{"title":"Pleuroparenchymal Fibroelastosis: Update on CT and Histologic Findings.","authors":"Joel L Gamble, Nestor L Müller, Andrew Churg, Ana-Maria Bilawich","doi":"10.1148/ryct.240382","DOIUrl":null,"url":null,"abstract":"<p><p>Pleuroparenchymal fibroelastosis (PPFE) is an interstitial lung disease (ILD) characterized at CT by upper lobe-predominant pleural thickening and subpleural fibrosis and histologically by visceral pleural fibrosis and subpleural fibroelastosis. Although initially classified as a rare idiopathic interstitial pneumonia, many cases are related to known risk factors, particularly hematopoietic stem cell and lung transplant, or observed in association with other ILDs. This review summarizes the diagnostic criteria for PPFE and illustrates the CT and histologic manifestations, aiming to familiarize the radiologist with the range of findings suggestive of the diagnosis. <b>Keywords:</b> Conventional Radiography, CT, Pulmonary, Thorax, Lung, Pleura, Complications, Transplantation, Fibrosis © RSNA, 2025.</p>","PeriodicalId":21168,"journal":{"name":"Radiology. Cardiothoracic imaging","volume":"7 2","pages":"e240382"},"PeriodicalIF":3.8000,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":null,"platform":"Semanticscholar","paperid":null,"PeriodicalName":"Radiology. Cardiothoracic imaging","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.1148/ryct.240382","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q1","JCRName":"RADIOLOGY, NUCLEAR MEDICINE & MEDICAL IMAGING","Score":null,"Total":0}
引用次数: 0
Abstract
Pleuroparenchymal fibroelastosis (PPFE) is an interstitial lung disease (ILD) characterized at CT by upper lobe-predominant pleural thickening and subpleural fibrosis and histologically by visceral pleural fibrosis and subpleural fibroelastosis. Although initially classified as a rare idiopathic interstitial pneumonia, many cases are related to known risk factors, particularly hematopoietic stem cell and lung transplant, or observed in association with other ILDs. This review summarizes the diagnostic criteria for PPFE and illustrates the CT and histologic manifestations, aiming to familiarize the radiologist with the range of findings suggestive of the diagnosis. Keywords: Conventional Radiography, CT, Pulmonary, Thorax, Lung, Pleura, Complications, Transplantation, Fibrosis © RSNA, 2025.
胸膜实质纤维弹性增生：CT和组织学表现的最新进展。
胸膜实质纤维弹性增生（PPFE）是一种间质性肺疾病（ILD）， CT表现为上肺叶为主的胸膜增厚和胸膜下纤维化，组织学表现为内脏性胸膜纤维化和胸膜下纤维弹性增生。虽然最初被归类为罕见的特发性间质性肺炎，但许多病例与已知的危险因素有关，特别是造血干细胞和肺移植，或观察到与其他ild相关。本文总结了PPFE的诊断标准，并阐述了CT和组织学表现，旨在使放射科医生熟悉提示诊断的一系列表现。关键词：常规影像学，CT，肺，胸，肺，胸膜，并发症，移植，纤维化©RSNA， 2025。
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