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Original Research: Diffuse Lung Disease |

Distinct Characteristics of Pleuroparenchymal Fibroelastosis With Usual Interstitial Pneumonia Compared With Idiopathic Pulmonary FibrosisPleuroparenchymal Fibroelastosis Pneumonia

Tsuneyuki Oda, MD; Takashi Ogura, MD; Hideya Kitamura, MD, PhD; Eri Hagiwara, MD, PhD; Tomohisa Baba, MD; Yasunori Enomoto, MD; Tae Iwasawa, MD, PhD; Koji Okudela, MD, PhD; Tamiko Takemura, MD, PhD; Fumikazu Sakai, MD, PhD; Yoshinori Hasegawa, MD, PhD, FCCP
Author and Funding Information

From the Department of Respiratory Medicine (Drs Oda, Ogura, Kitamura, Hagiwara, Baba, and Enomoto) and Department of Radiology (Dr Iwasawa), Kanagawa Cardiovascular and Respiratory Center, Yokohama; Department of Pathobiology (Dr Okudela), Yokohama City University Graduate School of Medicine, Yokohama; Department of Pathology (Dr Takemura), Japan Red Cross Medical Center, Tokyo; Department of Diagnostic Radiology (Dr Sakai), International Medical Center of Saitama Medical University, Saitama; and Department of Respiratory Medicine (Drs Oda and Hasegawa), Nagoya University Graduate School of Medicine, Nagoya, Japan.

CORRESPONDENCE TO: Takashi Ogura, MD, Department of Respiratory Medicine, Kanagawa Cardiovascular and Respiratory Center, 6-16-1 Tomioka-higashi, Kanazawa-ku, Yokohama 236-0051, Japan; e-mail: ogura@kanagawa-junko.jp


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.


Chest. 2014;146(5):1248-1255. doi:10.1378/chest.13-2866
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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.

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