Philippe Camus, M.D.

Dijon, France

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V.p

V - Pleural and/or pericardial 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

Causative drugs

2

Frequency - Incidence

Incidence of respiratory adverse event(s) due to the specific drug as assessed by number of reported/published cases in the literature. Red digits in stars indicate: 0 = Very rare, questionable signal · 1 = < 10 cases · 2 = 10-50 cases · 3 = 50–100 cases · 4 = 100-200 cases · 5 = >200 cases

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XI.b
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