The Orphalung Network

Antifibrotic therapy in patients with pleuroparenchymal fibroelastosis: a multicentric observational study

Pleuroparenchymal fibroelastosis (PPFE) is a rare interstitial lung disease characterized by upper-lobe predominant pleural and subpleural fibrosis and associated with poor prognosis. No evidence-based medical therapy is currently established, and lung transplantation remains the...