肺淋巴管肌瘤病CT特点及与血清学指标的关系
|
摘要
目的:探讨淋巴管肌瘤病(LAM)患者胸部影像学特征并评估肺气囊体积及其与患者体内血管内皮生长因子D(VEGF-D)的关系,以帮助提高对该疾病特征的认识。方法:分析自2015年8月至2017年6月于我院行多层螺旋计算机断层(MSCT)扫描的LAM患者胸部影像学表现,并对每位患者行肺气囊体积的测定;并行血清VEGF-D的测定。结果:本研究共收集69例LAM患者胸部CT数据,患者平均肺体积为4255.66±1000.89ml,平均肺气囊体积为1330.6±836.17ml,肺气囊体积平均占肺体积比为28.7%±13.9%。本研究中气囊分布于左右肺之间无统计学差异(P=0.84);而双肺上中叶肺气囊分布与双肺下叶肺气囊分布有统计学差异(P<0.01),双肺上中叶肺气囊体积分布(32.3%±14.6%)较双肺下叶分布(24.4%±14.6%)多;46例患者同意进行VEGF-D测定,血清VEGF-D的中位数为2925.0pg/ml(251.750~12000.000pg/ml),其升高水平与气囊体积占比无相关关系(r=0.12,P=0.41)。此外,69例患者中,7例(10.1%)患者合并气胸;17例(24.6%)患者合并胸腔积液;11例(15.9%)患者存在磨玻璃样改变;肺部实变和小叶间隔增厚各19(27.5%)例。结论:淋巴管肌瘤病患者主要肺部表现为弥漫分布的气囊,肺气囊分布上中叶多于下叶。患者体内VEGF-D的升高水平不能作为评价该疾病严重程度的指标。
Purpose: To evaluate the imaging features of lymphangioleiomyomatosis(LAM) in lungs and quantify the volume of pulmonary cysts and their association with vascular endothelial growth factor(VEGF-D). Methods: Patients with LAM who had undergone chest MSCT examinations in our hospital from August 2015 to June 2017 were enrolled. The volume of pulmonary cysts and serum levels of VEGF-D were measured. Results: Sixty-nine patients were included in our study. The average volume of both lungs in these patients was 4255.66±1000.89 cc(mean±SD), the average volume of pulmonary cysts was 1330.6±836.17 cc, the proportion of pulmonary cysts to lung volume was 28.7±13.9%. The distribution of lung cysts was with no statistical significant difference between left and right lung(P=0.84).But,there were statistical significant differences between middleupper and lower lungs(P<0.01),there were more lung cysts in middle-upper lungs(32.3±14.6%).The median VEGF-D serum level was 2925.0pg/ml(251.750pg/ml-12000.000 pg/ml)in 46 patients.There was no correlation with the elevatory of VEGF-D(r=0.12,P=0.41).Besides,in these 69 patients,pneumothorax was found in 7(10.1%)patients;17(24.6%)patients have chylothorax;ground-glass opacity,air-space consolidation and inter-lobular septal thickening were also frequent(11/15.9%,19/27.5%and 19/27.5%respectively).Conclusion:Diffuse lung cysts are the most common imaging feature in patients with LAM,there are more cysts in middle-upper lungs than that in lower lungs.And the elevatory degree of VEGF-D cannot be defined as a valuable tool to access the disease severity in LAM.
Purpose: To evaluate the imaging features of lymphangioleiomyomatosis(LAM) in lungs and quantify the volume of pulmonary cysts and their association with vascular endothelial growth factor(VEGF-D). Methods: Patients with LAM who had undergone chest MSCT examinations in our hospital from August 2015 to June 2017 were enrolled. The volume of pulmonary cysts and serum levels of VEGF-D were measured. Results: Sixty-nine patients were included in our study. The average volume of both lungs in these patients was 4255.66±1000.89 cc(mean±SD), the average volume of pulmonary cysts was 1330.6±836.17 cc, the proportion of pulmonary cysts to lung volume was 28.7±13.9%. The distribution of lung cysts was with no statistical significant difference between left and right lung(P=0.84).But,there were statistical significant differences between middleupper and lower lungs(P<0.01),there were more lung cysts in middle-upper lungs(32.3±14.6%).The median VEGF-D serum level was 2925.0pg/ml(251.750pg/ml-12000.000 pg/ml)in 46 patients.There was no correlation with the elevatory of VEGF-D(r=0.12,P=0.41).Besides,in these 69 patients,pneumothorax was found in 7(10.1%)patients;17(24.6%)patients have chylothorax;ground-glass opacity,air-space consolidation and inter-lobular septal thickening were also frequent(11/15.9%,19/27.5%and 19/27.5%respectively).Conclusion:Diffuse lung cysts are the most common imaging feature in patients with LAM,there are more cysts in middle-upper lungs than that in lower lungs.And the elevatory degree of VEGF-D cannot be defined as a valuable tool to access the disease severity in LAM.
