Autopsy analyses in acute exacerbation of idiopathic pulmonary fibrosis

作者：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^*

来源：Respiratory Research, 2014, 15(1): 109.

DOI：10.1186/s12931-014-0109-y

• Acute exacerbation
• Idiopathic pulmonary fibrosis
• Autopsy
• Diffuse alveolar damage

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. %26lt;br%26gt;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. %26lt;br%26gt;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. %26lt;br%26gt;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.

• 出版日期2014-9-1