Correlation of serum vascular endothelial growth factor-D concentration with clinical presentation and course of lymphangioleiomyomatosis.

Background: Increased serum vascular endothelial growth factor D (VEGF-D) concentration has been accepted as a diagnostic marker in lymphangioleiomyomatosis (LAM). The study was performed to evaluate the correlation of VEGF-D with clinical presentation and course of LAM.

Material: The study group comprised of 48 women with LAM (27 with sLAM, 9 with sLAM and lymphangioma (sLAM-LYM) and 12 patients with TSC/LAM). Patients were assessed at the time of VEGF-D examination, and pulmonary function parameters were compared with those, obtained one year before. VEGF-D serum concentration was measured by ELISA method.

Results: Patients with TSC/LAM and sLAM-LYM displayed higher concentrations of VEGF-D than patients with sLAM (2682 ± 1347 pg/mL and 2223 ± 1184 pg/mL vs.1281 ± 791 pg/mL; p = 0.0002, p = 0.009) respectively. Patients with sLAM and VEGF-D concentration <800 pg/mL (sLAM-L) had better lung function as assessed by FEV1 (2.38 ± 0.88 L vs. 1.75 ± 0.8 L; p < 0.015) and DL,CO (5.8 ± 2.25 vs. 3.93 ± 1.74 mL/min/mmHg; p < 0.028), had higher blood oxygenation, then those with VEGF-D >800 pg/mL (sLAM-H). Significant yearly increase of TLC (390 ± 700 mL; p < 0.021) and RV (340 ± 790 mL; p < 0.03), and decrease of distance in 6MWT (-30 ± 50 m; p = 0.04) were observed in sLAM-H group. Lung function parameters remained constant in sLAM-L patients. Patients with sLAM-H displayed higher yearly decline of FVC (120 vs. 50 mL; p = 0.035) and increase of TLC (390 vs. -80 mL; p = 0.038) and RV (340 vs. 90 mL; p = 0.045) than sLAM-L patients. Negative correlations between VEGF-D concentration and DL,CO, PaO2, PaCO2, and positive with HRCT grading, and desaturation in 6MWT were noticed in sLAM patients without lymphangioma.

Conclusions: Serum VEGF-D is the useful biomarker of LAM extension, and might also prove predictive towards therapeutic decision.