08.01.2019 | INTERSTITIAL LUNG DISEASE | Ausgabe 2/2019
Concentration of Serum Vascular Endothelial Growth Factor (VEGF-D) and Its Correlation with Functional and Clinical Parameters in Patients with Lymphangioleiomyomatosis from a Brazilian Reference Center
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- Lung > Ausgabe 2/2019
Wichtige Hinweise
Electronic supplementary material
The online version of this article (https://doi.org/10.1007/s00408-018-00191-3) contains supplementary material, which is available to authorized users.
Alexandre Franco Amaral, Martina Rodrigues de Oliveira, Fábio Eiji Arimura, Carolina Salim Gonçalves Freitas, Carlos Roberto Ribeiro Carvalho, and Bruno Guedes Baldi—on behalf of Tuberous Sclerosis, Lymphangioleiomyomatosis and Angiomyolipima Study Group, Universidade de Sao Paulo, Brazil.
The members of Tuberous Sclerosis, Lymphangioleiomyomatosis and Angiomyolipoma Study Group are listed in Acknowledgements section.
Abstract
Introduction
Serum vascular endothelial growth factor-D (VEGF-D) is a lymphangiogenic growth factor that is considered a valuable tool in the diagnosis of lymphangioleiomyomatosis (LAM). Previous studies have reported a wide variability in VEGF-D serum levels in LAM patients and it seems to be associated with pulmonary impairment and lymphatic involvement.
Methods
We conducted a cross-sectional study from 2009 to 2017 that evaluated VEGF-D serum levels in a cohort of LAM patients who were never treated with mTOR inhibitors and compared them to healthy age-matched volunteers. Clinical and functional parameters were assessed and correlated with their respective serum VEGF-D levels.
Results
One hundred and four patients were included in the analysis. Serum VEGF-D levels were higher in LAM patients compared to healthy controls: 796 (404–1588) versus 162 (117–232) pg/mL, respectively (p < 0.001). Patients with tuberous sclerosis complex–LAM, TSC–LAM (20%), had higher levels of VEGF-D when compared to patients with sporadic LAM (80%) [1005 (641–2732) vs. 772 (370–1383), p = 0.05]. Serum VEGF-D levels were weakly correlated with DLCO (r = − 0.26, p = 0.001) and lymphatic involvement was more frequent in those with serum VEGF-D levels equal or above 800 pg/mL (35% vs. 13%, p = 0.02).
Conclusions
In LAM, serum VEGF-D is weakly associated with lung function impairment and strongly associated with lymphatic involvement. VEGF-D is validated for use in Brazilian patients with LAM whose characteristics must be accounted for when evaluating their serum VEGF-D levels.
