Lung Transplantation for Pleuroparenchymal Fibroelastosis

Overview

Journal J Clin Med

Specialty General Medicine

Date 2021 Apr 3

PMID 33804467

Citations 6

Authors

Haruhiko Shiiya

Masaaki Sato

Affiliations

Soon will be listed here.

Abstract

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.

Citing Articles

Rare interstitial lung diseases: a narrative review.

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Proposed Clinical Algorithm for Pleuroparenchymal Fibroelastosis (PPFE).

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Prognostic value of coexisting conditions and complications in pleuroparenchymal fibroelastosis: a single-center retrospective study.

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Patient selection, surgery and perioperative management in lung transplantation in Japan.

Konoeda C, Sato M J Thorac Dis. 2024; 16(4):2613-2622.

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