Criteria for the diagnosis of idiopathic pleuroparenchymal fibroelastosis: A proposal

Watanabe, Kentaro; Ishii, Hiroshi; Kiyomi, Fumiaki; Terasaki, Yasuhiro; Hebisawa, Akira; Kawabata, Yoshinori; Johkoh, Takeshi; Sakai, Fumikazu; Kondoh, Yasuhiro; Inoue, Yoshikazu; Azuma, Arata; Suda, Takafumi; Ogura, Takashi; Inase, Naohiko; Homma, Sakae

doi:10.1016/j.resinv.2019.02.007

Cited by 68 publications

(63 citation statements)

References 40 publications

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“…Subjects. We retrospectively reviewed our clinical records between 1995 and 2017, and selected 18 patients with IPPFE who had been clinically diagnosed and whose diagnosis had been confirmed by a pathological examination in Fukuoka University Hospital 1,[28][29][30] . Twelve patients underwent a surgical lung biopsy and 6 were examined at autopsy.…”

Section: Methodsmentioning

confidence: 99%

Remodeling of the pulmonary artery in idiopathic pleuroparenchymal fibroelastosis

Kinoshita

Ishii

Kushima

et al. 2020

Sci Rep

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Idiopathic pleuroparenchymal fibroelastosis (IPPFE) is a rare subtype of idiopathic interstitial pneumonia that consists of alveolar septal elastosis and intra-alveolar collagenosis, which is predominantly located in the upper lobes. The aim of this study was to examine the remodeling of the pulmonary arteries in patients with IPPFE. This study included 18 patients with IPPFE, 24 patients with idiopathic pulmonary fibrosis (IPF), and 5 patients without pulmonary disease as controls. We selected muscular pulmonary arteries and calculated the percentage of the thickness of each layer of the wall (intima, media, and adventitia) in relation to the external diameter. We also quantified the percentage of areas of elastic fiber in the media divided by the whole area of the media (medial elastic fiber score). The percentage of adventitial thickness in IPPFE was significantly higher than that in IPF and in control lungs. The percentage of medial thickness did not differ statistically between IPPFE and IPF. However, the medial elastic fiber score in IPPFE was also significantly larger than that in IPF and control lungs. These results suggest that collagenous thickening of the adventitia and medial elastosis are distinct histological features in the muscular pulmonary arteries of patients with IPPFE.

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Section: Methodsmentioning

confidence: 99%

Remodeling of the pulmonary artery in idiopathic pleuroparenchymal fibroelastosis

Kinoshita

Ishii

Kushima

et al. 2020

Sci Rep

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“…However, because these patients showed superior fibrosis in the upper lobe and their respiratory function test results were available [(residual volume (RV)/total lung capacity (TLC)% predicted.) ≥ 115%)], we diagnosed these as probable PPFE [ 7 ]. The assessment of seasonal distribution showed five patients each in January and November, four in December, three each in October and February, two each in May and September, and one patient in March, April, June, and August.…”

Section: Resultsmentioning

confidence: 99%

Prevalence of viral infection in acute exacerbation of interstitial lung diseases in Japan

Matsumoto

Kushima

et al. 2020

Respiratory Investigation

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Background Fatal acute exacerbation of interstitial lung diseases is often accompanied by indicators of infection such as fever, cough, and sputum. Although viral infection can contribute to acute exacerbation of interstitial lung diseases, few studies have identified a relationship between acute exacerbations and viral infections. The present study aimed to prospectively clarify the role of viral infection in patients showing acute exacerbation of interstitial lung disease in Japan. Methods Nasopharyngeal swab specimens were collected from patients with acute exacerbation of interstitial lung disease between May 2017 and February 2019. Respiratory viruses were detected by the Luminex xTAG Respiratory Viral Panel FAST v2 RUO kit and the BioFire FilmArray Respiratory Panel assay. Results Three of 29 patients demonstrated respiratory viral infection during acute exacerbation of interstitial lung diseases. The infectious agents were identified as respiratory syncytial virus, respiratory syncytial virus and influenza A virus, and influenza A virus and rhino/enterovirus in the three patients, respectively. Conclusions These results suggest that viral infection did not frequently induce acute exacerbation of interstitial lung diseases in Japan.

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“…[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%

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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Criteria for the diagnosis of idiopathic pleuroparenchymal fibroelastosis: A proposal

Cited by 68 publications

References 40 publications

Remodeling of the pulmonary artery in idiopathic pleuroparenchymal fibroelastosis

Remodeling of the pulmonary artery in idiopathic pleuroparenchymal fibroelastosis

Prevalence of viral infection in acute exacerbation of interstitial lung diseases in Japan

Lower‐lobe predominant pleuroparenchymal fibroelastosis

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