Pleuroparenchymal fibroelastosis (PPFE)

(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 bilaterally. The corresponding pleura appears to be thickened. Pneumothorax. Biopsy extreme caution required as pneumothorax and prolonged air leak is a usual complication

Last update : 01/02/2016

Causative drugs