Our data confirm the finding that VEGF-D is elevated in LAM [18,26] and in our cohort, has a diagnostic specificity of 100% but a sensitivity of only 56%: suggesting that an elevated VEGF-D is helpful in confirming the diagnosis of LAM, but a normal VEGF-D level is not sufficient to exclude it. This evidence concerns the gene VEGFD and lymphangioleiomyomatosis.