1 Department of Radiology and Research Institute of Radiology, University of Ulsan College of Medicine, Asan Medical Center, 88 Olympic-Ro 43 Gil, Songpa-gu, Seoul 05505, Republic of Korea.
2 Department of Pulmonary and Critical Care Medicine, University of Ulsan College of Medicine, Asan Medical Center, Seoul, Republic of Korea.
AJR Am J Roentgenol. 2019 Apr;212(4):766-772. doi: 10.2214/AJR.18.20232. Epub 2019 Jan 23.
The purposes of this study were to identify diagnostic imaging markers for differentiating pulmonary cysts in lymphangioleiomyomatosis and Birt-Hogg-Dubé syndrome and to identify potential risk factors for spontaneous pneumothorax in the two diseases.
This retrospective study included 44 patients with lymphangioleiomyomatosis (44 women; mean age, 35 ± 10.9 years) and 13 patients with Birt-Hogg-Dubé syndrome (nine men, four women; mean age, 45.1 ± 10.9 years). CT findings were analyzed to determine the shape; presence of septation, wall visibility, and subpleural cysts; size; number; distribution; location of the largest cyst; and presence of cysts encircling the bronchovascular bundle ("air-cuff" sign) and of mediastinal fat indentation. Multiple logistic regression was performed to identify risk factors for spontaneous pneumothorax.
Compared with patients with lymphangioleiomyomatosis, patients with Birt-Hogg-Dubé syndrome were significantly older, and more of them were men. The cysts in these patients had a more irregular shape, more septation, lower and more peripheral distribution, larger maximum size, and more attachment to the pleura, air-cuff sign, indentation on mediastinal fat, and subpleural cysts larger than 2 cm. The maximum diameter of cysts was the sole independent risk factor for spontaneous pneumothorax (p = 0.027; 95% CI, 1.043-1.992) in both diseases. ROC analysis showed an AUC of 0.745 (95% CI, 0.612-0.851), and the optimal cutoff value was 22 mm (sensitivity, 72.5%; specificity, 76.5%).
Several CT imaging markers may help in differentiating pulmonary cysts in patients with lymphangioleiomyomatosis and those with Birt-Hogg-Dubé syndrome and in predicting spontaneous pneumothorax.
本研究旨在确定淋巴管肌瘤病和 Birt-Hogg-Dubé 综合征肺部囊肿的影像学诊断标志物，并确定这两种疾病自发性气胸的潜在危险因素。
这项回顾性研究纳入了 44 例淋巴管肌瘤病患者（44 名女性；平均年龄 35±10.9 岁）和 13 例 Birt-Hogg-Dubé 综合征患者（9 名男性，4 名女性；平均年龄 45.1±10.9 岁）。分析 CT 表现，以确定囊肿的形状、是否存在分隔、壁的可见性、是否存在胸膜下囊肿、大小、数量、分布、最大囊肿的位置，以及是否存在围绕支气管血管束的囊肿（“空气袖套”征）和纵隔脂肪凹陷。采用多变量逻辑回归分析确定自发性气胸的危险因素。
与淋巴管肌瘤病患者相比，Birt-Hogg-Dubé 综合征患者的年龄显著更大，且男性更多。这些患者的囊肿形状更不规则，分隔更多，分布更低且更外周，最大直径更大，与胸膜附着更多，存在空气袖套征、纵隔脂肪凹陷和胸膜下 2cm 以上的大囊肿。在这两种疾病中，囊肿的最大直径均是自发性气胸的唯一独立危险因素（p=0.027；95%CI，1.043-1.992）。ROC 分析显示 AUC 为 0.745（95%CI，0.612-0.851），最佳截断值为 22mm（敏感性为 72.5%，特异性为 76.5%）。
一些 CT 影像学标志物有助于鉴别淋巴管肌瘤病和 Birt-Hogg-Dubé 综合征患者的肺部囊肿，并预测自发性气胸。
