Autopsy analyses in acute exacerbation of idiopathic pulmonary fibrosis

• Published in: Respiratory research Vol. 15; no. 1; p. 109
• Main 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
• Format: Journal Article
• Language: English
• Published: England BioMed Central Ltd 01.09.2014; BioMed Central
• Subjects: Adrenal Cortex Hormones - administration & dosage; Adrenal Cortex Hormones - adverse effects; Aged; Aged, 80 and over; Analysis; Autopsy; Bacterial pneumonia; Biopsy; Care and treatment; Cause of Death; Comorbidity; Complications and side effects; Cytomegalovirus; Development and progression; Diagnosis; Disease Progression; Female; Health aspects; Humans; Idiopathic Pulmonary Fibrosis - complications; Idiopathic Pulmonary Fibrosis - drug therapy; Idiopathic Pulmonary Fibrosis - mortality; Idiopathic Pulmonary Fibrosis - pathology; Japan; Lung - drug effects; Lung - pathology; Male; Medical records; Medical research; Medical treatment; Medicine, Experimental; Middle Aged; Mortality; Patient outcomes; Pneumonia; Predictive Value of Tests; Respiratory distress syndrome; Respiratory Tract Infections - pathology; Retrospective Studies; Risk Factors; Rodents; Studies; Thromboembolism; Tomography, X-Ray Computed; Japan
• ISSN: 1465-993X; 1465-9921; 1465-993X
• DOI: 10.1186/s12931-014-0109-y

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.