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Original Research: LYMPHANGIOLEIOMYOMATOSIS |

Serum Vascular Endothelial Growth Factor-D Levels in Patients With Lymphangioleiomyomatosis Reflect Lymphatic Involvement

Connie G. Glasgow, BS; Nilo A. Avila, MD; Jing-Ping Lin, MD, PhD; Mario P. Stylianou, PhD; Joel Moss, MD, PhD
Author and Funding Information

*From the Translational Medicine Branch (Ms. Glasgow and Dr. Moss) and Office of Biostatistics Research (Drs. Lin and Stylianou), Division of Prevention and Population Science, National Heart, Lung, and Blood Institute, and the Diagnostic Radiology Department (Dr. Avila), Warren G. Magnuson Clinical Center, National Institutes of Health, Bethesda, MD.

Correspondence to: Joel Moss, MD, PhD, Translational Medicine Branch, NHLBI, National Institutes of Health, Building 10/Room 6D05/MSC-1590, Bethesda, MD 20892-1590; e-mail: mossj@nhlbi.nih.gov


This research was funded by the Intramural Research Program, National Institutes of Health, National Heart, Lung, and Blood Institute.

The authors have reported to the ACCP that no significant conflicts of interest exist with any companies/organizations whose products or services may be discussed in this article.

Reproduction of this article is prohibited without written permission from the American College of Chest Physicians (www.chestjournal.org/site/misc/reprints.xhtml).


© 2009 American College of Chest Physicians


Chest. 2009;135(5):1293-1300. doi:10.1378/chest.08-1160
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Background:  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.

Methods:  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.

Results:  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).

Conclusions:  Serum VEGF-D concentration is a measure of lymphatic involvement in patients with LAM.

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