The feasibility of pulmonary rehabilitation in patients with interstitial lung diseases including patients with combined pulmonary fibrosis and emphysema

Abstract

Introduction: Interstitial lung diseases (ILD) are characterized by interstitial inflammation or fibrosis, leading to impaired gas exchange, shortness of breath, decreased exercise tolerance, and reduced quality of life. Combined pulmonary fibrosis and emphysema (CPFE) is the co-existing presence of pulmonary fibrosis and emphysema. Data describing the experience of tailored pulmonary rehabilitation program (PRP) in people with ILD is rare and in particular in patients with CPFE are lacking. This PhD aimed to determine the characteristics and prognosis of patients with CPFE in a United Kingdom cohort, and to assess the feasibility of inspiratory muscle training (IMT) as part of a PRP for patients with ILD including patients with CPFE. Methods: A five-year retrospective single centre study was conducted at the regional ILD clinic. Then a feasibility study with a randomized controlled trial design was conducted Patients were randomized into intervention group IMT + PRP and a control group PRP only. The IMT was performed using POWERBreathe® twice daily. The PRP consisted of one session a week for 6-8 weeks. Results: Retrospective study showed that 203 patients with CPFE were diagnosed. Mean age and Body Mass Index (BMI) for patients with CPFE were 72 years (SD = 8.7), and 28.1 kg/m² (SD = 4.4) respectively. Median survival time for patients with idiopathic pulmonary fibrosis (IPF) subtype CPFE n= 93 was 3.2 years (2.1-4.2).. Kaplan Meier analysis showed statistically significant differences between Gender at birth-Age-Physiology (GAP) stages with a p-value of 0.012 in patients with IPF subtype CPFE. PRP had an attendance and completion rates of mean of 87% and 64% respectively. No side effects were reported during the study. The maximum inspiratory pressure (MIP) improved in all participants. Conclusion: Patients with CPFE were relatively old, majority male, with a history of smoking, and had poor prognosis. The GAP index and staging system demonstrated prognostic capability in patients with IPF subtype CPFE. At least half the patients with CPFE were not referred to PRP, indicating low referral rates. A tailored PRP program was feasible and well received in patients with CPFE, indicating that this was a viable and beneficial treatment option for patients with CPFE where therapeutic options are limited.