Vascular endothelial growth factor D in the diagnosis of lymphangioleiomyomatosis

Morphological verification is not always required to make a definitive diagnosis of lymphangioleiomyomatosis (LAM) with a typical picture of cysts in the lungs using high-resolution computed tomography. Vascular endothelial growth factor D – VEGF-D (vascular endothelial growth factor D) is used worldwide as a laboratory marker of LAM with a diagnostic threshold of 800 pg/ml and higher in the blood [1]. There was no such possibility in the Russian Federation until April 2023. The aim of the study was to evaluate the diagnostic value of the serum levels of vascular endothelial growth factor D (VEGF-D) in patients with LAM in the Russian Federation. Methods. Blood serum samples were prospectively collected in the laboratory of the State Budgetary Institution of Healthcare of the city of Moscow “Moscow Clinical Scientific and Practical Center named after A.S.Loginov of the Department of Healthcare of the City of Moscow” from 71 patients with multiple air cavities in the lungs using high-resolution computed tomography of the chest organs. VEGF-D levels were measured using enzyme immunoassay, and diagnostic value was assessed using performance curve analysis (ROC-curve). Results. Patients with identified LAM accounted for 48 (68%) of all the included patients (n = 71) with cystic lung disease, with the average age of 48.2 ± 12.9 years. The group of patients with lung cysts not associated with LAM and the group of patients with LAM significantly differed in VEGF-D levels: 552 ± 276.5 pg/ml vs 1,425 ± 872.1 pg/ml, respectively (p < 0.0001). The level of VEFG-D was higher in women with LAM and extrapulmonary manifestations of the disease than in women without extrapulmonary changes: 1,510 ± 968.6 pg/ml vs 1,328.5 ± 761.0 pg/ml, respectively. However, the difference was not statistically significant (p < 0.06). The level of VEFG-D was higher than 800 pg/ml in 75% of patients with LAM. The area under the ROC curve for VEFG-D in LAM was 0.866 (95% CI – 0.783 – 0.950; p < 0.0001). The threshold value of VEFG-D of 738 pg/ml had a sensitivity of 81% and a specificity of 79% for the diagnosis of an identified LAM. Conclusion. Our data indicate that measurement of VEGF-D blood serum levels allows diagnosing LAM in 75% of cases with clinical and radiological suspicion of LAM without morphological examination of lung tissue.

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