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

Distinct characteristics of pleuroparenchymal fibroelastosis with usual interstitial pneumonia compared with idiopathic pulmonary fibrosis.

Author information

1
From the Department of Respiratory Medicine, Kanagawa Cardiovascular and Respiratory Center, Yokohama, Japan; Department of Respiratory Medicine, Nagoya University Graduate School of Medicine, Nagoya, Japan.
2
From the Department of Respiratory Medicine, Kanagawa Cardiovascular and Respiratory Center, Yokohama, Japan. Electronic address: ogura@kanagawa-junko.jp.
3
From the Department of Respiratory Medicine, Kanagawa Cardiovascular and Respiratory Center, Yokohama, Japan.
4
Department of Radiology, Kanagawa Cardiovascular and Respiratory Center, Yokohama, Japan.
5
Department of Pathobiology, Yokohama City University Graduate School of Medicine, Yokohama, Japan.
6
Department of Pathology, Japan Red Cross Medical Center, Tokyo, Japan.
7
Department of Diagnostic Radiology, International Medical Center of Saitama Medical University, Saitama, Japan.
8
Department of Respiratory Medicine, Nagoya University Graduate School of Medicine, Nagoya, Japan.

Abstract

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.

PMID:
25367470
DOI:
10.1378/chest.13-2866
[Indexed for MEDLINE]
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