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目的 Lymphangioleiomyomatosis (LAM) is a rare multisystem disease occurring almost exclusively in premenopausal women. Serum vascular endothelial growth factor-D (VEGF-D) has been recently considered as a novel marker for LAM.Herein we reported the diagnostic and differential diagnostic value of serum VEGF-D in LAM patients and evaluated the change of serum VEGF-D on pre- and post-treatment with sirolimus. 方法 The study group included 66 patients with LAM (47 definite LAM and 19 probable LAM), 14 patients with other polycystic lung diseases and 20 with healthy female controls. 结果 Serum VEGF-D levels were significantly increased in definite LAM patients compared with health controls ((3890.3±373.3) pg/mL vs.(413.3±33.2) pg/mL, P＜0.05).The optimal cutoff point for LAM diagnosis was 692.5pg/mL with the sensitivity of 97.9％ and specificity of 100％ respectively.Serum VEGF-D levels were significantly increased in definite LAM patients who had chylothorax compared with those without ((5153.9±598.3) pg/mL vs.(2869.8±372.8) pg/mL, P＜0.05).Serum VEGF-D levels in definite LAM patients and patients with other cystic lung diseases were (3890.3±373.3) pg/mL and (412.6±27.5) pg/mL, respectively (P ＜0.05).Serum VEGF-D levels decreased after the treatment with sirolimus from (3135.0±909.4) pg/mL to (1731.8±621.2) pg/mL with a medium of 12 months treatments. 结论 Serum VEGF-D levels were significantly higher in LAM patients compared with healthy controls and patients with other polycystic lung diseases.They were further increased when complicated with chylothorax.Serum VEGF-D could be used for diagnosis and differential diagnosis and might be useful for the following up of treatment with sirolimus.