patient care perspectives

Pulmonary Rehabilitation in Interstitial Lung Disease

by Charles D. Burger, MD

Overview

Pulmonary rehabilitation is a viable treatment for many patients with interstitial lung disease (ILD), with benefits that may include increased exercise capacity, reduction of dyspnea, and a better quality of life. Still, for many patients with ILD, disease progression continues, and therapeutic advancements are greatly needed.

Expert Commentary

Charles D. Burger, MD

Professor of Medicine, Mayo Clinic College of Medicine
Consultant, Pulmonary, Allergy, Sleep, and Critical Care Medicine
Medical Director, Pulmonary Hypertension Clinic
Medical Director, Lymphangioleiomyomatosis Clinic
Mayo Clinic
Jacksonville, FL

“Strong consideration should be given as to whether pulmonary rehabilitation might benefit a patient with ILD. The majority of the patients seem to obtain a benefit, even if it is only short-term.”

Charles D. Burger, MD

Cardiopulmonary rehabilitation can provide multiple benefits to patients with chronic lung disease, including ILD. Such rehabilitation begins with a detailed assessment of the patient’s functional status to determine which interventions would help that individual the most. These interventions include education and instruction on the most appropriate use of and level of flow of their oxygen, proper use of inhalers, breathing techniques, and/or exercises. The ultimate goal is to increase the patient’s functional exercise capacity. Several fundamentals must be in place, of course, such as having preserved oxygen-carrying levels of hemoglobin and a heart and lungs that work together properly. Good conditioning is a critical aspect of rehabilitation in that the efficient use of oxygen in the peripheral musculature is even more important in patients with impaired cardiopulmonary function. Conditioning is thus a potential compensatory mechanism that can improve the individual’s exercise capacity, reduce their levels of dyspnea, and improve their quality of life. 

Traditionally, pulmonary rehabilitation was developed with a focus on chronic obstructive pulmonary disease, but it has since been used in patients with ILD, who have also been shown to benefit. A 2014 Cochrane Database of Systematic Reviews analysis examined trials of pulmonary rehabilitation in ILD, where the weighted improvement in the 6-minute walk distance was quite significant (ie, approximately 44 meters). Rehabilitation also resulted in improvements in oxygen consumption (VO2) peak, an important measure of overall exercise capacity, as well as reductions in dyspnea and improvements in quality of life. A 2020 review of 14 studies, distilled from more than 1600 articles on idiopathic pulmonary fibrosis or ILD and pulmonary rehabilitation, was published in the Journal of Thoracic Disease, also demonstrating improvement in those 3 important parameters: exercise capacity, reduction in dyspnea, and quality of life. 

Several factors are involved when considering whether a patient with ILD might be a good candidate for pulmonary rehabilitation. One relates to the patient’s willingness to undergo rehabilitation and/or the clinician’s success in motivating a reluctant individual to consider it. Additionally, some degree of disease stability is needed before a patient can be expected to successfully progress through a multiweek program. If the treatment includes medical therapies such as antifibrotic agents, the patient should be tolerating the therapy and, hopefully, demonstrating a flattening of the rate of deterioration. Another consideration is whether the patient is a candidate for lung transplantation in that pulmonary rehabilitation is a universal prerequisite among lung transplant centers. Lung transplant specialists want patients to maximize their conditioning and functional status prior to transplantation. Pulmonary rehabilitation and lung transplant evaluation can occur either sequentially or in parallel. The decision is often driven by the patient’s rate of decline and response, or lack thereof, to antifibrotic agents and their requirement for supplemental oxygen. A patient with ILD whose condition is not quite severe enough yet to be considered for transplant may still be referred for pulmonary rehabilitation.

In conclusion, strong consideration should be given as to whether pulmonary rehabilitation might benefit a patient with ILD. The majority of the patients seem to obtain a benefit, even if it is only short-term. Still, disease progression continues in many patients, and therapeutic advancements are greatly needed.

References

Dowman L, Hill CJ, Holland AE. Pulmonary rehabilitation for interstitial lung disease. Cochrane Database Syst Rev. 2014;(10):CD006322. doi:10.1002/14651858.CD006322.pub3

Gomes-Neto M, Silva CM, Ezequiel D, Conceição CS, Saquetto M, Machado AS. Impact of pulmonary rehabilitation on exercise tolerance and quality of life in patients with idiopathic pulmonary fibrosis: a systematic review and meta-analysis. J Cardiopulm Rehabil Prev. 2018;38(5):273-278. doi:10.1097/HCR.0000000000000273

Hanada M, Kasawara KT, Mathur S, et al. Aerobic and breathing exercises improve dyspnea, exercise capacity and quality of life in idiopathic pulmonary fibrosis patients: systematic review and meta-analysis. J Thorac Dis. 2020;12(3):1041‐1055. doi:10.21037/jtd.2019.12.27

Huppmann P, Sczepanski B, Boensch M, et al. Effects of inpatient pulmonary rehabilitation in patients with interstitial lung disease. Eur Respir J. 2013;42(2):444-453. doi:10.1183/09031936.00081512

Loe H, Nes BM, Wisløff U. Predicting VO2peak from submaximal- and peak exercise models: the HUNT 3 Fitness Study, Norway. PLoS One. 2016;11(1):e0144873. doi:10.1371/journal.pone.0144873

Wytrychowski K, Hans-Wytrychowska A, Piesiak P, Majewska-Pulsakowska M, Rożek-Piechura K. Pulmonary rehabilitation in interstitial lung diseases: a review of the literature. Adv Clin Exp Med. 2020;29(2):257‐264. doi:10.17219/acem/115238

More in Interstitial Lung Disease

Thumb

Interstitial Lung Disease

Clarified Hemodynamic Definitions of WHO Group 3 Pulmonary Hypertension

Clinical Topic Updates by Steven D. Nathan, MD

The updated hemodynamic criteria for pulmonary hypertension (PH) could lead to the earlier detection and treatment of patients with World Health Or...READ MORE

Thumb

Interstitial Lung Disease

Research on Pulmonary Hypertension–Specific Therapeutics for Patients With Chronic Lung Disease

Clinical Topic Updates by Steven D. Nathan, MD

Research continues to unfold on possible new treatments for pulmonary hypertension (PH) in patients with chronic lung disease (CLD), including thos...READ MORE

Thumb

Interstitial Lung Disease

Interstitial Lung Disease: Overcoming the Drivers of Poor Outcomes

Patient Care Perspectives by Steven D. Nathan, MD

Disease trajectories and outcomes in interstitial lung disease (ILD) are shaped by factors that include progressive fibrosis, comorbidities, and th...READ MORE

More In Pulmonology

Interstitial Lung Disease

Present And Future Therapies For Interstitial Lung Disease And Associated Pulmonary Hypertension

Expert Roundtables by Charles D. Burger, MD; Steven D. Nathan, MD; and Justin Oldham, MD, MS

Interstitial Lung Disease

Idiopathic Pulmonary Fibrosis: Challenges And Opportunities In Diagnosis And Treatment

Expert Roundtables by Charles D. Burger, MD; Steven D. Nathan, MD; and Justin Oldham, MD, MS

Interstitial Lung Disease

Improving Exercise Tolerance In Patients With Idiopathic Interstitial Pneumonias

Patient Care Perspectives by Justin Oldham, MD, MS