Open Access Highly Accessed Research article

Idiopathic pleuroparenchymal fibroelastosis: consideration of a clinicopathological entity in a series of Japanese patients

Hideki Kusagaya1, Yutaro Nakamura1*, Masato Kono1, Yusuke Kaida1, Shigeki Kuroishi1, Noriyuki Enomoto1, Tomoyuki Fujisawa12, Naoki Koshimizu1, Koshi Yokomura1, Naoki Inui13, Takafumi Suda1, Thomas V Colby4 and Kingo Chida1

Author Affiliations

1 Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine, Hamamatsu, Japan

2 Department of Laboratory Medicine, Hamamatsu University School of Medicine, Hamamatsu, Japan

3 Department of Clinical Pharmacology and Therapeutics, Hamamatsu University School of Medicine, Hamamatsu, Japan

4 Department of Laboratory Medicine/Pathology, Mayo Clinic Arizona, Scottsdale, Arizona, USA

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BMC Pulmonary Medicine 2012, 12:72  doi:10.1186/1471-2466-12-72

Published: 5 December 2012



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.


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±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.


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 interstitial lung disease; Pleural fibrosis; Fibroelastosis; Pleuroparenchymal fibroelastosis