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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
- Abstract
<bold>Background: </bold>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.<bold>Methods: </bold>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.<bold>Results: </bold>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.<bold>Conclusions: </bold>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.
- Publication
Respiratory Research, 2014, Vol 15, Issue 1, p109
- ISSN
1465-9921
- Publication type
journal article
- DOI
10.1186/s12931-014-0109-y