Pulmonary rehabilitation and noninvasive ventilation in patients with hypercapnic interstitial lung disease.

BACKGROUND: Pulmonary rehabilitation (PR) has a positive impact on functional status and quality of life in patients with interstitial lung disease (ILD).
OBJECTIVES: This study investigated the effects of PR in hypercapnic ILD patients receiving nighttime noninvasive positive pressure ventilation (NPPV).
METHODS: Consecutive ILD patients referred to a specialized inpatient PR center were included. All participated in a PR program. Those with hypercapnia received NPPV (NPPV group; n = 29); the remaining patients served as comparison group (n = 319).
RESULTS: PR improved the 6-min walk distance by 64.4 ± 67.1 m versus baseline (p < 0.0001) in NPPV patients and by 43.2 ± 55.1 m (p < 0.0001) in the comparison group (difference 21.1 m, 95% confidence interval 0.5-41.8; p = 0.045). There was no change in total lung capacity during PR in NPPV recipients or the comparison group. Forced vital capacity significantly increased from baseline in the comparison, but not the NPPV group. NPPV recipients were significantly more likely than the comparison group to have improved dyspnea during PR (p = 0.049). There was no improvement in the 36-item Short Form (SF-36) physical component score in the NPPV group after PR, but there was in the comparison group. PR improved the SF-36 mental component score versus baseline in both groups.
CONCLUSION: An individually tailored PR plus nighttime NPPV appears feasible in hypercapnic ILD patients and significantly improves exercise capacity and quality of life.