Pleuroparenchymal fibroelastosis (PPFE)

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

Last update : 20/09/2015

Causative drugs



I.a I - Interstitial/parenchymal lung disease
I.a - Pneumonitis (ILD), acute, severe (see also under ARDS)
I.b I - Interstitial/parenchymal lung disease
I.b - Pneumonitis (ILD)
I.c I - Interstitial/parenchymal lung disease
I.c - Eosinophilic pneumonia (pulmonary infiltrates and eosinophilia)
I.d I - Interstitial/parenchymal lung disease
I.d - Organizing pneumonia (A pattern consistent with)
I.f I - Interstitial/parenchymal lung disease
I.f - Acute fibrinous organizing pneumonia (AFOP)
I.g I - Interstitial/parenchymal lung disease
I.g - Pulmonary fibrosis
I.k I - Interstitial/parenchymal lung disease
I.k - Lung nodule or nodules
I.l I - Interstitial/parenchymal lung disease
I.l - Diffuse alveolar damage (DAD) (see alsoo under IIb and XVf)
I.v I - Interstitial/parenchymal lung disease
I.v - Subclinical changes in lung function/PFT
I.w I - Interstitial/parenchymal lung disease
I.w - Rapidly progressive ILD or pulmonary fibrosis (Hamman-Rich syndrome)
II.b II - Pulmonary edema - ARDS - Acute respiratory failure
V.f V - Pleural and/or pericardial involvement
V.f - Pneumothorax
V.p V - Pleural and/or pericardial involvement
V.p - Pleuroparenchymal fibroelastosis (PPFE)
VI.c VI - Pulmonary vasculopathy
VI.c - Pulmonary veno-occlusive disease
VI.f VI - Pulmonary vasculopathy
VI.f - The hemolytic and uremic syndrome (HUS). See also under Xaf
VII.e VII - Mediastinal involvement
VII.e - Granulomatous mediastinal lymphadenopathy
VII.h VII - Mediastinal involvement
VII.h - Pneumomediastinum
XI.b XI - Miscellaneous
XI.b - Chest pain (acute - subacute)
XI.c XI - Miscellaneous
XI.c - Pleuritic chest pain (see also under Vi)
XV.a XV - Pulmonary pathology
XV.a - Path: Cellular NSIP pattern (see also Ia, Ib)
XV.b XV - Pulmonary pathology
XV.b - Path: Eosinophilic pneumonia (subacute or acute) (see also Ic)
XV.c XV - Pulmonary pathology
XV.c - Path: Organizing pneumonia (OP/BOOP) pattern (see also Id)
XV.d XV - Pulmonary pathology
XV.d - Path: Acute fibrinous organizing pneumonia (AFOP-pattern) (see also If)
XV.f XV - Pulmonary pathology
XV.f - Path: Diffuse alveolar damage (DAD-pattern) (see also IL)
XV.h XV - Pulmonary pathology
XV.h - Path: Fibrotic NSIP pattern
XV.i XV - Pulmonary pathology
XV.i - Path: Pneumocyte atypia (reactive epithelial cells)
XV.ax XV - Pulmonary pathology
XV.ax - Path: Focal organizing pneumonia
XV.be XV - Pulmonary pathology
XV.be - Path: Interstitial pulmonary edema
XV.bf XV - Pulmonary pathology
XV.bf - Path: Pulmonary necrotizing granuloma
XVI.a XVI - Imaging
XVI.a - Imaging: Diffuse haze
XVI.b XVI - Imaging
XVI.b - Imaging: Ground-glass shadowing/opacities (GGO)
XVI.e XVI - Imaging
XVI.e - Imaging: Pulmonary opacities with a subpleural distribution
XVI.k XVI - Imaging
XVI.k - Imaging: An area or areas of consolidation
XVI.w XVI - Imaging
XVI.w - Imaging: Lung nodule or several nodules
XVI.ab XVI - Imaging
XVI.ab - Imaging: Cavitating lung nodules (see also Iq, XIs, XIIi, XVIaa and XVIIp)
XVI.af XVI - Imaging
XVI.af - Imaging: Lung cysts or bullae (see also XVI ah/bf)
XVI.bl XVI - Imaging
XVI.bl - Imaging: Avid lung uptake on PET-scan
XVIII.j XVIII - Distinctive patterns - 'Eye-catchers'
XVIII.j - Eye-catcher: Avid lung uptake on PET-scan
XIX.b XIX - Cytology, biochemistry of BAL, pleural fluid or FNA
XIX.b - BAL: An excess proportion of neutrophils