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

Kusagaya H, Nakamura Y, Kono M, Kaida Y, Kuroishi S, Enomoto N, Fujisawa T, Koshimizu N, Yokomura K, Inui N, Suda T, Colby TV, Chida K - BMC Pulm Med (2012)

Bottom Line: 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).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.

View Article: PubMed Central - HTML - PubMed

Affiliation: Department of Internal Medicine, Hamamatsu University School of Medicine, Hamamatsu, Japan.

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.

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Chest X-ray and Chest CT. Chest radiographs in all cases showed marked apical pleural thickening. High-resolution CT (HRCT) showed upper lobe volume loss, architectural distortion, traction bronchiectasis, and reticular abnormalities. There was no honeycombing.
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Figure 1: Chest X-ray and Chest CT. Chest radiographs in all cases showed marked apical pleural thickening. High-resolution CT (HRCT) showed upper lobe volume loss, architectural distortion, traction bronchiectasis, and reticular abnormalities. There was no honeycombing.

Mentions: Chest radiographs in all cases showed marked apical pleural thickening and superior hilar retraction (Figure1). Right lung is predominantly affected in case 2, 3, and 5, left lung in case 4. High-resolution CT (HRCT) showed intense pleural thickening associated with evidence of fibrosis. In Case 1 and 2, upper lobe volume loss, architectural distortion and traction bronchiectasis were also prominent.


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

Kusagaya H, Nakamura Y, Kono M, Kaida Y, Kuroishi S, Enomoto N, Fujisawa T, Koshimizu N, Yokomura K, Inui N, Suda T, Colby TV, Chida K - BMC Pulm Med (2012)

Chest X-ray and Chest CT. Chest radiographs in all cases showed marked apical pleural thickening. High-resolution CT (HRCT) showed upper lobe volume loss, architectural distortion, traction bronchiectasis, and reticular abnormalities. There was no honeycombing.
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC3539991&req=5

Figure 1: Chest X-ray and Chest CT. Chest radiographs in all cases showed marked apical pleural thickening. High-resolution CT (HRCT) showed upper lobe volume loss, architectural distortion, traction bronchiectasis, and reticular abnormalities. There was no honeycombing.
Mentions: Chest radiographs in all cases showed marked apical pleural thickening and superior hilar retraction (Figure1). Right lung is predominantly affected in case 2, 3, and 5, left lung in case 4. High-resolution CT (HRCT) showed intense pleural thickening associated with evidence of fibrosis. In Case 1 and 2, upper lobe volume loss, architectural distortion and traction bronchiectasis were also prominent.

Bottom Line: 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).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.

View Article: PubMed Central - HTML - PubMed

Affiliation: Department of Internal Medicine, Hamamatsu University School of Medicine, Hamamatsu, Japan.

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.

Show MeSH
Related in: MedlinePlus