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.
Access this chapter
Tax calculation will be finalised at checkout
Purchases are for personal use only
References
Schwarz MI, King Jr TE. Physiology of interstitial lung disease. In: Interstitial lung disease. 4th ed. London: BC Decker Inc; 2003. p. 54–74.
West JB. Diffusion. In: Respiratory physiology: the essentials. 9th ed. Baltimore: Wolters Kluwer Health/Lippincott Williams & Wilkins; 2012. p. 24–35.
De Vries J, Kessels BL, Drent M. Quality of life of idiopathic pulmonary fibrosis patients. Eur Respir J. 2001;17:954–61.
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.
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.
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.
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.
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.
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.
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.
Beekman AT, Copeland JR, Prince MJ. Review of community prevalence of depression in later life. Br J Psychiatry. 1999;174:307–11.
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.
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.
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.
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.
Jackson RM, GĂłmez-MarĂn 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.
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.
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.
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.
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.
Dowman L, Hill CJ, Holland AE. Pulmonary rehabilitation for interstitial lung disease. Cochrane Database Syst Rev. 2014;10:CD006322.
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.
Morrison DA, Stovall JR. Increased exercise capacity in hypoxemic patients after long-term oxygen therapy. Chest. 1992;102:542–50.
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.
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.
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.
Jaeschke R, Singer J, Guyatt GH. Measurement of health status. Ascertaining the minimal clinically important difference. Control Clin Trials. 1989;10:407–15.
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.
Kenn K, Gloeckl R, Behr J. Pulmonary rehabilitation in patients with idiopathic pulmonary fibrosis–a review. Respiration. 2013;86:89–99.
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.
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.
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.
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.
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.
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.
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.
Pitsiou G, Papakosta D, Bouros D. Pulmonary hypertension in idiopathic pulmonary fibrosis: a review. Respiration. 2011;82:294–304.
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.
Weitzenblum E, Ehrhart M, Rasaholinjanahary J, Hirth C. Pulmonary hemodynamics in idiopathic pulmonary fibrosis and other interstitial pulmonary diseases. Respiration. 1983;44:118–27.
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.
Mallick S. Outcome of patients with idiopathic pulmonary fibrosis (IPF) ventilated in intensive care unit. Respir Med. 2008;102:1355–9.
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.
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.
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.
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.
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.
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.
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.
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.
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.
Author information
Authors and Affiliations
Corresponding author
Editor information
Editors and Affiliations
Rights 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)