Skip to main content

Non-pharmacological Therapy for IPF

Is Respiratory Care Actually Effective?

  • Chapter
Idiopathic Pulmonary Fibrosis

Abstract

Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive interstitial lung disease characterized by a poor prognosis and the lack of proven effective treatments. The symptoms and complications of IPF such as dyspnea, exercise intolerance, and depression severely impair patients’ quality of life (QOL) and decrease social participation. Although evidence for the benefit of pulmonary rehabilitation (PR) in IPF is limited, it has recently been reported that PR can improve dyspnea and exercise tolerance. Furthermore, exercise training and educational programs may also be effective means of addressing low mood and depression in selected patients with IPF. Long-term oxygen therapy (LTOT) is also considered to improve QOL in patients with IPF. Although LTOT may have no survival benefit, patients with resting hypoxemia, pulmonary hypertension, exercise-induced hypoxemia, or nocturnal hypoxemia should be treated with LTOT to improve QOL. Noninvasive ventilation and nasal high-flow oxygen therapy have been recently used to manage acute respiratory failure complicating IPF. Early use of these techniques might afford the opportunity to avoid tracheal intubation and reduce the high incidence of mortality associated with acute deterioration of respiratory function in IPF. In conclusion, the evidence base informing management strategies for IPF has been growing gradually. It appears that PR may have an important role to play in improving the QOL of patients with IPF, although further research is still needed.

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

Access this chapter

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 109.99
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. Schwarz MI, King Jr TE. Physiology of interstitial lung disease. In: Interstitial lung disease. 4th ed. London: BC Decker Inc; 2003. p. 54–74.

    Google Scholar 

  2. West JB. Diffusion. In: Respiratory physiology: the essentials. 9th ed. Baltimore: Wolters Kluwer Health/Lippincott Williams & Wilkins; 2012. p. 24–35.

    Google Scholar 

  3. De Vries J, Kessels BL, Drent M. Quality of life of idiopathic pulmonary fibrosis patients. Eur Respir J. 2001;17:954–61.

    Article  PubMed  Google Scholar 

  4. Swigris JJ, Kuschner WG, Jacobs SS, Wilson SR, Gould MK. Health-related quality of life in patients with idiopathic pulmonary fibrosis: a systematic review. Thorax. 2005;60:588–94.

    Article  PubMed Central  CAS  PubMed  Google Scholar 

  5. Nishiyama O, Taniguchi H, Kondoh Y, Kimura T, Ogawa T, Watanabe F, et al. Health-related quality of life in patients with idiopathic pulmonary fibrosis. What is the main contributing factor? Respir Med. 2005;99:408–14.

    Article  CAS  PubMed  Google Scholar 

  6. Kozu R, Jenkins S, Senjyu H. Evaluation of activity limitation in patients with idiopathic pulmonary fibrosis grouped according to Medical Research Council dyspnea grade. Arch Phys Ned Rehabil. 2014;95:950–5.

    Article  Google Scholar 

  7. Nishiyama O, Taniguchi H, Kondoh Y, Kimura T, Kato K, Ogawa T, et al. Dyspnoea at 6-min walk test in idiopathic pulmonary fibrosis: comparison with COPD. Respir Med. 2007;101:833–8.

    Article  PubMed  Google Scholar 

  8. Nishiyama O, Taniguchi H, Kondoh Y, Kimura T, Ogawa T, Watanabe F, et al. Quadriceps weakness is related to exercise capacity in idiopathic pulmonary fibrosis. Chest. 2005;127:2028–33.

    Article  PubMed  Google Scholar 

  9. Kozu R, Senjyu H, Jenkins SC, Mukae H, Sakamoto N, Kohno S. Differences in response to pulmonary rehabilitation in idiopathic pulmonary fibrosis and chronic obstructive pulmonary disease. Respiration. 2011;81:196–205.

    Article  PubMed  Google Scholar 

  10. Ryerson CJ, Berkeley J, Carrieri-Kohlman VL, Pantilat SZ, Landefeld CS, Collard HR. Depression and functional status are strongly associated with dyspnea in interstitial lung disease. Chest. 2011;139:609–16.

