Shiiya Haruhiko, Sato Masaaki
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.
J Clin Med. 2021 Mar 1;10(5):957. doi: 10.3390/jcm10050957.
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.
胸膜实质纤维弹性组织增生症（PPFE）是一种与间质性肺炎相关的新疾病实体，其特征为胸膜和胸膜下肺实质出现纤维化和弹性组织变性，主要累及上叶。随着对这一疾病实体认识的增加，许多研究揭示了PPFE的患病率、发病率、临床和病理特征以及疾病进程。据报道，PPFE患者有几个独特的临床特征，包括极低的体重指数、身材消瘦和胸壁畸形，即所谓的“扁平胸”。随着这种疾病的进展，肺部萎缩常常会导致危及生命的并发症，如气胸及相关的空气泄漏综合征。肺移植被认为是晚期PPFE患者唯一有效的治疗方法；然而，关于PPFE特征对肺移植结果的影响知之甚少。本综述重点关注PPFE独特的临床病理特征以及这些患者肺移植的相关结果。
