Division of Pulmonology and Critical Care Medicine, Department of Internal Medicine, Inje University Haeundae Paik Hospital, Busan, Korea.
Department of Radiology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea.
Korean J Intern Med. 2021 Mar;36(Suppl 1):S132-S141. doi: 10.3904/kjim.2019.303. Epub 2020 Feb 24.
BACKGROUND/AIMS: Pleuroparenchymal fibroelastosis (PPFE) is a type of rare idiopathic interstitial pneumonia that is characterized by predominantly upper lobe involvement with pleural fibrosis and subjacent parenchymal fibrosis. This study aimed to determine the clinico-radiologic-pathologic features and prognosis of Korean patients with PPFE.
A total of 26 patients who were confirmed to have PPFE by lung biopsy, were included, and their clinico-radiologic-pathologic findings were retrospectively analyzed.
The mean follow-up period was 23.8 months, and the mean age of the patients was 62.5 years; 61.5% were men and 50% were smokers. Cough and dyspnea were the most frequent presenting symptoms, and restrictive pattern was the most common observation in lung function. In 84.6% of the subjects, lower lobe involvement was found on chest computed tomography, and the usual interstitial pneumonia (UIP) pattern was the most common (59.1%). Among patients whose lower lobe was biopsied (n = 13), the UIP pattern was the most common (46.2%). Patients with lower lobe involvement were older and walked a shorter distance during the 6-minute walk test, compared to those without. Spontaneous pneumothorax was a common complication (26.9%), and 15.4% of the patients died mostly due to pneumonia (100%). The 1- and 3-year survival rates were 90.2% and 84.5%, respectively.
Clinical features of Korean patients with PPFE were similar to those reported previously; however, lower lobe involvement was more frequent. During follow-up, one-fourth of the patients experienced pneumothorax and one-fifth died from pneumonia.
背景/目的：胸膜肺弹力纤维增生症（PPFE）是一种罕见的特发性间质性肺炎，其特征为主要累及上叶，伴胸膜纤维化和下叶实质纤维化。本研究旨在确定韩国 PPFE 患者的临床-放射-病理特征和预后。
共纳入 26 例经肺活检证实为 PPFE 的患者，回顾性分析其临床-放射-病理表现。
平均随访时间为 23.8 个月，患者平均年龄为 62.5 岁；61.5%为男性，50%为吸烟者。咳嗽和呼吸困难是最常见的首发症状，肺功能最常见的表现为限制性模式。84.6%的患者胸部 CT 显示下叶受累，最常见的表现为普通型间质性肺炎（UIP）模式（59.1%）。在下叶活检的患者中（n=13），UIP 模式最常见（46.2%）。与无下叶受累的患者相比，有下叶受累的患者年龄较大，6 分钟步行试验中行走的距离较短。自发性气胸是常见的并发症（26.9%），15.4%的患者死亡，主要死于肺炎（100%）。1 年和 3 年生存率分别为 90.2%和 84.5%。
韩国 PPFE 患者的临床特征与既往报道的相似，但下叶受累更为常见。在随访过程中，四分之一的患者发生气胸，五分之一的患者死于肺炎。
