title: Histological evolution of pleuroparenchymal fibroelastosis.
abstract: OBJECTIVE
  To investigate the histological evolution in the development of pleuroparenchymal fibroelastosis (PPFE).
  
  
  RESULTS
  We examined four patients who had undergone surgical lung biopsy twice, or who had undergone surgical lung biopsy and had been autopsied, and in whom the histological diagnosis of the first biopsy was not PPFE, but the diagnosis of the second biopsy or of the autopsy was PPFE. The histological patterns of the first biopsy were cellular and fibrotic interstitial pneumonia, cellular interstitial pneumonia (CIP) with organizing pneumonia, CIP with granulomas and acute lung injury in cases 1, 2, 3, and 4, respectively. Septal elastosis was already present in the non-specific interstitial pneumonia-like histology of case 1, but a few additional years were necessary to reach consolidated subpleural fibroelastosis. In case 3, subpleural fibroelastosis was already present in the first biopsy, but only to a small extent. Twelve years later, it was replaced by a long band of fibroelastosis. The septal inflammation and fibrosis and airspace organization observed in the first biopsies were replaced by less cellular subpleural fibroelastosis within 3-12 years.
  
  
  CONCLUSIONS
  Interstitial inflammation or acute lung injury may be an initial step in the development of PPFE.
authors.affiliation: Department of Respiratory Medicine, Fukuoka University School of Medicine, Fukuoka, Japan.
authors.forename: Takako
authors.initials: T
authors.lastname: Hirota
authors.forename: Yuji
authors.initials: Y
authors.lastname: Yoshida
authors.forename: Yasuhiko
authors.initials: Y
authors.lastname: Kitasato
authors.forename: Michihiro
authors.initials: M
authors.lastname: Yoshimi
authors.forename: Takaomi
authors.initials: T
authors.lastname: Koga
authors.forename: Nobuko
authors.initials: N
authors.lastname: Tsuruta
authors.forename: Masato
authors.initials: M
authors.lastname: Minami
authors.forename: Taishi
authors.initials: T
authors.lastname: Harada
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Ishii
authors.forename: Masaki
authors.initials: M
authors.lastname: Fujita
authors.forename: Kazuki
authors.initials: K
authors.lastname: Nabeshima
authors.forename: Nobuhiko
authors.initials: N
authors.lastname: Nagata
authors.forename: Kentaro
authors.initials: K
authors.lastname: Watanabe
mesh_terms: D000328:Adult; D000368:Aged; D018450:Disease Progression; D005260:Female; D006801:Humans; D008168:Lung / Q000473:pathology*; D017563:Lung Diseases, Interstitial / Q000473:pathology*; D008297:Male; D008875:Middle Aged; D011658:Pulmonary Fibrosis / Q000473:pathology*
keywords: acute lung injury; cellular interstitial pneumonia; idiopathic interstitial pneumonia; idiopathic pulmonary fibrosis; organizing pneumonia; pleuroparenchymal fibroelastosis; pulmonary upper lobe fibrosis