Watanabe Kentaro, Ishii Hiroshi, Kiyomi Fumiaki, Terasaki Yasuhiro, Hebisawa Akira, Kawabata Yoshinori, Johkoh Takeshi, Sakai Fumikazu, Kondoh Yasuhiro, Inoue Yoshikazu, Azuma Arata, Suda Takafumi, Ogura Takashi, Inase Naohiko, Homma Sakae
General Medical Research Center, Fukuoka University School of Medicine, 814-0180 Fukuoka, Japan.
Department of Respiratory Medicine, Fukuoka University Hospital, Fukuoka, Japan.
Respir Investig. 2019 Jul;57(4):312-320. doi: 10.1016/j.resinv.2019.02.007. Epub 2019 Apr 10.
Physicians have few opportunities to perform surgical lung biopsy (SLB) to diagnose idiopathic pleuroparenchymal fibroelastosis (IPPFE). Therefore, diagnostic criteria for IPPFE that do not require SLB must be established. Herein, we propose diagnostic criteria for IPPFE with and without SLB.
The diagnostic criteria for IPPFE with SLB are histological, based on computed tomography (CT) lesions compatible with PPFE, predominantly in the upper lobes. The three diagnostic criteria for IPPFE without SLB are as follows: (1) radiologically possible IPPFE (a radiological criterion confirming CT lesions in both lung apexes, regardless of the lower lobe lesions); (2) radiologically probable IPPFE (also a radiological criterion, but mandatory to confirm chest radiograph findings of bilateral upward shift of the hilar structures and/or CT findings of volume loss of the upper lobes); (3) radiologically and physiologically probable IPPFE. Our data from 41 patients with IPPFE and 97 with idiopathic pulmonary fibrosis (IPF) showed that the percentage of the predicted values of the ratio of residual volume to total lung capacity (RV/TLC %pred.) ≥115% and body mass index (BMI) ≤20 kg/m plus RV/TLC %pred. ≥80% performed well for discriminating IPPFE from IPF. These parameters were thus added to criterion (3).
We have proposed diagnostic criteria for IPPFE in patients with and without SLB. Both imaging criteria and physiological criteria using RV/TLC and BMI successfully discriminate IPPFE from chronic IIPs when SLB cannot be performed.
医生很少有机会通过手术肺活检（SLB）来诊断特发性胸膜肺实质纤维弹性组织增生症（IPPFE）。因此，必须建立无需SLB的IPPFE诊断标准。在此，我们提出了有或无SLB情况下的IPPFE诊断标准。
有SLB时IPPFE的诊断标准基于组织学，依据与胸膜肺实质纤维弹性组织增生症（PPFE）相符的计算机断层扫描（CT）病变，主要位于上叶。无SLB时IPPFE的三项诊断标准如下：（1）放射学上可能的IPPFE（一项放射学标准，确认双侧肺尖有CT病变，无论下叶病变情况）；（2）放射学上很可能的IPPFE（也是一项放射学标准，但必须确认胸部X线片显示肺门结构双侧上移以及/或者CT显示上叶容积缩小）；（3）放射学和生理学上很可能的IPPFE。我们对41例IPPFE患者和97例特发性肺纤维化（IPF）患者的数据显示，残气量与肺总量比值（RV/TLC %pred.）≥115%且体重指数（BMI）≤20 kg/m²加上RV/TLC %pred.≥80%的预测值百分比在区分IPPFE与IPF方面表现良好。因此这些参数被添加到标准（3）中。
我们提出了有或无SLB情况下IPPFE患者的诊断标准。当无法进行SLB时，使用RV/TLC和BMI的影像学标准和生理学标准都能成功区分IPPFE与慢性间质性肺疾病（IIP）。