引文
[1]Harknett E C,Chang W Y,Byrnes S,et al.Use of variability in national and regional data to estimate the prevalence of lymphangioleiomyomatosis.QJM,2011,104:971.
[2]Schiavina M,Di S V,Contini P,et al.Pulmonary lymphangioleiomyomatosis in a karyotypically normal man without tuberous sclerosis complex.Am J Respir Crit Care Med,2007,176:96.
[3]Young L R,Vandyke R,Gulleman P M,et al.Serum vascular endothelial growth factor-D prospectively distinguishes lymphangioleiomyomatosis from other diseases.Chest,2010,138:674-681.
[4]Xu K F,Zhang P,Tian X,et al.The role of vascular endothelial growth factor-D in diagnosis of lymphangioleiomyomatosis(LAM).Respir Med.,2013,107:263.
[5]Johnson S R,Cordier J F,Lazor R,et al.European Respiratory Society guidelines for the diagnosis and management of lymphangioleiomyomatosis.Eur Respir J,2010,35:14.
[6]Cui Y,Steagall W K,Lamattina A M,et al.Aberrant SYK Kinase Signaling Is Essential for Tumorigenesis Induced by TSC2 Inactivation.Cancer Res,2017,77:1492-1502.
[7]Taveira-Dasilva A M,Moss J.EPIDEMIOLOGY,PATHOGENESIS and DIAGNOSIS of LYMPHANGIOLEIOMYOMATOSIS.Expert Opin Orphan Drugs,2016,4:369-378.
[8]Kirchner J,Stein A,Viel K,et al.Pulmonary lymphangioleiomyomatosis:high-resolution CT findings.European Radiol,1999,9:49-54.
[9]Tobino K,Johkoh T,Fujimoto K,et al.Computed tomographic features of lymphangioleiomyomatosis:Evaluation in 138 patients.Eur J Radiol,2015,84:534-541.
[10]Baldi B G,Araujo M S,Freitas C S G,et al.Evaluation of the Extentof Pulmonary Cysts and Their Association with Functional Variables and Serum Markers in Lymphangioleiomyomatosis(LAM).Lung,2014,192:967-974.
[11]Wahid S,Chiang P C,Luo H L,et al.Pelvic lymphangioleiomyomatosis treated successfully with everolimus:Two case reports with literature review.Medicine,2017,96:e4562.
[2]Schiavina M,Di S V,Contini P,et al.Pulmonary lymphangioleiomyomatosis in a karyotypically normal man without tuberous sclerosis complex.Am J Respir Crit Care Med,2007,176:96.
[3]Young L R,Vandyke R,Gulleman P M,et al.Serum vascular endothelial growth factor-D prospectively distinguishes lymphangioleiomyomatosis from other diseases.Chest,2010,138:674-681.
[4]Xu K F,Zhang P,Tian X,et al.The role of vascular endothelial growth factor-D in diagnosis of lymphangioleiomyomatosis(LAM).Respir Med.,2013,107:263.
[5]Johnson S R,Cordier J F,Lazor R,et al.European Respiratory Society guidelines for the diagnosis and management of lymphangioleiomyomatosis.Eur Respir J,2010,35:14.
[6]Cui Y,Steagall W K,Lamattina A M,et al.Aberrant SYK Kinase Signaling Is Essential for Tumorigenesis Induced by TSC2 Inactivation.Cancer Res,2017,77:1492-1502.
[7]Taveira-Dasilva A M,Moss J.EPIDEMIOLOGY,PATHOGENESIS and DIAGNOSIS of LYMPHANGIOLEIOMYOMATOSIS.Expert Opin Orphan Drugs,2016,4:369-378.
[8]Kirchner J,Stein A,Viel K,et al.Pulmonary lymphangioleiomyomatosis:high-resolution CT findings.European Radiol,1999,9:49-54.
[9]Tobino K,Johkoh T,Fujimoto K,et al.Computed tomographic features of lymphangioleiomyomatosis:Evaluation in 138 patients.Eur J Radiol,2015,84:534-541.
[10]Baldi B G,Araujo M S,Freitas C S G,et al.Evaluation of the Extentof Pulmonary Cysts and Their Association with Functional Variables and Serum Markers in Lymphangioleiomyomatosis(LAM).Lung,2014,192:967-974.
[11]Wahid S,Chiang P C,Luo H L,et al.Pelvic lymphangioleiomyomatosis treated successfully with everolimus:Two case reports with literature review.Medicine,2017,96:e4562.