Edwards LB, Kucheryavaya AY, et al. The registry of the International Society for Heart and Lung Transplantation: thirty-first adult and heart lung transplant report—2014: focus theme: retransplantation. J Heart Lung Transplant. 2014;33(10):1009.

    Article  PubMed  Google Scholar 

  39. Newton CA, Kozlitina J, Lines JR, et al. Telomere length in patients with pulmonary fibrosis associated with chronic lung allograft and post-lung transplant survival. J Heart Lung Transplant. 2017;36(8):845–53.

    Article  PubMed  PubMed Central  Google Scholar 

  40. Collins J, Kazerooni EA, Lacomis J, et al. Bronchogenic carcinoma after lung transplantation: frequency, clinical characteristics and imaging findings. Radiology. 2002;224:131–18.

    Article  PubMed  Google Scholar 

  41. Dickinson RP, Davis RD, Rea JB, et al. High frequency of bronchogenic carcinoma after single lung transplantation. J Heart Lung Transplant. 2006;25:1297–301.

    Article  Google Scholar 

  42. Hubbard RB, Smith C, Le Jeune I. The association between idiopathic pulmonary fibrosis and vascular disease. A population-based study. Am J Respir Crit Care Med. 2008;178:1257–61. https://doi.org/10.1164/rccm.200805-725OC.

    Article  PubMed  Google Scholar 

  43. Kizer JR, Zisman DA, Blumenthal NP, et al. Association between pulmonary fibrosis and coronary artery disease. Arch Intern Med. 2004;164:551–5.

    Article  PubMed  Google Scholar 

  44. Lerreri CJ, Nathan SD, Barnett SD, et al. Prevalance and outcomes of pulmonary hypertension in advanced idiopathic pulmonary fibrosis. Chest. 2006;129:746–52.

    Article  Google Scholar 

  45. Collard HR, Anstrom KJ, et al. Sildenafil improves walk distance in idiopathic pulmonary fibrosis. Chest. 2009;136:10–5.

    Article  Google Scholar 

  46. Ghofrani HA, Wiedermann R, Rose F, et al. Sildenafil for treatment of lung fibrosis and pulmonary hypertension: a randomised controlled trial. Lancet. 2002;360:895–900.

    Article  CAS  PubMed  Google Scholar 

  47. Ragu G, Behr J, Brown KK, Artemis IPF Investigators*, et al. Treatment of idiopathic pulmonary fibrosis with ambrisentan: a parallel, randomised trial. Ann Intern Med. 2013;148:641–9.

    Article  Google Scholar 

  48. Jackson RM, Glassberg MK, Ramos CF, et al. Sildenafil therapy and exercise tolerance in idiopathic pulmonary fibrosis. Lung. 2010;188:115–23.

    Article  CAS  PubMed  Google Scholar 

  49. Zisman DA, Schartz M, Anstrom KL, Idiopathic Pulmonary Fibrosis Clinical Research Network, et al. A controlled trial of sildenafil in advanced idiopathic pulmonary fibrosis. N Engl J Med. 2010;363:620–8.

    Article  PubMed  Google Scholar 

  50. Delanote I, Wuyts WA, Yserbyt J, et al. BMC Pulm Med. 2016;16:156.

    Article  PubMed  PubMed Central  Google Scholar 

Download references

Acknowledgment

The author is grateful to the anatomical department of St. Vincent’s hospital for graciously providing anatomical histology of the various interstitial lung diseases required for this chapter.

Author information

Authors and Affiliations

  1. The Lung Transplant Unit Xavier Level 4, St. Vincent’s Hospital, Darlinghurst, NSW, Australia

    Monique Anne Malouf

Authors
  1. Monique Anne Malouf
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Monique Anne Malouf .

Editor information

Editors and Affiliations

  1. The Lung Transplant Unit, Department of Thoracic Medicine, St Vincent’s Hospital, Sydney, NSW, Australia

    Allan R. Glanville

Rights and permissions

Reprints and permissions

Copyright information

© 2019 Springer International Publishing AG, part of Springer Nature

About this chapter

Check for updates. Verify currency and authenticity via CrossMark

Cite this chapter

Malouf, M.A. (2019). Lung Transplantation for Interstitial Lung Disease. In: Glanville, A. (eds) Essentials in Lung Transplantation . Springer, Cham. https://doi.org/10.1007/978-3-319-90933-2_12

Download citation

  • DOI: https://doi.org/10.1007/978-3-319-90933-2_12

  • Published:

  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-319-90932-5

  • Online ISBN: 978-3-319-90933-2

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics

Search

Navigation