    Article  PubMed  Google Scholar 

  11. Beekman AT, Copeland JR, Prince MJ. Review of community prevalence of depression in later life. Br J Psychiatry. 1999;174:307–11.

    Article  CAS  PubMed  Google Scholar 

  12. Spruit MA, Singh SJ, Garvey C, ZuWallack R, Nici L, Rochester C, et al. An official American Thoracic Society/European Respiratory Society statement: key concepts and advances in pulmonary rehabilitation. Am J Respir Crit Care Med. 2013;188:e13–64.

    Article  PubMed  Google Scholar 

  13. Raghu G, Collard HR, Egan JJ, Martinez FJ, Behr J, Brown KK, 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;183:788–824.

    Article  PubMed  Google Scholar 

  14. Vainshelboim B, Oliveira J, Yehoshua L, Weiss I, Fox BD, Fruchter O, et al. Exercise training-based pulmonary rehabilitation program is clinically beneficial for idiopathic pulmonary fibrosis. Respiration. 2014;88:378–88.

    Article  PubMed  Google Scholar 

  15. Arizono S, Taniguchi H, Sakamoto K, Kondoh Y, Kimura T, Kataoka K, et al. Endurance time is the most responsive exercise measurement in idiopathic pulmonary fibrosis. Respir Care. 2014;59:1108–15.

    Article  PubMed  Google Scholar 

  16. Jackson RM, Gómez-Marín OW, Ramos CF, Sol CM, Cohen MI, Gaunaurd IA, et al. Exercise limitation in IPF patients: a randomized trial of pulmonary rehabilitation. Lung. 2014;192:367–76.

    Article  CAS  PubMed  Google Scholar 

  17. Holland AE, Hill CJ, Glaspole I, Goh N, McDonald CF. Predictors of benefit following pulmonary rehabilitation for interstitial lung disease. Respir Med. 2012;106:429–35.

    Article  PubMed  Google Scholar 

  18. Kozu R, Jenkins S, Senjyu H. Effect of disability level on response to pulmonary rehabilitation in patients with idiopathic pulmonary fibrosis. Respirology. 2011;16:1196–202.

    Article  PubMed  Google Scholar 

  19. Swigris JJ, Fairclough DL, Morrison M, Make B, Kozora E, Brown KK, et al. Benefits of pulmonary rehabilitation in idiopathic pulmonary fibrosis. Respir Care. 2011;56:783–9.

    Article  PubMed Central  PubMed  Google Scholar 

  20. Nishiyama O, Kondoh Y, Kimura T, Kato K, Kataoka K, Ogawa T, et al. Effects of pulmonary rehabilitation in patients with idiopathic pulmonary fibrosis. Respirology. 2008;13:394–9.

    Article  PubMed  Google Scholar 

  21. Dowman L, Hill CJ, Holland AE. Pulmonary rehabilitation for interstitial lung disease. Cochrane Database Syst Rev. 2014;10:CD006322.

    PubMed  Google Scholar 

  22. Swigris JJ, Wamboldt FS, Behr J, du Bois RM, King TE, Raghu G, et al. The 6 minute walk in idiopathic pulmonary fibrosis: longitudinal changes and minimum important difference. Thorax. 2010;65:173–7.

    Article  PubMed Central  PubMed  Google Scholar 

  23. Morrison DA, Stovall JR. Increased exercise capacity in hypoxemic patients after long-term oxygen therapy. Chest. 1992;102:542–50.

    Article  CAS  PubMed  Google Scholar 

  24. Hallstrand TS, Boitano LJ, Johnson WC, Spada CA, Hayes JG, Raghu G. The timed walk test as a measure of severity and survival in idiopathic pulmonary fibrosis. Eur Respir J. 2005;25:96–103.

    Article  CAS  PubMed  Google Scholar 

  25. Holland AE, Hill CJ, Conron M, Munro P, McDonald CF. Short term improvement in exercise capacity and symptoms following exercise training in interstitial lung disease. Thorax. 2008;63:549–54.

