Serum Vascular Endothelial Growth Factor-D Prospectively Distinguishes Lymphangioleiomyomatosis From Other Diseases

Abstract

L ymphangioleiomyomatosis (LAM) is a rare, progressive, frequently fatal cystic lung disease that affects women almost exclusively. 1,2 LAM occurs in up to 40% of women with tuberous sclerosis complex (TSC-LAM), 3-5 a tumor suppressor syndrome associated with seizures, cognitive impairment, and hamartomas in multiple organs, and in a nonheritable sporadic form (S-LAM) that involves only the lung,

Chest Computed Tomographic Image Screening for Cystic Lung Diseases in Patients with Spontaneous Pneumothorax Is Cost Effective

“…According to the European Respiratory Society guidelines, the diagnosis of LAM can be established with certainty on the basis of a compatible chest CT, along with the presence of chylous effusions, angiomyolipomas or lymphangiomyomas, or a personal/family history of tuberous sclerosis (22). Serum VEGF-D is a useful diagnostic biomarker, and if elevated to greater than 800 pg/ml, specificity for the diagnosis of LAM approaches 100% (23,24). Tissue confirmation may be needed if the diagnosis of LAM is not fully established based on above steps.…”

Effect of Fasting on the Size of Lymphangioleiomyomas in Patients With Lymphangioleiomyomatosis

“…20 Th e disease phenotype is associated with lung nodules infi ltrated with lymphatics, lymphatic vessels showing LAM cells penetrating through the walls, and chylous eff usions. [19][20][21][22][23][24][25] Th ese forms of LAM are characterized by the presence of vascular abnormalities and evidence of arteriolar and venular channels as well as lymphatic channel infi ltration by LAM cells, resulting in both vascular obstruction and vascular wall disruption. 6,18 In the lungs, the consequences of these abnormalities are twofold.…”

“…7,26,27 In one study, CT scans taken in the evening hours showed a 140% increase in volume from morning values. 24 Th is fi nding was also documented by ultrasonography. 27 In the latter study, 21 of 44 patients with LAM showed morning to evening variation, with an increase in the size of the lymphangioleiomyomas ranging from 10% to 484%.…”

“…22,23 Measurement of serum VEGF-D has been shown to be useful as a diagnostic tool and in grading the severity of the disease and the potential response to therapy. 24,25 Lymphangioleiomyomas have a distinctive radiologic appearance, and diurnal variation in size of these tumors has been reported. 7,26,27 In one study, CT scans taken in the evening hours showed a 140% increase in volume from morning values.…”

Linfangioleiomiomatosis esporádica. Presentación de un caso con enfermedad pulmonar y linfangioleiomiomas retroperitoneales

“…El punto de corte de 0,8 ng/ml tiene una sensibilidad del 73% y una especificidad del 100% (12). Se estima que el 90% de las LAM probables por criterios ERS serían definitivas si se adicionara VEGF-D a los criterios diagnósticos (12). Estudios recientes han evaluado la utilidad del VEGF-D como factor pronóstico y para seguimiento de pacientes con LAM en tratamiento.…”