title: Pleuroparenchymal fibroelastosis: a rare interstitial lung disease.
abstract: Pleuroparenchymal fibroelastosis (PPFE) is a newly described form of interstitial lung disease that originates in the upper lung zones and typically progresses to involve the entire lung. The disease may be idiopathic but is often associated with other pre- or coexisting conditions. Pneumothorax is a common complication and can occur at presentation or at other times during the course of the disease. Pathologically, interstitial fibrosis takes the form of a dense consolidation with some preservation of alveolar septal outlines and demonstrates a distinctly abrupt interface with residual normal lung. Unrecognized cases of PPFE may be incorrectly diagnosed as sarcoidosis, atypical idiopathic pulmonary fibrosis, or other unclassifiable interstitial pneumonias.
authors.affiliation: Department of Pathology and Laboratory Medicine, Vancouver General Hospital Vancouver, British Columbia, Canada ; University of British Columbia Vancouver, British Columbia, Canada.
authors.forename: John C
authors.initials: JC
authors.lastname: English
authors.affiliation: University of British Columbia Vancouver, British Columbia, Canada ; Department of Radiology, Vancouver General Hospital Vancouver, British Columbia, Canada.
authors.forename: John R
authors.initials: JR
authors.lastname: Mayo
authors.affiliation: University of British Columbia Vancouver, British Columbia, Canada ; Division of Respirology, Department of Medicine, Vancouver General Hospital Vancouver, British Columbia, Canada.
authors.forename: Robert
authors.initials: R
authors.lastname: Levy
authors.affiliation: University of British Columbia Vancouver, British Columbia, Canada ; Division of Thoracic Surgery, Department of Surgery, Vancouver General Hospital Vancouver, British Columbia, Canada.
authors.forename: John
authors.initials: J
authors.lastname: Yee
authors.affiliation: Department of Pathology, Mayo Clinic Arizona Scottsdale, Arizona ; Mayo Clinic Medical School Scottsdale, Arizona.
authors.forename: Kevin O
authors.initials: KO
authors.lastname: Leslie
keywords: Pleuroparenchymal fibroelastosis (PPFE)