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The pathogenesis and pathology of idiopathic pleuroparenchymal fibroelastosis

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dc.contributor.authorKinoshita, Yoshiaki
dc.contributor.authorIshii, Hiroshi
dc.contributor.authorNabeshima, Kazuki
dc.contributor.authorWatanabe, Kentaro
dc.date.accessioned2023-01-10T09:44:30Z
dc.date.available2023-01-10T09:44:30Z
dc.date.issued2021
dc.description.abstractIdiopathic pleuroparenchymal fibroelastosis (IPPFE) is a rare subtype of idiopathic interstitial pneumonias that consists of elastofibrosis involving the lung parenchyma and pleural collagenous fibrosis predominantly located in the upper lobes. IPPFE has various distinct clinical and physiological characteristics, including platythorax and a marked decrease of forced vital capacity with an increased residual volume on a respiratory function test. The concept of IPPFE is now widely recognized and some diagnostic criteria have been proposed. In addition, the accumulation of cases has revealed the pathological features of IPPFE. However, little is known about the pathogenesis or the process of disease formation in IPPFE. This review article will provide a summary of the pathological features and previously reported hypotheses on disease formation in IPPFE, to discuss the potential etiologies and pathogenesis of IPPFE.es
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dc.format.extent13es
dc.identifier.citationHistology and Histopathology Vol. 36, nº3 (2021)
dc.identifier.doihttps://doi.org/10.14670/HH-18-289
dc.identifier.doi1699-5848
dc.identifier.issn0213-3911
dc.identifier.urihttp://hdl.handle.net/10201/127008
dc.languageenges
dc.relationSin financiación externa a la Universidades
dc.rightsinfo:eu-repo/semantics/openAccesses
dc.rightsAttribution-NonCommercial-NoDerivatives 4.0 Internacional*
dc.rights.urihttp://creativecommons.org/licenses/by-nc-nd/4.0/*
dc.subjectElastofibrosises
dc.subjectElastic fiberes
dc.subjectEtiologyes
dc.subjectDiagnostic criteriaes
dc.subjectHistopathologyes
dc.subject.otherCDU::6 - Ciencias aplicadas::61 - Medicina::616 - Patología. Medicina clínica. Oncologíaes
dc.titleThe pathogenesis and pathology of idiopathic pleuroparenchymal fibroelastosis
dc.typeinfo:eu-repo/semantics/articlees
dspace.entity.typePublicationes
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Histology and histopathology Vol.36, nº3 (2021)