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Serum Vascular Endothelial Growth Factor-D Prospectively Distinguishes Lymphangioleiomyomatosis From Other Diseases

  1. Author:
    Young, L. R.
    VanDyke, R.
    Gulleman, P. M.
    Inoue, Y.
    Brown, K. K.
    Schmidt, L. S.
    Linehan, W. M.
    Hajjar, F.
    Kinder, B. W.
    Trapnell, B. C.
    Bissler, J. J.
    Franz, D. N.
    McCormack, F. X.

  2. Author Address

    [Young, Lisa R.; Hajjar, Fuad; Kinder, Brent W.; McCormack, Francis X.] Univ Cincinnati, Coll Med, Div Pulm Crit Care & Sleep Med, Cincinnati, OH 45267 USA. [Young, Lisa R.; VanDyke, Rhonda; Gulleman, Peter M.] Cincinnati Childrens Hosp, Med Ctr, Div Pulm Med, Cincinnati, OH USA. [VanDyke, Rhonda] Cincinnati Childrens Hosp, Med Ctr, Div Biostat, Cincinnati, OH USA. [Trapnell, Bruce C.] Cincinnati Childrens Hosp, Med Ctr, Div Pulm Biol, Cincinnati, OH USA. [Bissler, John J.] Cincinnati Childrens Hosp, Med Ctr, Div Nephrol, Cincinnati, OH USA. [Franz, David N.] Cincinnati Childrens Hosp, Med Ctr, Div Neurol, Cincinnati, OH USA. [Inoue, Yoshikazu] Natl Hosp Org, Kinki Chuo Chest Med Ctr, Osaka, Japan. [Brown, Kevin K.] Natl Jewish Hlth, Dept Med, Denver, CO USA. [Schmidt, Laura S.] SAIC Frederick Inc, NCI Frederick, Basic Res Program, Frederick, MD USA. [Schmidt, Laura S.; Linehan, W. Marston] NCI, Urol Oncol Branch, NIH, Bethesda, MD 20892 USA.;McCormack, FX, Univ Cincinnati, Coll Med, Div Pulm Crit Care & Sleep Med, 231 Albert Sabin Way,Med Sci Bldg,Room 6114,MLC 0, Cincinnati, OH 45267 USA.;Frank.McCormack@uc.edu
    1. Year: 2010
    2. Date: Sep
  2. Journal: Chest
    1. 138
    2. 3
    3. Pages: 674-681
  4. Type of Article: Article
  5. ISSN: 0012-3692
  1. Abstract:

    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 <.001). In the cohort evaluated prospectively, 12 of the 15 individuals ultimately diagnosed with LAM by biopsy had VEGF-D levels of > 800 pg/mL, whereas levels were <600 pg/mL in all 18 subjects later diagnosed with other causes of cystic lung disease. Receiver operating characteristic curves demonstrated that VEGF-D effectively identified LAM, with an area under the curve of 0.961(95% CI, 0.923-0.992). A VEGF-D level of > 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<.001). Conclusions: A serum VEGF-D level of > 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

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External Sources

  1. View Full Text
  2. DOI: 10.1378/chest.10-0573
  3. View record in Web of Science®
  4. WOS: 000282561500033

Library Notes

  1. Fiscal Year: FY2009-2010
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