title: Idiopathic pleuroparenchymal fibroelastosis: consideration of a clinicopathological entity in a series of Japanese patients.
abstract: BACKGROUND
  Idiopathic pleuroparenchymal fibroelastosis (IPPFE) is a recently reported group of disorders characterized by fibrotic thickening of the pleural and subpleural parenchyma predominantly in the upper lobes. We report five Japanese cases fulfilling the criteria of IPPFE and address whether it should be considered a separate clinicopathologic entity. And this study was an attempt to identify features in common between IPPFE and previously described idiopathic upper lobe fibrosis (IPUF), allowing IPPFE to be considered as a distinct entity in our Japanese series.
  
  
  METHODS
  Five consecutive cases of idiopathic interstitial lung disease confirmed as IPPFE by surgical lung biopsy were studied.
  
  
  RESULTS
  There were four males and one female, aged 70±2.76 yr. No associated disorder or presumed cause was found in any case. Lung function tests found a restrictive ventilatory defect (4/5) and/or impairment of DLco (4/5). Chest X-ray showed marked apical pleural thickening in all cases. Computed tomography of the chest in all cases mainly showed intense pleural thickening and volume loss associated with evidence of fibrosis, predominantly in the upper lobes. In all cases in this study, markedly thickened visceral pleura and prominent subpleural fibrosis characterized by both elastic tissue and dense collagen were clearly shown. All cases were alive at the last follow-up, 17.6±13.59 months after diagnosis; however, all had deteriorated both clinically and radiologically.
  
  
  CONCLUSIONS
  IPPFE deserves to be defined as a separate, original clinicopathologic entity owing to its uniformity and IPPFE has some features in common with previously described idiopathic upper lobe fibrosis (IPUF). Our limited experience with a cohort of 5 subjects suggests that IPPFE can be rapidly progressive.
authors.affiliation: Department of Internal Medicine, Hamamatsu University School of Medicine, Hamamatsu, Japan.
authors.forename: Hideki
authors.initials: H
authors.lastname: Kusagaya
authors.forename: Yutaro
authors.initials: Y
authors.lastname: Nakamura
authors.forename: Masato
authors.initials: M
authors.lastname: Kono
authors.forename: Yusuke
authors.initials: Y
authors.lastname: Kaida
authors.forename: Shigeki
authors.initials: S
authors.lastname: Kuroishi
authors.forename: Noriyuki
authors.initials: N
authors.lastname: Enomoto
authors.forename: Tomoyuki
authors.initials: T
authors.lastname: Fujisawa
authors.forename: Naoki
authors.initials: N
authors.lastname: Koshimizu
authors.forename: Koshi
authors.initials: K
authors.lastname: Yokomura
authors.forename: Naoki
authors.initials: N
authors.lastname: Inui
authors.forename: Takafumi
authors.initials: T
authors.lastname: Suda
authors.forename: Thomas V
authors.initials: TV
authors.lastname: Colby
authors.forename: Kingo
authors.initials: K
authors.lastname: Chida
mesh_terms: D000368:Aged; D044466:Asian People; D001706:Biopsy; D003094:Collagen / Q000378:metabolism; D004547:Elastic Tissue / Q000473:pathology; D005260:Female; D006801:Humans; D008168:Lung / Q000473:pathology; D008297:Male; D010995:Pleural Diseases / Q000145:classification / Q000000981:diagnostic imaging* / Q000473:pathology*; D011658:Pulmonary Fibrosis / Q000145:classification / Q000000981:diagnostic imaging* / Q000473:pathology*; D013902:Radiography, Thoracic; D012129:Respiratory Function Tests; D014057:Tomography, X-Ray Computed
chemical_list: D003094:Collagen