Vascular Changes of the Retina and Choroid in Systemic Lupus Erythematosus: Pathology and Pathogenesis

Abstract

Systemic lupus erythematosus (SLE) is a chronic autoimmune disorder that affects multiple organ systems. When eyes are involved, the principle manifestations are hemorrhage, retinal cotton wool spots, microangiopathy and vaso-occlusion. Research in the past two decades has significantly contributed to our understanding about this disease in general and its therapeutic management, although knowledge about the mechanism of ocular involvement and pathogenesis in SLE is limited. This is an important issue, because the ocular symptoms in this disease could be potentially sight threatening in acute cases. Here, we present an overview of the clinical and histopathologic features of retinal and choroidal vascular changes, as seen in patients with SLE. We discuss the role of immune complex deposition in vascular pathogenesis in the eye. Reports indicated an involvement of antiphospholipid antibodies (APAs) in the retinal and choroidal vasculopathy in SLE, although their precise role in this process is uncertain. It is important to look for mechanisms of immune complex-mediated vasculopathy and role of inflammatory mediators in this process in SLE. For this, established animal models can be utilized in research to learn about the precise role of various autoantibodies and complements involved in disease pathogenesis. A clear knowledge about the immunopathogenesis is warranted, and the rationale for the future therapy should be based on reducing vascular inflammation as well as ameliorating autoimmunity in this disease.

RETRACTED: A novel pseudovirus encoding Diphtheriatoxin A fragment eliminates autoantibody-producing B cells and inhibits lupus in the BWF1 mouse model

“…(B) Survival was also monitored from six to ten months of age. trolled formation of autoantibodies, leading to the excess formation of immune complexes that deposit in different tissues, causing inflammation and tissue damage [14,15].…”

Autoimmune Retinopathy in Systemic Lupus Erythematosus: Histopathologic Features

“…In a study of 550 patients with SLE, Stafford-Brady and colleagues observed retinopathy in 41 patients (7.1%) [6], while a study of children with SLE reported ocular involvement in 35% of patients [7]. In the case of our patient, histopathologic examination of the peripheral retina revealed many small occluded vessels, some appearing similar to changes seen in chronic retinal vasculitis [8]. The vascular occlusive findings and cobblestone degeneration are likely the consequence of SLE vasculopathy and lupus anticoagulant thrombosis.…”

Choroidal Thickness in Patients with Systemic Lupus Erythematosus Analyzed by Spectral-domain Optical Coherence Tomography

“…Additionally, choroidal vasculature may be damaged by fibrin deposition, which often occurs in the vessel wall and lumen. 8,9 The choroid has been shown to be affected in patient of SLE in several experimental and clinical studies. [10][11][12][13][14][15][16] Enhanced depth imaging optical coherence tomography (EDI-OCT), which is a recent modification of the standard OCT technique, offers a noninvasive, rapid, objective, and reliable diagnostic modality for the imaging of choroidal alterations and allows in vivo examination and quantification of the choroid.…”