Pleuroparenchymal fibroelastosis: from diagnosis to pulmonary rehabilitation

Abstract

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.