Autopsy analyses in acute exacerbation of idiopathic pulmonary fibrosis.

Autopsy analyses in acute exacerbation of idiopathic pulmonary fibrosis.
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DOI:
10.1186/s12931-014-0109-y
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发表时间:
2014-09-01
影响因子:
5.8
通讯作者:
Mukae H
Mukae H
中科院分区:
医学2区
文献类型:
--
作者:
Oda K;Ishimoto H;Yamada S;Kushima H;Ishii H;Imanaga T;Harada T;Ishimatsu Y;Matsumoto N;Naito K;Yatera K;Nakazato M;Kadota J;Watanabe K;Kohno S;Mukae H

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特发性肺纤维化急性加重(AE-IPF)与高死亡率相关。然而,到目前为止,很少有研究审查AE-IPF患者尸检结果的分析。我们回顾性分析了1999年至2013年期间在5家大学医院和1家市立医院接受尸检的52例连续AE-IPF患者。从病历中提取以下变量:人口统计学和临床数据、尸检结果和临床过程中直至死亡的并发症。尸检时的中位年龄为71岁(范围47-86岁),受试者包括38例(73.1%)男性。45例(86.5%)患者在AE-IPF后开始接受高剂量皮质类固醇治疗。在所有病例中,基础纤维化病变被归类为具有常见的间质性肺炎(UIP)模式。弥漫性肺泡损伤41例(78.8%),肺出血15例(28.8%),肺血栓栓塞9例(17.3%),肺癌6例(11.5%)。此外,6例(11.5%)患者在死亡前发生气胸,26例(53.1%)患者在接受高剂量皮质类固醇治疗后发生需要胰岛素治疗的糖尿病。此外,15例(28.8%)患者在临床过程中和/或直至死亡时出现支气管肺炎,包括真菌(7例,13.5%)、巨细胞病毒(6例,11.5%)和细菌(5例,9.6%)感染。AE-IPF患者的病理学结果不仅代表DAD,还代表各种病理状况。因此,AE-IPF的诊断通常很困难,需要使用谨慎的诊断方法进行适当的治疗。
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. 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. 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. 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.
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发表时间: 2000-12-01
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发表时间: 2001-06-01
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