Different Location and Extent Of Impaired Perfusion Of The Choroid Plexus In Primary and Secondary Raynaud’s Phenomenon

Background/Purpose: Raynaud’s phenomenon (RP) is a disorder characterized by systemic vascular dysregulation which has been related with vasospasms and retinal blood flow abnormalities. Nevertheless among the potential complications of RP, eye involvement is often overlooked. The aim of this study was to evaluate the presence of vascular eye involvement measuring the choroidal and macular thickness in a cross-sectional cohort of RP at the first rheumatologic evaluation. Methods: Thirty adult consecutive RP, without visual symptoms, underwent: clinical evaluation and nailfold capillaroscopy. Sera were tested for anti-nuclear antibodies, anti-dsDNA, anti-extractable nuclear antigens. Patients underwent a complete ocular examination including: best corrected visual acuity; slit lamp biomicroscopy; intraocular pressure measurements and fundus examination; choroidal thickness by Zeiss Cirrus Spectral Domain Optical Coherence Tomography with enhanced depth imaging scan system at the fovea and up to 2 mm at intervals of 1 mm from the fovea in the superior, inferior, nasal and temporal choroid; central foveal thickness (CFT) was also measured. 27 healthy, sex and age-matched, subjects were analysed as control group. Statistical analysis was performed by one-way ANOVA and multiple comparisons. Results: Eight primary RP (pRP) (median age 53 yrs), 12 early systemic sclerosis (SSc) (median age 57 yrs), 10 RP secondary to suspected connective tissue disease (CTD) (i.e. capillaroscopy abnormalities or antibody positivity and without any symptoms/signs suggesting a CTD) (median age 54 yrs). Slitlamp biomicrocopy was within normal limits in all patients and fundus examination revealed normal arterial and venous vessels with no capillary abnormalities. The mean best corrected visual acuity was 20/40 and the mean intraocular pressure measurement was 14 mmHg. In pRP, mean choroidal thickness was significantly thinner than healthy controls in the outer nasal (224.8m vs 289m; p0.05) and outer temporal (263.3m vs 299m; p0.05) regions. In patients with RP secondary to suspected CTD the inner and outer nasal were significantly thinner (244.1m vs 297.4m; p0.05 and 199.7m vs 289m; p0.0001 respectively). In SSc all the areas examined were significantly thinner than healthy controls except for the inner temporal region which however was decreased: central (250.5m vs 313.2m; p0.01), inner inferior (239.9m vs 293.1m; p0.05), inner nasal (221m vs 297.4m; p0.001), inner superior (243.9m vs 293.2m; p0.05), outer temporal (219.3m vs 299m; p0.001), outer inferior (229.9m vs 289.8 m; p0.001), outer nasal (187m vs 289m; p0.001), outer superior (240.4m vs 293.3m; p0.05). CFT was also thinner in all the patients than healthy controls although not significant. Conclusion: A thinner choroidal and macular thickness was observed in all the patients, with progressive decrease from pRP to SSc. These data suggest an early involvement of ocular microcirculation with significant reduction of choroidal perfusion. This process likely starts from the external regions (outer nasal and temporal) in pRP, and becomes more severe and extensive in RP secondary to a suspected CTD and even more severe and in SSc.