利用定量 CT 评估淋巴管平滑肌瘤病的肺部损害.

Objective To quantify the degree of pulmonary cystic damage in CT and compare its correlation with pulmonary function tests（PFTs）and serum levels of vascular endothelial growth factor （VEGF-D），and to study the potential of quantitative CT to assess the degree of lung damage and decide treatment in lymphangioleiomyomatosis（LAM）. Methods Pulmonary cystic areas in CT images were identified by the artificial intelligence software.The volumetric percentage of cystic regions of the total lung volume was calculated to get a cyst score.The association between the 36 times of CT scan and PFTs were analyzed in 21 patients. The correlation of serum VEGF-D levels of 17 patients and their 24 times of CT scan were studied.A ROC curve was calculated to select the threshold at which the cyst scores could predict the lung impairment best. Results Cyst scores correlated significantly with percentage of predicted forced of expiratory volume in one second （FEV1%pred）（r=-0.72，P<0.001） and percentage of predicted diffusion capacity（DLCO/VA%pred）（r=-0.821，P<0.000 1），while its correlation with serum VEGF-D levels（r=-0.143，P=0.506）was not obvious. The Youden index is max（0.667）with a 94.44% sensitivity and a 72.22% specificity when cyst score is 15.80%. Conclusion Pulmonary cyst scores on CT is strongly correlated with FEV1%pred and DLCO/VA% pred. However，the association with serum levels of VEGF-D is not obvious. Cyst score>15.80% might indicate an abnormal lung function and patients are recommended a sirolimus treatment. [ABSTRACT FROM AUTHOR]