Chest
Volume 146, Issue 5, November 2014, Pages 1248-1255
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Original Research Diffuse Lung Disease
Distinct Characteristics of Pleuroparenchymal Fibroelastosis With Usual Interstitial Pneumonia Compared With Idiopathic Pulmonary Fibrosis

https://doi.org/10.1378/chest.13-2866Get rights and content

BACKGROUND

Pleuroparenchymal fibroelastosis (PPFE) is a rare form of interstitial pneumonia and sometimes coexists with a histologic usual interstitial pneumonia (UIP) pattern. This study aimed to describe the distinct clinical features of PPFE with UIP pattern compared with idiopathic pulmonary fibrosis (IPF).

METHODS

We conducted a retrospective review of the medical records of 110 consecutive patients with IPF with a histologic UIP pattern on surgical lung biopsy specimen. Patients meeting radiologic criteria for the diagnosis of PPFE based on high-resolution CT scan and with a histologic UIP pattern were included.

RESULTS

Nine of eleven patients meeting radiologic criteria for the diagnosis of PPFE were histologically confirmed as having PPFE with UIP pattern. The PPFE with UIP pattern group showed a significantly higher residual volume (1.8 L vs 1.3 L,P< .01), higher Paco2(44.6 mm Hg vs 41.7 mm Hg,P= .04), and higher complication rate of pneumothorax and pneumomediastinum than the 99 patients with IPF/UIP. The ratio of anteroposterior to transthoracic diameter in patients with PPFE with UIP pattern was significantly lower than that in patients with IPF/UIP (P= .04). Survival time tended to be shorter in patients with PPFE with UIP pattern.

CONCLUSIONS

The results support the view that PPFE with UIP pattern is a disease entity distinct from IPF/UIP and may well be classified as PPFE.

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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    • Pulmonary Hypertension or Pulmonary Arterial Hypertension in Idiopathic Pleuroparenchymal Fibroelastosis: An Updated Comprehensive Review

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      The presence of a family link between these patients has been reported in up to 57% of cases.9 History of chemotherapy treatment, autoimmune or connective tissue disease, acute lung injury, infectious complications, chronic hypersensitivity pneumonitis (CHP), and occupational exposure to asbestos and aluminum have also been associated with iPPFE.10,11 Although several disease associations have been described, no single cause has been unequivocally identified.

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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].

    Reproduction of this article is prohibited without written permission from the American College of Chest Physicians. See online for more details.

    originally published Online First June 19, 2014.

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