Optical coherence tomography angiography to assess for retinal vascular changes in Neuro-Sjögren

Abstract

Background: Sjögren's syndrome is an autoimmune disease characterized by sicca symptoms and various extraglandular manifestations including vasculitis. Neurological involvement occurs frequently (Neuro-Sjögren) and often mimics immune neuropathies such as chronic inflammatory demyelinating polyneuropathy (CIDP).

Objectives: We aim to assess relevant differences in vessel density (VD) in Optical Coherence Tomography Angiography (OCTA) in those diseases to use it as an easily available diagnostic tool.

Design: Prospective, monocentric pilot-study.

Methods: OCTA (Heidelberg Engineering OCT SPECTRALIS) of the superficial vascular plexus, intermediate capillary plexus (ICP) and deep capillary plexus (DCP) of the retina was prospectively performed in Neuro-Sjögren, age-matched CIDP patients (n = 31, each), and healthy controls (n = 30). Vessel density (VD) and foveal avascular zone (FAZ) was measured with Erlangen Angio Tool.

Results: Significantly lower VD were found for the DCP and ICP in Neuro-Sjögren and CIDP patients compared to healthy controls (p = 0.0002 and <0.0001). When group comparison was age-adjusted, these differences were not found anymore. Different frequencies of "low" retinal blood flow in each layer comparing Neuro-Sjögren and CIDP patients were not found. FAZ revealed no significant differences between patients with Neuro-Sjögren, CIDP and healthy controls.

Conclusion: This study found no significant differences in VD or the foveal avascular zone between Neuro-Sjögren and CIDP patients using OCTA, suggesting that inflammatory vascular changes in the retina are uncommon in Neuro-Sjögren patients.

Keywords: Sjögren’s syndrome; chronic inflammatory demyelinating polyneuropathy; neuro-Sjögren; optical coherence tomography angiography; retinal vasculature; vasculitis.

Figure 1.

Exemplary OCTA analysis of the right eye in one patient with Neuro-Sjögren. (a)–(c) SVP analysis is shown, (d)–(f) the ICP and (g)–(i) the DCP. DCP, deep capillary plexus; ICP, intermediate capillary plexus; SVP, superficial vascular plexus.

Figure 2.

Exemplary measurement of the FAZ of the right eye in one patient with Neuro-Sjögren. FAZ, foveal avascular zone.

Figure 3.

Comparison of VD percentages of both eyes of different retinal layers in patients with Neuro-Sjögren, CIDP and controls. The pooled VD percentages of different retinal layers (a) DCP, (b) ICP, (c) SVP are depicted. The comparison of different VD percentages shows significant differences in DCP and ICP layer of Neuro-Sjögren and CIDP patients compared with healthy controls. Exclusion of older patients (>70 years of age) from the Neuro-Sjögren and CIDP group resulted in nonstatistically significant differences. The level of statistical significance is shown above the line. CIDP, chronical inflammatory demyelinating polyneuropathy; DCP, deep capillary plexus; ICP, intermediate capillary plexus; SVP, superficial vascular plexus; VD, vessel density.