V.p
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
Causative drugs
2
Alkylating agents
I.a
I - Interstitial/parenchymal lung disease
I.a - Pneumonitis (ILD), acute and/or severe (may cause ARDS) I.b I - Interstitial/parenchymal lung disease
I.b - Pneumonitis (ILD) I.g I - Interstitial/parenchymal lung disease
I.g - Pulmonary fibrosis I.x I - Interstitial/parenchymal lung disease
I.x - Pleuroparenchymal fibroelastosis (PPFE) II.b II - Pulmonary edema - Acute lung injury - ARDS
II.b - ARDS - Acute lung injury V.p V - Pleural and/or pericardial involvement
V.p - Pleuroparenchymal fibroelastosis (PPFE) VI.c VI - Pulmonary vasculopathies
VI.c - Pulmonary veno-occlusive disease XV.ak XV - Pathology
XV.ak - Path: Pulmonary veno-occlusive disease (PVOD) XV.ap XV - Pathology
XV.ap - Path: Pleuroparenchymal fibrosis/fibroelastosis (PPFE)
I.a - Pneumonitis (ILD), acute and/or severe (may cause ARDS) I.b I - Interstitial/parenchymal lung disease
I.b - Pneumonitis (ILD) I.g I - Interstitial/parenchymal lung disease
I.g - Pulmonary fibrosis I.x I - Interstitial/parenchymal lung disease
I.x - Pleuroparenchymal fibroelastosis (PPFE) II.b II - Pulmonary edema - Acute lung injury - ARDS
II.b - ARDS - Acute lung injury V.p V - Pleural and/or pericardial involvement
V.p - Pleuroparenchymal fibroelastosis (PPFE) VI.c VI - Pulmonary vasculopathies
VI.c - Pulmonary veno-occlusive disease XV.ak XV - Pathology
XV.ak - Path: Pulmonary veno-occlusive disease (PVOD) XV.ap XV - Pathology
XV.ap - Path: Pleuroparenchymal fibrosis/fibroelastosis (PPFE)
2
Bleomycin
I.a
I - Interstitial/parenchymal lung disease
I.a - Pneumonitis (ILD), acute and/or severe (may cause 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 pattern (an area or areas of consolidation on imaging) 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.p I - Interstitial/parenchymal lung disease
I.p - Fatal pneumonitis (ILD) I.v I - Interstitial/parenchymal lung disease
I.v - Abnormal lung function/pulmonary physiology (PFTs) without necessarily imaging or clinical evidence I.w I - Interstitial/parenchymal lung disease
I.w - Rapidly progressive ILD/pulmonary fibrosis (Hamman-Rich syndrome) II.b II - Pulmonary edema - Acute lung injury - ARDS
II.b - ARDS - Acute lung injury II.u II - Pulmonary edema - Acute lung injury - ARDS
II.u - Acute respiratory failure (e.g. from ARDS, ILD, PIE, OP, pulmonary edema or bronchospasm) requiring ECMO V.f V - Pleural and/or pericardial involvement
V.f - Pneumothorax V.p V - Pleural and/or pericardial involvement
V.p - Pleuroparenchymal fibroelastosis (PPFE) V.z V - Pleural and/or pericardial involvement
V.z - Pneumothorax (associated or secondary to drug-induced ILD) VI.c VI - Pulmonary vasculopathies
VI.c - Pulmonary veno-occlusive disease VI.f VI - Pulmonary vasculopathies
VI.f - 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 or subacute), lone or prominent XI.c XI - Miscellaneous
XI.c - Pleuritic chest pain XV.a XV - Pathology
XV.a - Path: NSIP-cellular pattern (see also Ia, Ib) XV.b XV - Pathology
XV.b - Path: Eosinophilic pneumonia (subacute or acute) (see also Ic) XV.c XV - Pathology
XV.c - Path: Organizing pneumonia (OP/BOOP) pattern (see also Id) XV.d XV - Pathology
XV.d - Path: Acute fibrinous organizing pneumonia (AFOP-pattern) (see also If) XV.f XV - Pathology
XV.f - Path: Diffuse alveolar damage (DAD-pattern) (see also IL) XV.h XV - Pathology
XV.h - Path: NSIP-fibrotic pattern XV.i XV - Pathology
XV.i - Path: Pneumocyte atypia (reactive epithelial cells) (a.k.a. the "Napoleon Hat" sign) XV.j XV - Pathology
XV.j - Path: Pulmonary fibrosis (UIP-pattern) XV.l XV - Pathology
XV.l - Path: Pulmonary fibrosis (not otherwise specified or unclassifiable) XV.ax XV - Pathology
XV.ax - Path: Focal organizing pneumonia XV.be XV - Pathology
XV.be - Path: Interstitial pulmonary edema XV.bf XV - Pathology
XV.bf - Path: Pulmonary necrotizing granuloma or granulomas XVI.a XVI - Imaging
