XIV - Pleural involvement
Pleuroparenchymal fibroelastosis (PPFE)
Last update 17/06/2026
(Fr: fibroelastose pleuropulmonaire - Fibrose biapicale). A disease of the pleuropulmonary interface (see also under Vp). Spontaneous pneumothorax often seen as a complication. Formerly called 'Upper Lobe Fibrosis' - Usually localizes in the apices and upper halves bilaterally. The pleura en-face appears to be thickened. Pneumothorax common and difficult to treat. Biopsy: extreme caution required as pneumothorax and prolonged air leak is a common complication. Not all cases are histopathologically documented. Pleural biopsy may be detrimental with no consistent benefit in terms of management (PMID 25082907). Some case reports, despite containing the label PPFE in title and or text, may merely correspond to a 'clinical-imaging pattern consistent with pleuroparenchymal fibroelastosis' (i.e. PPFE sine pathology)
