title: Autopsy analyses in acute exacerbation of idiopathic pulmonary fibrosis.
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.
authors.forename: Keishi
authors.initials: K
authors.lastname: Oda
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Ishimoto
authors.forename: Sohsuke
authors.initials: S
authors.lastname: Yamada
authors.forename: Hisako
authors.initials: H
authors.lastname: Kushima
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Ishii
authors.forename: Tomotoshi
authors.initials: T
authors.lastname: Imanaga
authors.forename: Tatsuhiko
authors.initials: T
authors.lastname: Harada
authors.forename: Yuji
authors.initials: Y
authors.lastname: Ishimatsu
authors.forename: Nobuhiro
authors.initials: N
authors.lastname: Matsumoto
authors.forename: Keisuke
authors.initials: K
authors.lastname: Naito
authors.forename: Kazuhiro
authors.initials: K
authors.lastname: Yatera
authors.forename: Masamitsu
authors.initials: M
authors.lastname: Nakazato
authors.forename: Jun-Ichi
authors.initials: J
authors.lastname: Kadota
authors.forename: Kentaro
authors.initials: K
authors.lastname: Watanabe
authors.forename: Shigeru
authors.initials: S
authors.lastname: Kohno
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Mukae
mesh_terms: D000305:Adrenal Cortex Hormones / Q000008:administration & dosage / Q000009:adverse effects; D000368:Aged; D000369:Aged, 80 and over; D001344:Autopsy; D001706:Biopsy; D002423:Cause of Death; D015897:Comorbidity; D018450:Disease Progression; D005260:Female; D006801:Humans; D054990:Idiopathic Pulmonary Fibrosis / Q000150:complications / Q000188:drug therapy / Q000401:mortality / Q000473:pathology*; D007564:Japan; D008168:Lung / Q000187:drug effects / Q000473:pathology*; D008297:Male; D008875:Middle Aged; D011237:Predictive Value of Tests; D012141:Respiratory Tract Infections / Q000473:pathology; D012189:Retrospective Studies; D012307:Risk Factors; D014057:Tomography, X-Ray Computed
chemical_list: D000305:Adrenal Cortex Hormones