Ocular Involvement in Systemic Sclerosis: Updated Review and New Insights on Microvascular Impairment

Figures & data

Table 1. Prevalence of ocular findings among SSc patients in different researches.

Ocular findings	Percent (%)
Eyelid (stiffness, tightness, telangiectasia)	51.1–77.0
Conjunctiva (neovascularization, teleangiectasia, varicose vein dilatation)	15.7–63.0
Dry eye disease	48.9–64.7
Iris (transilumination, vascular deformation)	8.9–13.7
Cataract	42.2–51.0
Retina (hard exudates, tortuosity of vessels)	10.0–34.0
Choroid (perfusion abnormalities)	33.0–55.0
Glaucoma	11.1–21.6

Table 2. Review of recent reports regarding SSc microvascular changes using OCTA.

Study authors	OCTA devices	Scan width (mm^2)	No. of eyes SSc/controls	Findings in FD patients
Ranjbar et al.^Citation75	Spectral Domain	3 × 3	12/12	↓VD in CC and Sattler’s Layer
Hekimsoy et al.^Citation76	Spectral Domain	6 × 6	45/45	↓VD in SCP in fovea, parafovea, perifovea, whole image ↓VD DCP only in fovea = FAZ parameters and VD in CC
Rommel et al.^Citation77	Spectral Domain	3 × 3	30/30	↓VD in SCP and DCP =VD in CC ↓VD in Sattler’s and Haller’s Layers
Rothe et al.^Citation78	Spectral Domain	3 × 3	24/24	↓VD in SCP, =VD in DCP and FAZ parameters
Carnevali et al.^Citation79	Spectral Domain	3 × 3	20/20	↓VD in DCP =VD in SCP
Fang et al.^Citation80	Spectral Domain	3 × 3	32/32	↓VD in SCP in all regions of ETDRS map
Mihailovic et al.^Citation81	Spectral Domain	Macula: 3 × 3 Optic nerve: 4.5 × 4.5	22 (7 VEDOSS)/22	↓VD in SCP and CC ↓VD in radial peripapillary capillary network =VD in DCP and FAZ parameters ↓VD in CC also in VEDOSS
Ertuk et al. (2023)	Spectral Domain	Macula: 3 × 3 Optic nerve: 4.5 × 4.5	22 PRP 19 VEDOSS 38 SSc (25 lcSSc, 13 dcSSc	↓VD in SCP and DCP in SSc vs. PRP ↓VD in foveal SCP in lcSSc vs. VEDOSS ↓VD in RNFL peripapillary optic disc in lcSSC vs. VEDOSS and PRP =VD in CC among all groups

OCTA = Optical Coherence Tomography Angiography, SSc = systemic sclerosis; lcSSc = limited cutaneous SSc; VEDOSS = Very Early Disease Of Systemic Sclerosis; VD = vessel density; CC = choriocapillaris; DCP = Deep Capillary Plexus; SCP = Superficial Capillary Plexus, FAZ = Foveal Avascular Zone; ETDRS = Early Treatment Diabetic Retinopathy Study; PRP = Primary Raynaud Phenomenon; RNFL = Retinal Nerve Fiber Layer.

Ranjbar M, Rothe M, Klapa S, et al. Evaluation of choroidal substructure perfusion in patients affected by systemic sclerosis: an optical coherence tomography angiography study. Scand J Rheumatol. 2020;49(2):141–145. doi:10.1080/03009742.2019.1641616.

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