J Korean Ophthalmol Soc.  2013 Mar;54(3):518-523. 10.3341/jkos.2013.54.3.518.

A Case of Severe Vaso-Occlusive Retinopathy as the First Manifestation Associated with Systemic Lupus Erythematosus

• Kim SB
• Cho WH
• Chang MH

• KMID: 2216776
• DOI: http://doi.org/10.3341/jkos.2013.54.3.518

Abstract

PURPOSE
To report a case of severe vaso-occlusive retinopathy with significant decrease of bilateral visual acuity as the first manifestation associated with systemic lupus erythematosus (SLE).
CASE SUMMARY
A 23-year-old man was referred to our clinic with bilateral visual impairment of hand motion (HH). Fundus examination revealed severe retinal hemorrhage, cotton-wool patch, occlusive retinal vasculitis with vascular engorgement, and diffuse retinal edema in both eyes. Because of a malar rash on both cheeks, generalized edema was observed on initial examination with hypertension, azotemia, anemia, and thrombocytopenia, The patient was diagnosed with SLE, strongly positive to antinuclear antibody (ANA), and received an intravitreal injection of Bevacizumab (Avastin, Genentech Inc., San Francisco, CA, USA) in the left eye in addition to hemodialysis, transfusion, systemic corticosteroid and immunosuppressant treatment due to lupus nephritis. Eighteen months later, the retinal edema, cotton-wool patch and hemorrhage resolved, leaving epiretinal membrane without traction in his left eye and diffuse degeneration of the right eye. Final visual acuity was HM in the right eye and 20/100 in the left eye.
CONCLUSIONS
Vaso-occlusive retinopathy in SLE can result in permanent visual impairment. In a patient with a high possibility of SLE retinopathy, a periodic fundus examination and intensive management of systemic disease should be considered.

Keyword

Antiphopholipid antibody; Lupus retinopathy; Occlusive retinal vasculitis; Systemic lupus erythematosus; Vaso-occlusive Retinopathy

MeSH Terms

Anemia
Antibodies, Antinuclear
Antibodies, Monoclonal, Humanized
Azotemia
Cheek
Edema
Epiretinal Membrane
Exanthema
Eye
Hand
Hemorrhage
Humans
Hypertension
Intravitreal Injections
Lupus Erythematosus, Systemic
Lupus Nephritis
Papilledema
Renal Dialysis
Retinal Hemorrhage
Retinal Vasculitis
San Francisco
Thrombocytopenia
Traction
Vision Disorders
Visual Acuity
Bevacizumab
Antibodies, Antinuclear
Antibodies, Monoclonal, Humanized

Figure

• 

  Figure 1. Fundus (upper) and optical coherence tomographic (lower) findings of the right (A and C) and left (B and D) eyes at presentation. (A, B) Fundus findings revealed vessel engorgement, multiple flame-shaped retinal hemorrhages, cotton wool patchs, diffuse retinal edema and optic disc swelling. (C, D) Spectal-domein optical coherence tomographic findings showed severe macular cystoids edema (both eyes) and serous retinal detachment (left eye).

• 

  Figure 2. Initial findings of both cheeks. M alar rashes of both cheeks are observed.

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  Figure 3. Fluorescein angiographic findings showed neovascularization at the optic dsc, total defect of choroidal filling, occlusion of the inferior retinal artery, diffuse leakage around the vessel of the right eye (A and C), and leakage around the optic disc head, diffuse peripheral non-perfusion area, multiple occlusions of peripheral retinal vessels (B and D).

• 

  Figure 4. Final ocular findings of the right (A and C) and left (B, D and E) eyes. (A, C) Fundus photographs show diffuse degenerative changes, pale optic disc, narrowing of arteries, and OCT findings revealed diffuse retinal thinning and uncertain foveal contour. (B, D, E) fundus photograph show focal degenerative change and parafoveal tractional fibrotic membrane, and OCT findings reveals relatively clear foveal contour and parafoveal, focal retinal detachment due to tractional membrane.

Reference

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