XIV.p
XIV - Pleural involvement
Pleuroparenchymal fibroelastosis (PPFE)
Last update 17/06/2026
See under Ix. A disease of the pleuropulmonary interface (see under Ix). PPFE has a predilection to involve the apical visceral pleural surface. More progressive and aggressive than the apical cap (Vt). Cases have been described in hematopoietic and lung transplant recipients (PMID 14627569, 23277524). PPFE may also occur as a complication of drugs or randomly and idiopathically (IPPFE). PPFE may overlap with upper lobe fibrosis
