Tsuneyuki Oda1, Takashi Ogura2, Hideya Kitamura3, Eri Hagiwara3, Tomohisa Baba3, Yasunori Enomoto3, Tae Iwasawa4, Koji Okudela5, Tamiko Takemura6, Fumikazu Sakai7, Yoshinori Hasegawa8. 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.
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.
Authors: Paula Silva Gomes; Christina Shiang; Gilberto Szarf; Ester Nei Aparecida Martins Coletta; Carlos Alberto de Castro Pereira Journal: J Bras Pneumol Date: 2017-01-23 Impact factor: 2.624