Pleuroparenchymal fibroelastosis diagnosed by multidisciplinary discussions in Japan

Ishii, Hiroshi; Watanabe, Kentaro; Kushima, Hisako; Baba, Tomohisa; Watanabe, Satoshi; Yamada, Yoshihito; Arai, Toru; Tsushima, Kenji; Kondoh, Yasuhiro; Nakamura, Yutaro; Terasaki, Yasuhiro; Hebisawa, Akira; Johkoh, Takeshi; Sakai, Fumikazu; Takemura, Tamiko; Kawabata, Yoshinori; Ogura, Takashi

doi:10.1016/j.rmed.2018.06.022

Cited by 61 publications

(101 citation statements)

References 34 publications

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“…Thus, PPFE appears to be a multifactorial disease, which raises an important question of why and how these apparently different types of lung disorders are associated with PPFE . PPFE generally has a poor prognosis; according to the most recent study, the median survival time was 96 months, and the cumulative survival rate was 58% at 5 years …”

Section: Introductionmentioning

confidence: 99%

Whole‐lung pathology of pleuroparenchymal fibroelastosis (PPFE) in an explanted lung: Significance of elastic fiber‐rich, non‐specific interstitial pneumonia‐like change in chemotherapy‐related PPFE

Tsubosaka

Matsushima

Ota

et al. 2019

Pathology International

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Pleuroparenchymal fibroelastosis (PPFE) is characterized by upper lobe‐predominant subpleural fibroelastosis. Despite its characteristic uneven distribution, detailed whole‐lung pathological features of PPFE have rarely been studied. We investigated PPFE in the explanted lungs from a 19‐year‐old male patient with a history of chemotherapy. Grossly, the explanted lungs showed upper lobe‐predominant shrinkage with subpleural and central consolidation. Histologically, fibroelastosis was prominent in the perilobular areas and along the bronchovascular bundles. The other areas of the lung showed diffuse, non‐specific interstitial pneumonia (NSIP)–like change with a characteristic increase of septal elastic fibers. In the digital image analysis, the ratio of elastic fibers to whole fibrosis (EF score) was lower in the subpleural areas than in the NSIP‐like lesions, but the EF scores of the latter showed no significant difference between upper and middle/lower lobes. In the present case, the diffusely distributed elastic fiber‐rich NSIP‐like change, probably caused by the earlier chemotherapy, may have been conducive to the development of PPFE. This suggests that some unknown vulnerability of the upper lobe may exist, various primary lesions converging to the upper lobe predominance of PPFE.

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Section: Introductionmentioning

confidence: 99%

Whole‐lung pathology of pleuroparenchymal fibroelastosis (PPFE) in an explanted lung: Significance of elastic fiber‐rich, non‐specific interstitial pneumonia‐like change in chemotherapy‐related PPFE

Tsubosaka

Matsushima

Ota

et al. 2019

Pathology International

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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%

“…The fibroelastosis in PPFE usually distributes predominantly in the upper lobes. 2,5,7,8 Why, then, did elastofibrosis develop predominantly in the lower lobes in the present case? We speculate that the repeated acute exacerbation of interstitial pneumonia might result in the formation of elastofibrosis.…”

Section: Discussionmentioning

confidence: 78%

“…[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: 93%

“…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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