OCULAR MANIFESTATIONS AND TREATMENT OUTCOMES IN CHINESE PATIENTS WITH POEMS SYNDROME.

Abstract

To evaluate the relationship of serum vascular endothelial growth factor (VEGF) levels and ocular manifestations in Chinese patients with POEMS syndrome. This is a prospective study. Forty-one treatment-naive patients were enrolled from April 2014 to November 2014. Among the 41 patients, 40 had complete ocular examination, spectral domain optical coherence tomography scan, and serum VEGF measurement before treatment and every 3-month interval after lenalidomide and dexamethasone treatment. Twenty-seven (67.5%) patients had optic disk edema (ODE) at baseline. Retinal manifestations included retinal hemorrhage, subretinal fluid, macular edema, and cotton wool spot. The difference in mean serum VEGF concentrations between patients with and without ODE was significant (P = 0.017). Among patients with ODE, there was a significant positive correlation between mean serum VEGF levels and the binocular mean retinal nerve fiber layer thickness (P = 0.008), as well as mean peripapillary retinal thickness (P = 0.020) before treatment. After 3 months to 17 months treatment, mean serum VEGF concentrations decreased significantly (P < 0.001). Mean retinal nerve fiber layer thickness and mean peripapillary retinal thickness decreased significantly (P < 0.001). The remission rate of ODE was 87.5%, and complete remission rate was 58.3%. The ODE is a common manifestation in POEMS syndrome, and raised VEGF might explain the development and mechanism. Systemic treatment could lead to decrease in serum VEGF levels accompanied by regression of ODE.