The role of ophthalmic imaging in central nervous system degeneration in systemic lupus erythematosus

Abstract

Systemic lupus erythematosus (SLE) is an autoimmune connective tissue disorder that can involve any organ system. Central nervous system involvement can be a severe life threatening complication, ultimately resulting in severe neurodegenerative changes. Magnetic resonance imaging suggests that neurodegeneration, which may have deleterious effects on brain function, may occur early in SLE and experimental models suggest that neuroprotection may be feasible and beneficial. The retina is an extension of the brain. Recent ophthalmic imaging technologies are capable of identifying early changes in retinal and choroidal morphology and circulation that may reflect CNS degeneration. However, their utility in monitoring CNS involvement in SLE has been poorly studied as these have only been performed in small cohorts, in a cross-sectional design, non-quantitatively and without correlation to disease activity. The authors aim to review the current understanding of neurodegeneration associated with SLE, with particular focus on the visual pathway. We describe the neuropathology of the visual system in SLE and the evidence for retinal and choroidal neurodegenerative and microvascular changes using optical coherence tomography technology. We aim to describe the potential role of optical imaging modalities in NPSLE diagnosis and their likely impact on the study of neuronal function.

Ocular involvement in systemic lupus erythematosus patients: a paradigm shift based on the experience of a tertiary referral center

“…Lupus retinopathy is a potentially sight-threatening complication that may manifest as retinal microangiopathy, retinal vasculitis or severe arterial or venous vaso-occlusion. 2 Lupus choroidopathy is another sight-threatening manifestation that can occur in isolation or associated with lupus retinopathy. It may present with single or multiple areas of serous retinal detachment, retinal pigment epitheliopathy, retinal pigment epithelium (RPE) detachment, choroidal ischemia or effusion.…”

“…1 Posterior segment involvement, namely lupus retinopathy, choroidopathy and optic neuropathy is particularly concerning given their potential for a devastating impact on visual prognosis and their relation with poor systemic disease control. 2 According to most reports, lupus retinopathy is the second most common ocular manifestation with an incidence that may reach 29%. 3 However, over the last few years we have been entering a new era in the treatment of SLE.…”

Association of Lipoprotein and Thyroid Hormones With Cognitive Dysfunction in Patients With Systemic Lupus Erythematosus

“…NPSLE is a severe complication of SLE, which results in severe neurodegenerative changes and threatens life [58]. NPSLE is characterized by a variety of neurological manifestations, making the diagnosis of NPSLE a formidable challenge for rheumatologists [59][60].…”

<p>Choroidal thickness changes in systemic lupus erythematosus patients</p>

“…Histopathology studies of the choroid in SLE patients have demonstrated mononuclear inflammatory cells within the choroid, reflecting choroidal vasculitis as well as immunoglobulin and complement deposition in the choroidal vasculature. 6,36 Consequently, choroidal blood supply is compromised, leading to choroidal thinning. Chronic ischemia induces long-term atrophy of choroidal stromal, which also leads to choroidal thinning.…”

“…5 In the eye, SLE can affect almost any structure, including the vascular supply of the retina, the choroid and the optic nerve head. 6 Lupus choroidopathy is rare and can present with serous retinal detachment, retinal pigment epithelium detachment, retinal pigment epitheliopathy, choroidal ischemia or effusion. 5 When present, lupus choroidopathy is usually a marker of high disease activity and often associated with the central nervous system and renal disease.…”