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Alveolar Epithelial Denudation Is a Major Factor in the Pathogenesis of Pleuroparenchymal Fibroelastosis

Lung Transplantation for Pleuroparenchymal Fibroelastosis

by 1,2 and 1,*
Department of Thoracic Surgery, The University of Tokyo Graduate School of Medicine, 7-3-1 Hongo, Bunkyo-ku, Tokyo 113-8655, Japan
Department of Cardiovascular and Thoracic Surgery, Hokkaido University Graduate School of Medicine, Kita 15 Nishi 7, Kita-ku, Sapporo 060-8638, Hokkaido, Japan
Author to whom correspondence should be addressed.
Academic Editor: Hiroshi Ishii
J. Clin. Med. 2021, 10(5), 957;
Received: 25 January 2021 / Revised: 20 February 2021 / Accepted: 20 February 2021 / Published: 1 March 2021
(This article belongs to the Special Issue Pleuroparenchymal Fibroelastosis: Obstacles and Challenges)
Pleuroparenchymal fibroelastosis (PPFE), a new disease entity associated with interstitial pneumonia, is characterized by fibrosis and elastosis involving the pleura and subpleural lung parenchyma, predominantly in the upper lobe. As the awareness of this disease entity has increased, many studies have revealed the prevalence and incidence, clinical and pathological characteristics, and disease course of PPFE. Patients with PPFE reportedly have several unique clinical characteristics—including an extremely low body mass index with a slender body and chest wall deformity, known as “flat chest”. As this disease progresses, shrinking of the lungs often causes life-threatening complications, such as pneumothorax, and associated air leak syndrome. Lung transplantation is considered the only effective treatment for patients with advanced PPFE; however, little is known about the influences of the characteristics of PPFE on the outcome of lung transplantation. This review focuses on the unique clinicopathologic characteristics of PPFE and associated outcomes of lung transplantation for these patients. View Full-Text
Keywords: lung transplantation; pleuroparenchymal fibroelastosis; interstitial lung disease; chest wall lung transplantation; pleuroparenchymal fibroelastosis; interstitial lung disease; chest wall
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MDPI and ACS Style

Shiiya, H.; Sato, M. Lung Transplantation for Pleuroparenchymal Fibroelastosis. J. Clin. Med. 2021, 10, 957.

AMA Style

Shiiya H, Sato M. Lung Transplantation for Pleuroparenchymal Fibroelastosis. Journal of Clinical Medicine. 2021; 10(5):957.

Chicago/Turabian Style

Shiiya, Haruhiko, and Masaaki Sato. 2021. "Lung Transplantation for Pleuroparenchymal Fibroelastosis" Journal of Clinical Medicine 10, no. 5: 957.

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