Evaluation of the direct effects on retinal and choroidal microvascularity of systemic scleroderma

Ocular involvement in systemic sclerosis (SSc) has been documented; however it cannot be distinguished from secondary changes due to concomitant hypertension.Therefore, the aim of this prospective cross-sectional study was to demonstrate the direct effects of Ssc on retinal and choroidal microvasculature in patients without hypertension.

47 SSc patients and 44 age- and sex-matched healthy control subjects were enrolled in this study. In fundus examination: Increased vascular tortitis, focal or general arteriolar narrowing, arteriovenous notch, severe exudation, microhemorrhage, and pigment epithelial changes in the retina of SSc patients without hypertension were investigated. Patients with at least two of the above findings were considered to have retinopathy After that, patients were divided into two groups according to the presence of retinopathy in this study. Retinal and choroidal microvasculature were evaluated using optical coherence tomography angiography.

There was a significant decrease in SSc patients with retinopathy in both superficial capillary plexus vessel density (SCP VD) and deep capillary plexus vessel density (DCP VD) compared to the control group. Full avascular zone (FAZ) evaluation tool variables (FAZ area, FAZ perimeter, foveal density) were significantly lower in all Ssc patients than in the healthy control group. It was found that the flow in the 1 mm and 3 mm circular area (Outer Retina 1-3 mm Flow Area) increased significantly in Ssc patients with retinopathy. Choroidal flow (Choriocapillaries 1 mm Flow Area) was statistically lower in Ssc patients with retinopathy.

We have showed an increase in the outer retina 1-3 mm flow area (circular area of the outer retina fold covering the fovea) despite the decrease in vascular density and choroidal thickness in scleroderma patients with retinopathy. Hence, we first demonstrated that Ssc itself may have an effect on retinal and choroidal microvasculature, independent of hypertension.