Department of Pulmonary and Critical Care Medicine, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Peking Union Medical College, Beijing, 100730, China.
Department of Internal Medicine, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Peking Union Medical College, Beijing, 100730, China.
Orphanet J Rare Dis. 2020 Jul 6;15(1):176. doi: 10.1186/s13023-020-01448-y.
The purpose of this study was to create a practical CT-based algorithm to differentiate Birt-Hogg-Dubé (BHD) syndrome from other diffuse cystic lung diseases (DCLD).
The study was a retrospective review of the CT images of 33 patients with BHD syndrome, 33 patients with LAM, and 23 patients with NBNL (non-BHD and non-LAM) among DCLD patients. On the basis of the data collected, the CT images were reviewed again to evaluate the characteristics (size, number, distribution, and morphology) of pulmonary cysts.
Lower lung-predominant cysts were more likely to be found in patients with BHD syndrome than in patients with LAM or in the NBNL DCLD group. In the axial distribution, 18 of 33 patients in BHD group had cysts that were predominantly near the mediastinum, and all the patients in the LAM and NBNL DCLD groups had diffuse cysts. The appearance of fusiform cysts was more easily observed in patients in the BHD group. In total, 58% patients in the BHD group had less than 50 lung cysts, while all patients in the non-BHD group had more than 50 lung cysts. The biggest cyst was located in the lower lobe in 28 of 33 patients in the BHD group, while 11 of 33 patients in LAM group and 10 patients in the NBNL DCLD group had the biggest cyst in the lower lobe.
The pulmonary cysts in patients with BHD tended to be fusiform, less numerous and located predominantly in the lower lobe and near the mediastinum. These radiologic pulmonary features could assist physicians in differentiating BHD from other DCLDs.
本研究旨在创建一种实用的基于 CT 的算法，以区分 Birt-Hogg-Dubé（BHD）综合征与其他弥漫性囊性肺病（DCLD）。
该研究回顾性分析了 33 例 BHD 综合征、33 例淋巴管肌瘤病（LAM）和 23 例非 BHD 和非 LAM（DCLD）患者的 CT 图像。根据收集的数据，再次对 CT 图像进行评估，以评估肺囊肿的特征（大小、数量、分布和形态）。
与 LAM 或 NBNL DCLD 组患者相比，BHD 综合征患者更易出现下肺为主的囊肿。在轴位分布中，BHD 组 18 例患者的囊肿主要位于纵隔附近，而 LAM 和 NBNL DCLD 组的所有患者均有弥漫性囊肿。BHD 组患者更容易观察到梭形囊肿。BHD 组患者中，有 58%的患者的肺囊肿少于 50 个，而非 BHD 组的所有患者的肺囊肿均多于 50 个。在 BHD 组的 33 例患者中，最大的囊肿位于下叶的有 28 例，而 LAM 组的 33 例患者中，有 11 例最大的囊肿位于下叶，NBNL DCLD 组有 10 例。
BHD 患者的肺囊肿呈梭形，数量较少，主要位于下叶和纵隔附近。这些影像学肺部特征有助于医生区分 BHD 与其他 DCLD。
