Chest
Original Research Diffuse Lung DiseaseDistinct Characteristics of Pleuroparenchymal Fibroelastosis With Usual Interstitial Pneumonia Compared With Idiopathic Pulmonary Fibrosis
Section snippets
Study Population
We retrospectively reviewed medical records of 291 consecutive patients with idiopathic interstitial pneumonia who underwent surgical lung biopsy between January 2001 and December 2011 at Kanagawa Cardiovascular and Respiratory Center. Included were 110 consecutive patients with IPF diagnosed according to the 2002 ATS/ERS statement,18 with histologic UIP pattern shown in the biopsy specimen from the lower lobe. This study was approved by an institutional review board of Kanagawa Cardiovascular
Patient Identification
Eleven of the 110 consecutive patients with IPF met radiologic criteria for PPFE. Of these, nine fulfilled the histologic criteria of PPFE. Two patients were excluded because their upper lobe biopsy specimens showed marked interstitial inflammatory cell infiltrates within the intraalveolar fibrosis, and we found it difficult to completely rule out other interstitial pneumonia, such as connective tissue disease-related UIP. Accordingly, the nine patients meeting both radiologic and histologic
Discussion
To our knowledge, this study is the first to describe the clinical characteristics of PPFE with UIP pattern compared with IPF/UIP. We identified nine patients with PPFE with UIP pattern among 110 consecutive patients with surgically proven IPF/UIP diagnosed according to the 2002 ATS/ERS statement.18 We identified three characteristic features of PPFE with UIP pattern. Compared with patients with IPF/UIP, patients with PPFE with UIP pattern had (1) a higher complication rate of pneumothorax or
Acknowledgments
Author contributions:T. Oda had full access to all of the data in the study and takes responsibility for the integrity of the data and the accuracy of the data analysis. T. Ogura contributed to the study concept and design, data analysis and interpretation, and final approval of the manuscript; H. K. and Y. H. contributed to the study concept and design and submission of the manuscript; E. H. contributed to the data collection, organization, and analysis and preparation, review, writing, and
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Funding/Support:This study was supported in part by a grant-in-aid for interstitial lung diseases from the Japanese Ministry of Health, Labor and Welfare [Grant H23-Nanchi-Ippan-023].
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originally published Online First June 19, 2014.