Glasgow Connie G, Avila Nilo A, Lin Jing-Ping, Stylianou Mario P, Moss Joel
Translational Medicine Branch, National Institutes of Health, Bethesda, MD.
Diagnostic Radiology Department, Warren G. Magnuson Clinical Center, National Institutes of Health, Bethesda, MD.
Chest. 2009 May;135(5):1293-1300. doi: 10.1378/chest.08-1160.
Lymphangioleiomyomatosis (LAM) is a rare multisystem disorder affecting primarily women of child-bearing age, and characterized by cystic lung destruction, tumors of the kidney (angiomyolipomas [AMLs]), and involvement of the axial lymphatics (lymphangioleiomyomas). Patients with LAM experience loss of pulmonary function attributed to the proliferation of abnormal-appearing smooth muscle-like cells (LAM cells). It is possible to group the LAM population by the presence or absence of extrapulmonary involvement (eg, AMLs, lymphangioleiomyomas, chylous effusions). Serum vascular endothelial growth factor (VEGF)-D, a lymphangiogenic factor, is higher in LAM patients than in healthy volunteers and has been proposed as a tool in the differential diagnosis of cystic lung disease. We assessed serum VEGF-D concentrations in relationship to clinical phenotype in LAM patients.
Serum VEGF-D levels were quantified by enzyme immunosorbent assay for 111 patients with LAM and 40 healthy volunteers. VEGF-D levels in patients with pulmonary LAM, with or without extrapulmonary manifestations, were compared to those of healthy volunteers.
Serum VEGF-D levels were greater in patients with LAM compared to those of healthy volunteers (p < 0.001). However, when patient samples were grouped based on the extent of lymphatic extrapulmonary involvement (eg, lymphangioleiomyomas and adenopathy), the statistical difference was maintained only for patients with LAM with lymphatic involvement (p < 0.001), not for those patients whose disease was restricted to the lung. Serum VEGF-D levels are a good biomarker for lymphatic involvement (area under the curve [AUC], 0.845; p < 0.0001), and a fair predictor for LAM disease (AUC, 0.751; p < 0.0001). Serum levels correlated to CT scan grade (p = 0.033).
Serum VEGF-D concentration is a measure of lymphatic involvement in patients with LAM.
淋巴管平滑肌瘤病（LAM）是一种罕见的多系统疾病，主要影响育龄女性，其特征为肺囊性破坏、肾脏肿瘤（血管平滑肌脂肪瘤[AMLs]）以及轴索淋巴管受累（淋巴管平滑肌瘤）。LAM患者因异常的平滑肌样细胞（LAM细胞）增殖而出现肺功能丧失。根据是否存在肺外受累情况（如AMLs、淋巴管平滑肌瘤、乳糜性胸腔积液），可将LAM患者进行分组。血清血管内皮生长因子（VEGF）-D是一种淋巴管生成因子，LAM患者血清中的VEGF-D水平高于健康志愿者，有人提出将其作为诊断肺囊性疾病的一项指标。我们评估了LAM患者血清VEGF-D浓度与临床表型之间的关系。
采用酶联免疫吸附测定法对111例LAM患者和40名健康志愿者的血清VEGF-D水平进行定量分析。比较有或无肺外表现的肺LAM患者与健康志愿者的VEGF-D水平。
LAM患者血清VEGF-D水平高于健康志愿者（p<0.001）。然而，根据肺外淋巴管受累程度（如淋巴管平滑肌瘤和淋巴结病）对患者样本进行分组时，仅LAM伴淋巴管受累患者的差异具有统计学意义（p<0.001），而疾病局限于肺部的患者则无统计学差异。血清VEGF-D水平是淋巴管受累的良好生物标志物（曲线下面积[AUC]，0.845；p<0.0001），也是LAM疾病的较好预测指标（AUC，0.751；p<0.0001）。血清水平与CT扫描分级相关（p=0.033）。
血清VEGF-D浓度可作为衡量LAM患者淋巴管受累情况的指标。
