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

Hideki Kusagaya,Noriyuki Enomoto,Takafumi Suda,Naoki Inui,Masato Kono,Yusuke Kaida,Kingo Chida,Koshi Yokomura,Yutaro Nakamura,Naoki Koshimizu,Thomas V Colby,Shigeki Kuroishi,Tomoyuki Fujisawa

doi:10.1186/1471-2466-12-72

Hideki Kusagaya, Noriyuki Enomoto + Show 11 more

Open Access

https://doi.org/10.1186/1471-2466-12-72

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Abstract

BackgroundIdiopathic 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.MethodsFive consecutive cases of idiopathic interstitial lung disease confirmed as IPPFE by surgical lung biopsy were studied.ResultsThere 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.ConclusionsIPPFE 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.

Highlights

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
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]
The level of surfactant protein D (SP-D) was higher than the normal range in all the cases

Summary

Introduction

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. 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. 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]. 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.

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