    Article  CAS  PubMed  Google Scholar 

  26. Ferreira A, Garvey C, Connors GL, Hilling L, Rigler J, Farrell S, et al. Pulmonary rehabilitation in interstitial lung disease: benefits and predictors of response. Chest. 2009;135:442–7.

    Article  PubMed  Google Scholar 

  27. Jaeschke R, Singer J, Guyatt GH. Measurement of health status. Ascertaining the minimal clinically important difference. Control Clin Trials. 1989;10:407–15.

    Article  CAS  PubMed  Google Scholar 

  28. Swigris JJ, Brown KK, Make BJ, Wamboldt FS. Pulmonary rehabilitation in idiopathic pulmonary fibrosis: a call for continued investigation. Respir Med. 2008;102:1675–80.

    Article  PubMed Central  PubMed  Google Scholar 

  29. Kenn K, Gloeckl R, Behr J. Pulmonary rehabilitation in patients with idiopathic pulmonary fibrosis–a review. Respiration. 2013;86:89–99.

    Article  CAS  PubMed  Google Scholar 

  30. Ryerson CJ, Cayou C, Topp F, Hilling L, Camp PG, Wilcox PG, et al. Pulmonary rehabilitation improves long-term outcomes in interstitial lung disease: a prospective cohort study. Respir Med. 2014;108:203–10.

    Article  PubMed  Google Scholar 

  31. Huppmann P, Sczepanski B, Boensch M, Winterkamp S, Schönheit-Kenn U, Neurohr C, et al. Effects of inpatient pulmonary rehabilitation in patients with interstitial lung disease. Eur Respir J. 2013;42:444–53.

    Article  PubMed  Google Scholar 

  32. Salhi B, Troosters T, Behaegel M, Joos G, Derom E. Effects of pulmonary rehabilitation in patients with restrictive lung diseases. Chest. 2010;137:273–9.

    Article  PubMed  Google Scholar 

  33. William W, Ryu JH, Schroeder DR. Idiopathic pulmonary fibrosis: impact of oxygen and colchicine, prednisone, or no therapy on survival. Am J Respir Crit Care Med. 2000;161:1172–8.

    Article  Google Scholar 

  34. Nocturnal Oxygen Therapy Trial Group. Continuous or nocturnal oxygen therapy in hypoxemic chronic obstructive lung disease: a clinical trial. Ann Intern Med. 1980;93:391–8.

    Google Scholar 

  35. Report of the Medical Research Council Working Party. Long term domiciliary oxygen therapy in chronic hypoxic cor pulmonale complicating chronic bronchitis and emphysema. Lancet. 1981;1:681–6.

    Google Scholar 

  36. Clark M, Cooper B, Singh S, Cooper M, Carr A, Hubbard R. A survey of nocturnal hypoxaemia and health related quality of life in patients with cryptogenic fibrosing alveolitis. Thorax. 2001;56:482–6.

    Article  PubMed Central  CAS  PubMed  Google Scholar 

  37. Pitsiou G, Papakosta D, Bouros D. Pulmonary hypertension in idiopathic pulmonary fibrosis: a review. Respiration. 2011;82:294–304.

    Article  PubMed  Google Scholar 

  38. McLaughlin VV, Archer SL, Badesch DB, Barst RJ, Farber HW, Lindner JR, et al. ACCF/AHA 2009 expert consensus document on pulmonary hypertension a report of the American College of Cardiology Foundation Task Force on Expert Consensus Documents and the American Heart Association developed in collaboration with the American College of Chest Physicians; American Thoracic Society, Inc.; and the Pulmonary Hypertension Association. J Am Coll Cardiol. 2009;53:1573–619.

    Article  PubMed  Google Scholar 

  39. Weitzenblum E, Ehrhart M, Rasaholinjanahary J, Hirth C. Pulmonary hemodynamics in idiopathic pulmonary fibrosis and other interstitial pulmonary diseases. Respiration. 1983;44:118–27.

