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Published in: Lung 2/2019

01-04-2019 | Pulmonary-Function Tests | INTERSTITIAL LUNG DISEASE

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

Authors: Alexandre Franco Amaral, Martina Rodrigues de Oliveira, Olívia Meira Dias, Fábio Eiji Arimura, Carolina Salim Gonçalves Freitas, Milena Marques Pagliarelli Acencio, Vanessa Adélia de Alvarenga, Ronaldo Adib Kairalla, Carlos Roberto Ribeiro Carvalho, Bruno Guedes Baldi, Tuberous Sclerosis, Lymphangioleiomyomatosis and Angiomyolipoma Study Group, Universidade de Sao Paulo, Brazil

Published in: Lung | Issue 2/2019

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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.
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Metadata
Title
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
Authors
Alexandre Franco Amaral
Martina Rodrigues de Oliveira
Olívia Meira Dias
Fábio Eiji Arimura
Carolina Salim Gonçalves Freitas
Milena Marques Pagliarelli Acencio
Vanessa Adélia de Alvarenga
Ronaldo Adib Kairalla
Carlos Roberto Ribeiro Carvalho
Bruno Guedes Baldi
Tuberous Sclerosis, Lymphangioleiomyomatosis and Angiomyolipoma Study Group, Universidade de Sao Paulo, Brazil
Publication date
01-04-2019
Publisher
Springer US
Published in
Lung / Issue 2/2019
Print ISSN: 0341-2040
Electronic ISSN: 1432-1750
DOI
https://doi.org/10.1007/s00408-018-00191-3

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