Pleuroparenchymal fibroelastosis (PPFE) is an uncommon disease of which diagnosis should be established multidisciplinary fashion and has no effective medical therapy. Pulmonary rehabilitation (PR) can be applied, but lung transplantation is the only therapeutic option. There have been few reported cases or studies showing the efficacy of PR in patients with PPFE in the literature. We present our multidisciplinary PR programme including confirmation of the diagnosis and a structured follow-up programme in two PPFE patients. In both cases, after multidisciplinary PR the diagnoses were confirmed and body composition, quality of life, exercise capacity and psychological status improved and some improvements preserved for 6–12 months. They underwent lung transplantation about 2 years after PR. Patients with PPFE should be directed and encouraged to participate in comprehensive multidisciplinary PR programmes. Long-term structured follow-up programmes could preserve the improvements, increase adherence and save time while waiting on the transplant list.
- interstitial lung disease
- physiotherapy (rehabilitation)
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Contributors Conceptualisation: IC and PE; Methodology: IC, PE and ND. Formal analysis: IC and PE; Data curation: IC, PE and ND; Software: IC and ND. Investigation: IC and PE; Writing-original draft preparation: IC, PE and ND; Writing-review and editing: IC and PE. Approval of final manuscript: all authors.
Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.
Competing interests None declared.
Provenance and peer review Not commissioned; externally peer reviewed.
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