Pulmonary rehabilitation for idiopathic pleuroparenchymal fibroelastosis: A retrospective study on its efficacy, feasibility, and safety.

Abstract

The beneficial effects of pulmonary rehabilitation (PR) for patients with idiopathic pleuroparenchymal fibroelastosis (IPPFE) remain unknown. This study aimed to examine the efficacy, feasibility, and safety of PR for IPPFE. We retrospectively investigated 25 patients with IPPFE referred for PR between April 2007 and March 2017. The PR mainly consisted of a 10-week exercise training program. The primary outcome was a change in 6-min walk distance (6MWD). Secondary outcomes included changes in dyspnea (transition dyspnea index [TDI]), anxiety and depression (hospital anxiety and depression scale [HADS]), and health-related quality of life (HRQoL) (St George's respiratory questionnaire [SGRQ]). Thirteen patients participated in the PR program (PRP). Recurrent pneumothorax was the most common reason for patients not participating in the PRP. Four patients discontinued the PRP due to the recurrence of pneumothorax, new onset of pneumomediastinum, stroke, and another reason, respectively. Nine patients completed the PRP. Significant improvement was observed in 6MWD (median [interquartile range], 90m [55-116m]; P=0.033). Clinically important improvements in the 6MWD, and TDI, HADS-anxiety, HADS-depression, and SGRQ total domain scores were observed in seven (78%), five (56%), four (44%), four (44%), and five (56%) of the nine patients, respectively. Patients with IPPFE benefited from PR in terms of exercise capacity, dyspnea, anxiety, depression, and HRQoL. Pneumothorax and pneumomediastinum may impede the implementation of a PRP for patients with IPPFE. While careful patient selection is required, PR may be an efficacious non-pharmacological approach for managing disabilities in patients with IPPFE.