%0 Journal Article
%T Idiopathic pleuroparenchymal fibroelastosis: consideration of a clinicopathological entity in a series of Japanese patients
%A Hideki Kusagaya
%A Yutaro Nakamura
%A Masato Kono
%A Yusuke Kaida
%A Shigeki Kuroishi
%A Noriyuki Enomoto
%A Tomoyuki Fujisawa
%A Naoki Koshimizu
%A Koshi Yokomura
%A Naoki Inui
%A Takafumi Suda
%A Thomas V Colby
%A Kingo Chida
%J BMC Pulmonary Medicine
%D 2012
%I BioMed Central
%R 10.1186/1471-2466-12-72
%X Five consecutive cases of idiopathic interstitial lung disease confirmed as IPPFE by surgical lung biopsy were studied.There were four males and one female, aged 70¡À2.76 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¡À13.59 months after diagnosis; however, all had deteriorated both clinically and radiologically.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.Idiopathic pleuroparenchymal fibroelastosis (IPPFE) is a recently reported rare disorder characterized by fibrotic thickening of the pleural and subpleural parenchyma predominantly in the upper lobes [1]; as such IPPFE is distinct from other types of idiopathic interstitial pneumonia [2]. Since 1992 several reports of upper lobe-predominant pulmonary fibrosis (Idiopathic upper lobe fibrosis/IPUF) have been reported in the Japanese literature [3-9] and some of these cases share features with IPPFE. Herein we report five Japanese cases of IPPFE, and describe the clinical, radiologic and evolutionary profile of IPPFE in a retrospective series in order to determine if IPPFE represents a separate clinicopathologic entity and to clarify its relationship with IPUF.The subjects included 5 patients fu
%K Idiopathic interstitial lung disease
%K Pleural fibrosis
%K Fibroelastosis
%K Pleuroparenchymal fibroelastosis
%U http://www.biomedcentral.com/1471-2466/12/72