Proliferation of elastic fibres in idiopathic pulmonary fibrosis: a whole‐slide image analysis and comparison with pleuroparenchymal fibroelastosis

Kinoshita, Yoshiaki; Watanabe, Kentaro; Ishii, Hiroshi; Kushima, Hisako; Fujita, Masaki; Nabeshima, Kazuki

doi:10.1111/his.13312

Cited by 25 publications

(31 citation statements)

References 21 publications

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“…Centriacinar or paraseptal emphysema is usually distributed in the upper lobes or the superior segments of the lower lobes . In contrast, fibrosis in IPF is generally distributed predominantly in the lower lobes . Since Cottin et al .…”

Section: Discussionmentioning

confidence: 99%

Distribution of emphysema and fibrosis in idiopathic pulmonary fibrosis with coexisting emphysema

et al. 2019

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Aims Combined pulmonary fibrosis and emphysema (CPFE) is a syndrome that results from tobacco smoking. Emphysema and fibrosis in CPFE patients have been considered to exist separately, with emphysema in the upper lobes and interstitial pneumonia in the lower lobes. The aim of this study was to examine the intrapulmonary distribution of fibrosis and emphysema in clinically diagnosed patients with idiopathic pulmonary fibrosis (IPF) and coexisting emphysema. Methods and results Among IPF patients (n = 40) who had been autopsied or pneumonectomised for lung transplantation from 1993 to 2018, we retrospectively selected patients with IPF and coexisting emphysema (n = 19) on the basis of the appearance on chest computed tomography (IPF patients with emphysema). We then histologically determined the intrapulmonary distribution of emphysema and fibrosis in the upper lobes and the lower lobes separately. In 15 of the 19 IPF patients with emphysema (79%), fibrosis and emphysema coexisted in the upper lobes and the lower lobes. No patients showed emphysema exclusively in the upper lobes and fibrosis exclusively in the lower lobes. Conclusions In the autopsied and pneumonectomised specimens of IPF patients with emphysema, craniocaudal separation of emphysema and fibrosis (emphysema in the upper lobes and interstitial pneumonia in the lower lobes) was histologically rare; coexistence or collision of fibrosis and emphysema in each lobe was common.

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Section: Discussionmentioning

confidence: 99%

Distribution of emphysema and fibrosis in idiopathic pulmonary fibrosis with coexisting emphysema

et al. 2019

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“…Conversely, usual interstitial pneumonia (UIP) is the most common fibrotic interstitial pneumonia, that presents as subpleural or paraseptal fibrosis and results in architectural distortion . Given the morphological similarity regarding the subpleura‐orientated distribution of fibrosis between PPFE and UIP, distinguishing between these entities can be difficult, particularly in cases of PPFE with mild elastosis versus UIP with severe elastosis . Therefore, identifying specific immunostaining markers to distinguish between these two diseases would be helpful for lung pathologists.…”

Section: Introductionmentioning

confidence: 99%

Podoplanin‐positive myofibroblasts: a pathological hallmark of pleuroparenchymal fibroelastosis

et al. 2018

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Pathological differential diagnoses of pleuroparenchymal fibroelastosis (PPFE) include usual interstitial pneumonia (UIP) and pulmonary apical cap (PAC); however, there are no specific immunostaining makers to distinguish between these diseases. We performed immunohistochemistry using several pleural mesothelial cell-related markers, including cytokeratin-5/6, CAM5.2, WT-1, calretinin, desmin and podoplanin, for PPFE (n = 4), UIP (n = 10) and PAC (n = 3) lung sections. Among the examined markers, in PPFE and PAC lungs podoplanin commonly showed positivity for spindle cells both in thickened pleura and subpleural fibroelastosis lesions; these cells were also stained with α-smooth muscle actin, a marker of myofibroblasts. However, even in elastic fibre-rich cases, UIP lungs did not show such podoplanin-positive myofibroblasts in pleura/subpleura and fibroblastic foci. These findings were also verified using immunofluorescence. By contrast, immunohistochemically as well as morphologically, the difference between PPFE and PAC was not apparent. The presence of podoplanin-positive myofibroblasts could be a pathological hallmark of PPFE, suggesting a pathogenic process distinct from UIP but common to PAC.

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“…The fibroelastosis in PPFE usually distributes predominantly in the upper lobes . Why, then, did elastofibrosis develop predominantly in the lower lobes in the present case?…”

Section: Discussionmentioning

confidence: 60%

“…[1][2][3] Although broadly-accepted diagnostic criteria of PPFE have never been established, the upper-lobe predominance of elastofibrosis is agreed upon for the diagnosis of clinical PPFE. 2,[4][5][6][7][8] We herein report a patient with chronic fibrosing interstitial pneumonia who presented with a histology of lower-lobepredominant PPFE.…”

Section: Introductionmentioning

confidence: 92%

“…Pleuroparenchymal fibroelastosis (PPFE) is a distinct clinicopathologic entity of pulmonary fibrosis characterized by upper‐lobe predominant elastofibrosis involving the subpleural lung parenchyma with or without collagenous thickening of visceral pleura . Although broadly‐accepted diagnostic criteria of PPFE have never been established, the upper‐lobe predominance of elastofibrosis is agreed upon for the diagnosis of clinical PPFE …”

Section: Introductionmentioning

confidence: 99%

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Lower‐lobe predominant pleuroparenchymal fibroelastosis

Kinoshita

Watanabe

Ishii

et al. 2019

Pathology International

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Upper‐lobe predominance of elastofibrosis is agreed upon for the diagnosis of clinical pleuroparenchymal fibroelastosis (PPFE). We herein describe a patient with dermatomyositis‐related interstitial pneumonia with a histology of lower‐lobe predominant PPFE. A 71‐year‐old woman who had been diagnosed with dermatomyositis‐related interstitial pneumonia died of respiratory failure. The computed tomography patterns of the lower lobes showed reticular and ground‐glass opacities with traction bronchiectasis. An autopsy revealed that the bilateral lower lobes were sclerotic with decreased air volume. A microscopic examination of the lower lobes showed pleural fibrosis and subpleural elastofibrosis without the structural destruction, indicative of histological PPFE. PPFE histology was also evident in the upper lobes but relatively modest compared to that of the lower lobes. In addition, because the computed tomography images of the patient were suggestive of non‐PPFE‐type fibrosis, lower‐lobe dominant PPFE might be overlooked in daily practice.

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Proliferation of elastic fibres in idiopathic pulmonary fibrosis: a whole‐slide image analysis and comparison with pleuroparenchymal fibroelastosis

Abstract: IPF occasionally shows intense elastosis in the upper lobes, and such cases are histologically indistinguishable from PPFE. There seem to be histologically borderline cases between PPFE and IPF.

Cited by 25 publications

References 21 publications

Distribution of emphysema and fibrosis in idiopathic pulmonary fibrosis with coexisting emphysema

Distribution of emphysema and fibrosis in idiopathic pulmonary fibrosis with coexisting emphysema

Podoplanin‐positive myofibroblasts: a pathological hallmark of pleuroparenchymal fibroelastosis

Lower‐lobe predominant pleuroparenchymal fibroelastosis

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