    Article  CAS  PubMed  Google Scholar 

  40. Pouwels-Fry S, Pouwels S, Fournier C, Duchemin A, Tillie-Leblond I, Le Tourneau T, et al. Effects of oxygen on exercise-induced increase of pulmonary arterial pressure in idiopathic pulmonary fibrosis. Sarcoidosis Vasc Diffuse Lung Dis. 2008;25:133–9.

    CAS  PubMed  Google Scholar 

  41. Mallick S. Outcome of patients with idiopathic pulmonary fibrosis (IPF) ventilated in intensive care unit. Respir Med. 2008;102:1355–9.

    Article  PubMed  Google Scholar 

  42. Vianello A, Arcaro G, Battistella L, Pipitone E, Vio S, Concas A, et al. Noninvasive ventilation in the event of acute respiratory failure in patients with idiopathic pulmonary fibrosis. J Crit Care. 2014;29:562–7.

    Article  PubMed  Google Scholar 

  43. Yokoyama T, Kondoh Y, Taniguchi H, Kataoka K, Kato K, Nishiyama O, et al. Noninvasive ventilation in acute exacerbation of idiopathic pulmonary fibrosis. Intern Med. 2010;49:1509–14.

    Article  PubMed  Google Scholar 

  44. Kim DS, Park JH, Park BK, Lee JS, Nicholson AG, Colby T. Acute exacerbation of idiopathic pulmonary fibrosis: frequency and clinical features. Eur Respir J. 2006;27:143–50.

    Article  CAS  PubMed  Google Scholar 

  45. Schwabbauer N, Berg B, Blumenstock G, Haap M, Hetzel J, Riessen R. Nasal high-flow oxygen therapy in patients with hypoxic respiratory failure: effect on functional and subjective respiratory parameters compared to conventional oxygen therapy and non-invasive ventilation (NIV). BMC Anesthesiol. 2014;14:66.

    Article  PubMed Central  PubMed  Google Scholar 

  46. Bräunlich J, Beyer D, Mai D, Hammerschmidt S, Seyfarth HJ, Wirtz H. Effects of nasal high flow on ventilation in volunteers, COPD and idiopathic pulmonary fibrosis patients. Respiration. 2013;85:319–25.

    Article  PubMed  Google Scholar 

  47. O’Donnell DE, Sanii R, Younes M. Improvement in exercise endurance in patients with chronic airflow limitation using continuous positive airway pressure. Am Rev Respir Dis. 1988;138:1510–14.

    Article  PubMed  Google Scholar 

  48. Keilty SEJ, Ponte J, Fleming TA, Moxham J. Effect of inspiratory pressure support on exercise tolerance and breathlessness in patients with severe stable chronic obstructive pulmonary disease. Thorax. 1994;49:990–4.

    Article  PubMed Central  CAS  PubMed  Google Scholar 

  49. Van’t Hul A, Kwakkel G, Gosselink R. The acute effects of noninvasive ventilatory support during exercise on exercise endurance and dyspnea in patients with chronic obstructive pulmonary disease: a systematic review. J Cardiopulm Rehabil. 2002;22:290–7.

    Article  Google Scholar 

  50. Moderno EV, Yamaguti WP, Schettino GP, Kairalla RA, Martins MA, Carvalho CR, et al. Effects of proportional assisted ventilation on exercise performance in idiopathic pulmonary fibrosis patients. Respir Med. 2010;104:134–41.

    Article  CAS  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Yukihiro Umeda .

Editor information

Editors and Affiliations

Rights and permissions

Reprints and permissions

Copyright information

© 2016 Springer Japan

About this chapter

Cite this chapter

Umeda, Y., Ishizuka, T., Ishizaki, T. (2016). Non-pharmacological Therapy for IPF. In: Nakamura, H., Aoshiba, K. (eds) Idiopathic Pulmonary Fibrosis. Springer, Tokyo. https://doi.org/10.1007/978-4-431-55582-7_11

Download citation

  • DOI: https://doi.org/10.1007/978-4-431-55582-7_11

  • Publisher Name: Springer, Tokyo

  • Print ISBN: 978-4-431-55581-0

  • Online ISBN: 978-4-431-55582-7

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics