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Poster Walks: Poster Walk 8: Interstitial lung disease/ICU/Pediatrics |

P235 Serum VEGF-D level as a diagnostic biomarker of lymphangioleiomyomatosis in clinical practice

C. Daccord; V. Aubert; N. Félix; R. Lazor
Author and Funding Information

1Division of Respiratory Medicine

2Division of Immunology and Allergy, Lausanne University Hospital, Lausanne, Switzerland


Copyright 2017, American College of Chest Physicians and Swiss Respiratory Society SGP. All Rights Reserved.


Chest. 2017;151(5_S):A135. doi:10.1016/j.chest.2017.04.142
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Introduction: Lymphangioleiomyomatosis (LAM) is a rare cystic lung disease (CLD) affecting almost exclusively adult women and occurring sporadically (S-LAM) or in association with tuberous sclerosis complex (TSC-LAM). LAM is currently considered as a low-grade metastasizing neoplasm. Vascular endothelial growth factor D (VEGF-D) is a lymphangiogenic growth factor produced by LAM cells which promotes their migration and proliferation. Serum VEGF-D concentration is increased in about 70% of patients with LAM, correlates with disease severity and treatment response to mTOR inhibitors, and has proven to be a useful diagnostic biomarker to distinguish LAM from other CLD, thus obviating the need for confirmatory lung biopsy in 10% of cases. A VEGF-D level of > 600 pg/ml identifies LAM among other CLD with a sensitivity of 84% and a specificity of 98%, whereas a threshold of > 800 pg/ml has a sensitivity of 73% and a specificity of 100%.

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