XVI.a - Imaging: Diffuse haze XVI.b XVI - Imaging
XVI.b - Imaging: Ground-glass opacities (GGO) / shadowing 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 nodules XVI.ab XVI - Imaging
XVI.ab - Imaging: Cavitating/cavitary lung nodule, mass or 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: An area or areas of avid tracer uptake in the lung/chest on 18F-PET scan XVIII.j XVIII - Distinctive patterns - 'Eye-catchers'
XVIII.j - Eye-catcher: Avid lung uptake on 18F-PET-CT XIX.b XIX - Cytological, biochemical features of/in BAL, pleural fluid or FNA
XIX.b - BAL: An excess proportion of neutrophils XXIV.a XXIV - Veterinary medicine
XXIV.a - Veterinary: Pneumonitis - Interstitial lung disease XXIV.b XXIV - Veterinary medicine
XXIV.b - Veterinary: Pulmonary fibrosis
I.a - Pneumonitis (ILD), acute and/or severe (may cause 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 pattern (an area or areas of consolidation on imaging) 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.p I - Interstitial/parenchymal lung disease
I.p - Fatal pneumonitis (ILD) I.v I - Interstitial/parenchymal lung disease
I.v - Abnormal lung function/pulmonary physiology (PFTs) without necessarily imaging or clinical evidence I.w I - Interstitial/parenchymal lung disease
I.w - Rapidly progressive ILD/pulmonary fibrosis (Hamman-Rich syndrome) II.b II - Pulmonary edema - Acute lung injury - ARDS
II.b - ARDS - Acute lung injury II.u II - Pulmonary edema - Acute lung injury - ARDS
II.u - Acute respiratory failure (e.g. from ARDS, ILD, PIE, OP, pulmonary edema or bronchospasm) requiring ECMO V.f V - Pleural and/or pericardial involvement
V.f - Pneumothorax V.p V - Pleural and/or pericardial involvement
V.p - Pleuroparenchymal fibroelastosis (PPFE) V.z V - Pleural and/or pericardial involvement
V.z - Pneumothorax (associated or secondary to drug-induced ILD) VI.c VI - Pulmonary vasculopathies
VI.c - Pulmonary veno-occlusive disease VI.f VI - Pulmonary vasculopathies
VI.f - 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 or subacute), lone or prominent XI.c XI - Miscellaneous
XI.c - Pleuritic chest pain XV.a XV - Pathology
XV.a - Path: NSIP-cellular pattern (see also Ia, Ib) XV.b XV - Pathology
XV.b - Path: Eosinophilic pneumonia (subacute or acute) (see also Ic) XV.c XV - Pathology
XV.c - Path: Organizing pneumonia (OP/BOOP) pattern (see also Id) XV.d XV - Pathology
XV.d - Path: Acute fibrinous organizing pneumonia (AFOP-pattern) (see also If) XV.f XV - Pathology
XV.f - Path: Diffuse alveolar damage (DAD-pattern) (see also IL) XV.h XV - Pathology
XV.h - Path: NSIP-fibrotic pattern XV.i XV - Pathology
XV.i - Path: Pneumocyte atypia (reactive epithelial cells) (a.k.a. the "Napoleon Hat" sign) XV.j XV - Pathology
XV.j - Path: Pulmonary fibrosis (UIP-pattern) XV.l XV - Pathology
XV.l - Path: Pulmonary fibrosis (not otherwise specified or unclassifiable) XV.ax XV - Pathology
XV.ax - Path: Focal organizing pneumonia XV.be XV - Pathology
XV.be - Path: Interstitial pulmonary edema XV.bf XV - Pathology
XV.bf - Path: Pulmonary necrotizing granuloma or granulomas XVI.a XVI - Imaging
XVI.a - Imaging: Diffuse haze XVI.b XVI - Imaging
XVI.b - Imaging: Ground-glass opacities (GGO) / shadowing 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 nodules XVI.ab XVI - Imaging
XVI.ab - Imaging: Cavitating/cavitary lung nodule, mass or 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: An area or areas of avid tracer uptake in the lung/chest on 18F-PET scan XVIII.j XVIII - Distinctive patterns - 'Eye-catchers'
XVIII.j - Eye-catcher: Avid lung uptake on 18F-PET-CT XIX.b XIX - Cytological, biochemical features of/in BAL, pleural fluid or FNA
XIX.b - BAL: An excess proportion of neutrophils XXIV.a XXIV - Veterinary medicine
XXIV.a - Veterinary: Pneumonitis - Interstitial lung disease XXIV.b XXIV - Veterinary medicine
XXIV.b - Veterinary: Pulmonary fibrosis
1