Autopsy analyses in acute exacerbation of idiopathic pulmonary fibrosis.

Keishi Oda,Hiroshi Mukae,Tatsuhiko Harada,Hiroshi Ishii,Masamitsu Nakazato,Shigeru Kohno,Tomotoshi Imanaga,Nobuhiro Matsumoto,Keisuke Naito,Jun-Ichi Kadota,Sohsuke Yamada,Hisako Kushima,Yuji Ishimatsu,Kentaro Watanabe,Kazuhiro Yatera,Hiroshi Ishimoto

doi:10.1186/s12931-014-0109-y

Abstract

BackgroundAcute 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.MethodsWe 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.ResultsThe 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.ConclusionsThe 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.

Highlights

Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive, fibrosing form of interstitial pneumonia with a median survival after diagnosis of three to five years [1,2,3,4]
Lung tissue derived from patients with IPF shows a characteristic histopathological pattern known as usual interstitial pneumonia (UIP), which includes the presence of fibroblastic foci
Previous reports have focused on the postmortem pathological findings of patients with IPF [15,16], no studies have focused on the characteristics of “acute exacerbation”, and the pathological findings and clinical manifestations of Acute exacerbation of idiopathic pulmonary fibrosis (AE-IPF) remain only partially understood

Summary

Introduction

Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive, fibrosing form of interstitial pneumonia with a median survival after diagnosis of three to five years [1,2,3,4]. In 1993, AE-IPF was first described in a case report by Kondoh et al as acute clinical deterioration in three IPF patients in the absence of. Lung tissue derived from patients with IPF shows a characteristic histopathological pattern known as usual interstitial pneumonia (UIP), which includes the presence of fibroblastic foci. Previous reports have focused on the postmortem pathological findings of patients with IPF [15,16], no studies have focused on the characteristics of “acute exacerbation”, and the pathological findings and clinical manifestations of AE-IPF remain only partially understood. Acute exacerbation of idiopathic pulmonary fibrosis (AE-IPF) is associated with high mortality. Few studies have so far reviewed analyses of autopsy findings in patients with AE-IPF

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Results

Discussion

Conclusion