Serum Vascular Endothelial Growth Factor-D Prospectively Distinguishes Lymphangioleiomyomatosis From Other Diseases
Serum Vascular Endothelial Growth Factor-D Prospectively Distinguishes Lymphangioleiomyomatosis From Other Diseases
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DOI：
10.1378/chest.10-0573
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发表时间：
2010-09-01
期刊：
影响因子：
9.6
通讯作者：
McCormack, Francis X.
中科院分区：
文献类型：
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作者：
Young, Lisa R.;VanDyke, Rhonda;McCormack, Francis X.
目的：大多数患有淋巴管平滑肌瘤病（LAM）的女性表现为囊性肺疾病，大多数需要肺活检才能确诊。本研究的目的是确定血管内皮生长因子-D（VEGF-D），淋巴管生成生长因子.Methods：我们前瞻性地测定了48名女性囊性肺疾病的血清VEGF-D水平的血清学检测的前瞻性诊断有用性。诊断测试性能是从195名妇女的队列中确定的，这些妇女患有结节性硬化症复合体（TSC）、TSC-LAM、散发性LAM（S-LAM）和鉴别诊断中的其他囊性肺疾病，包括活检证实或遗传学证实的肺朗格汉斯细胞组织细胞增多症、肺气肿、舍格伦综合征或Birt-Hogg-Dube综合征。S-LAM组血清VEGF-D水平显著高于对照组，（中位数1，175 [四分位距（IQR）：780- 2，013] pg/mL; n = 56）（中位数281 [IQR 203-351] pg/mL; n=44，P 800 pg/mL，而水平600 pg/mL与LAM的诊断高度相关（特异性97.6%，似然比35.2），>800 pg/mL的值具有诊断特异性。TSC-LAM女性患者的血清VEGF-D水平（中位数3，465 [IQR 1，970 - 7，1951 pg/mL]）与仅TSC女性患者（中位数370 [IQR 291-5201 pg/mL]）相比显著升高，高分辨率CT（HRCT）扫描显示具有典型囊性改变的女性患者的P 800 pg/mL在诊断S-LAM方面具有特异性，可识别TSC女性患者的LAM。阴性VEGF-D结果并不能排除LAM的诊断。血清VEGF-D检测在男性或女性谁没有囊性肺疾病的HRCT扫描是未知的有用。胸部2010; 138（3）：674-681
Objectives: The majority of women with lymphangioleiomyomatosis (LAM) present with cystic lung disease, and most require lung biopsy for definitive diagnosis. The purpose of this study was to determine the prospective diagnostic usefulness of a serologic test for vascular endothelial growth factor-D (VEGF-D), a lymphangiogenic growth factor.Methods: We prospectively measured serum VEGF-D levels by enzyme-linked immunoassay in 48 women presenting with cystic lung disease. Diagnostic test performance was determined from a cohort of 195 women, with tuberous sclerosis complex (TSC), TSC-LAM, sporadic LAM (S-LAM), and other cystic lung diseases in the differential diagnosis, including biopsy-proven or genetically proven pulmonary Langerhans cell histiocytosis, emphysema, Sjogren syndrome, or Birt-Hogg-Dube syndrome.Results: Serum VEGF-D levels were significantly greater in S-LAM (median 1,175 [interquartile range (IQR): 780-2,013] pg/mL; n = 56) than in other cystic lung diseases (median 281 [IQR 203-351] pg/mL; n=44, P 800 pg/mL, whereas levels were 600 pg/mL was highly associated with a diagnosis of LAM (specificity 97.6%, likelihood ratio 35.2) and values >800 pg/mL were diagnostically specific. Serum VEGF-D levels were significantly elevated in women with TSC-LAM (median 3,465 [IQR 1,970-7,1951 pg/mL) compared with women with TSC only (median 370 [IQR 291-5201 pg/mL), P 800 pg/mL in a woman with typical cystic changes on high-resolution CT (HRCT) scan is diagnostically specific for S-LAM and identifies LAM in women with TSC. A negative VEGF-D result does not exclude the diagnosis of LAM. The usefulness of serum VEGF-D testing in men or in women who do not have cystic lung disease on HRCT scan is unknown. CHEST 2010; 138(3):674-681