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Autopsy analyses in acute exacerbation of idiopathic pulmonary fibrosis

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Title: Autopsy analyses in acute exacerbation of idiopathic pulmonary fibrosis
Authors: Oda, Keishi / Ishimoto, Hiroshi / Yamada, Sohsuke / Kushima, Hisako / Ishii, Hiroshi / Imanaga, Tomotoshi / Harada, Tatsuhiko / Ishimatsu, Yuji / Matsumoto, Nobuhiro / Naito, Keisuke / Yatera, Kazuhiro / Nakazato, Masamitsu / Kadota, Jun-ichi / Watanabe, Kentaro / Kohno, Shigeru / Mukae, Hiroshi
Issue Date: 1-Sep-2014
Publisher: BioMed Central Ltd.
Citation: Respiratory Research, 15(1), 109; 2014
Abstract: Background: Acute exacerbation of idiopathic pulmonary fibrosis (AE-IPF) is associated with high mortality. However, few studies have so far reviewed analyses of autopsy findings in patients with AE-IPF.Methods: We retrospectively reviewed 52 consecutive patients with AE-IPF who underwent autopsies at five university hospitals and one municipal hospital between 1999 and 2013. The following variables were abstracted from the medical records: demographic and clinical data, autopsy findings and complications during the clinical course until death.Results: The median age at autopsy was 71 years (range 47-86 years), and the subjects included 38 (73.1%) males. High-dose corticosteroid therapy was initiated in 45 (86.5%) patients after AE-IPF. The underling fibrotic lesion was classified as having the usual interstitial pneumonia (UIP) pattern in all cases. Furthermore, 41 (78.8%) patients had diffuse alveolar damage (DAD), 15 (28.8%) exhibited pulmonary hemorrhage, nine (17.3%) developed pulmonary thromboembolism and six (11.5%) were diagnosed with lung carcinoma. In addition, six (11.5%) patients developed pneumothorax prior to death and 26 (53.1%) developed diabetes that required insulin treatment after the administration of high-dose corticosteroid therapy. In addition, 15 (28.8%) patients presented with bronchopneumonia during their clinical course and/or until death, including fungal (seven, 13.5%), cytomegalovirus (six, 11.5%) and bacterial (five, 9.6%) infections.Conclusions: The pathological findings in patients with AE-IPF represent not only DAD, but also a variety of pathological conditions. Therefore, making a diagnosis of AE-IPF is often difficult, and the use of cautious diagnostic approaches is required for appropriate treatment.
URI: http://hdl.handle.net/10069/34818
ISSN: 14659921
DOI: 10.1186/s12931-014-0109-y
PubMed ID: 25176016
Rights: © 2014 Oda et al.; licensee BioMed Central Ltd. / This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/4.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly credited. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.
Type: Journal Article
Text Version: publisher
Appears in Collections:Articles in academic journal

Citable URI : http://hdl.handle.net/10069/34818

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