Cennamo Gilda, Montorio Daniela, Santoro Ciro, Cocozza Sirio, Spinelli Letizia, Di Risi Teodolinda, Riccio Eleonora, Russo Camilla, Pontillo Giuseppe, Esposito Roberta, Imbriaco Massimo, Pisani Antonio
Eye Clinic, Public Health Department, University of Naples "Federico II", 80131 Naples, Italy.
Department of Neurosciences, Reproductive Sciences and Dentistry, University of Naples "Federico II", 80131 Naples, Italy.
J Clin Med. 2020 Dec 18;9(12):4087. doi: 10.3390/jcm9124087.
In this study, we evaluated the possible relationship between the changes in retinal vessel density (VD) by optical coherence tomography angiography (OCTA) and the vascular alterations involving renal, cardiovascular and central nervous systems in patients affected by Fabry disease (FD). In 50 FD patients, the retinal superficial capillary plexus (SCP) and deep capillary plexus (DCP) in macular region were evaluated by OCTA examination. The patients also underwent a brain magnetic resonance imaging scan, renal and echocardiographic examinations with quantification of systolic pulmonary arterial pressure (PAPs) and left atrial volume index (LAVi). The VD of SCP and DCP was inversely related with E/e' ratio, LAVi, interventricular septal thickness, global longitudinal strain (GLS) and PAPs ( < 0.05). No relationship was found, with a multivariate analysis, between OCTA parameters and kidney function and neuroradiological signs of central nervous system involvement. OCTA could be a new vascular biomarker in FD, revealing a strong correlation between retinal capillary damage and myocardial impairment, possibly preceding both renal dysfunction and cerebrovascular involvement.
在本研究中，我们评估了通过光学相干断层扫描血管造影（OCTA）检测到的视网膜血管密度（VD）变化与法布里病（FD）患者肾脏、心血管和中枢神经系统血管改变之间的可能关系。对50例FD患者，通过OCTA检查评估黄斑区视网膜浅表毛细血管丛（SCP）和深部毛细血管丛（DCP）。患者还接受了脑磁共振成像扫描、肾脏和超声心动图检查，并对收缩期肺动脉压（PAPs）和左心房容积指数（LAVi）进行了量化。SCP和DCP的VD与E/e'比值、LAVi、室间隔厚度、整体纵向应变（GLS）和PAPs呈负相关（P<0.05）。多因素分析未发现OCTA参数与肾功能以及中枢神经系统受累的神经放射学征象之间存在关联。OCTA可能是FD中的一种新型血管生物标志物，揭示了视网膜毛细血管损伤与心肌损伤之间的强相关性，这可能先于肾功能不全和脑血管受累出现。
