Department of Pulmonary and Critical Care Medicine, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Republic of Korea.
Department of Pulmonary and Critical Care Medicine, Chungnam National University Hospital, Chungnam National University School of Medicine, Daejeon, Republic of Korea.
Respirology. 2020 Oct;25(10):1046-1052. doi: 10.1111/resp.13796. Epub 2020 Mar 9.
PPFE is characterized by fibrosis in the pleura and subpleural lung parenchyma in the upper lobes, while other types of ILD, mainly UIP, can be observed in about half of the patients in their lower lobes. The aim of this study was to evaluate the clinical significance of the radiologically defined PPFE in patients with IPF.
Clinical data and chest CT images were retrospectively analysed in 445 patients with IPF (biopsy-proven cases, n = 165). The radiological criteria of PPFE were defined as follows: (i) bilateral subpleural dense fibrosis with or without pleural thickening in the upper lobes, (ii) evidence of disease progression and (iii) no clinical evidence of identifiable aetiologies.
The median follow-up period was 43.0 months. The mean age of the patients was 66.4 years and 76.4% were male. PPFE was identified in 28 patients (6.3%). The PPFE group showed lower BMI and lung function (FVC and TLC) at baseline, more frequent pneumothorax and pneumomediastinum, higher decline rates in lung function and poorer prognosis during follow-up than the no-PPFE group. PPFE was an independent risk factor (HR = 2.953, 95% CI: 1.350-6.460, P = 0.007) for pneumothorax or pneumomediastinum, but not for mortality in patients with IPF.
Among patients with IPF, the PPFE group, when compared to the no-PPFE group, showed lower BMI and lung function and showed more frequent complications and poorer survival during follow-up.
PPFE 的特征是上叶胸膜和肺实质的纤维化，而下叶则主要表现为其他类型的间质性肺病，主要为 UIP。本研究旨在评估影像学定义的 PPFE 在 IPF 患者中的临床意义。
回顾性分析 445 例 IPF 患者（经活检证实的病例，n=165）的临床资料和胸部 CT 图像。PPFE 的影像学标准定义为：（i）双侧上叶胸膜下致密纤维化，伴或不伴胸膜增厚，（ii）有疾病进展的证据，（iii）无可识别病因的临床证据。
中位随访时间为 43.0 个月。患者的平均年龄为 66.4 岁，76.4%为男性。28 例（6.3%）患者存在 PPFE。PPFE 组患者在基线时的 BMI 和肺功能（FVC 和 TLC）较低，气胸和纵隔气肿的发生率较高，肺功能下降速度较快，随访期间的预后较差。PPFE 是 IPF 患者气胸或纵隔气肿的独立危险因素（HR=2.953，95%CI：1.350-6.460，P=0.007），但不是死亡率的独立危险因素。
在 IPF 患者中，与无 PPFE 组相比，PPFE 组患者的 BMI 和肺功能较低，在随访期间发生并发症和死亡的风险更高。
