Case Report Submission for Residents Day (Academy 2012)
Christopher Lee, O.D.
Ocular Disease Residency
New York Harbor Healthcare System (Brooklyn/St. Albans VAMC); SUNY Optometry
American Academy of Optometry: Case Report
Clinical Management of Retinopathy Secondary to Systemic Lupus Erythematosus
Christopher Lee, O.D., Annie Wan, O.D., Prashant Ankola, O.D., Evan Canellos, O.D., F.A.A.O,
Maria Bosco, O.D., F.A.A.O
Abstract
Systemic lupus erythematosus (SLE) is an autoimmune condition that may have ocular
manifestations. This case illustrates a patient presenting with a rare retinal vasculitis that
would not be expected based her recent laboratory studies.
Case History
A pleasant 24 year old Asian female with a history of SLE currently on Plaquenil therapy
presents to the eye clinic for a 6 month follow-up exam. Patient reports no new ocular or
visual complaints.
Patient is currently taking Plaquenil 400 mg/daily (started August 2011). Last eye exam
including dilation was 6 months ago with unremarkable results—no
retinopathy/maculopathy, good acuity, normal color vision, and normal red Amsler testing.
Patient was diagnosed with SLE on February 2011 with multiple organ involvement
including the skin, heart, lungs, blood, and kidneys. As a result the patient has suffered
from many complications including dermatologic (malar rash and photosensitivity),
cardiac (pericarditis/cardiomyopathy), pulmonary (pleurisy and Nocardia infection),
hematologic (hemolytic anemia), and renal (class IV Lupus nephritis with stage 3 chronic
kidney disease) problems that all appeared to be under better control when the patient
presented to the eye clinic for follow up.
Patient’s current medications are listed below:
- Plaquenil 400 mg/daily
- Predisone 20 mg/daily
- Mycophenolate (Cellcept) 500 mg BID
- Calcium 500 mg/Vitamin D 200 unit Tab
- Lisinopril 20 mg
- Darbepoetin Alfa 40MCG/0.4ML/Syringe
- Rituximab
- Simvastatin 20 mg
- Bactrim DS
- Omeprazole 20 mg
Case Report Submission for Residents Day (Academy 2012)
Christopher Lee, O.D.
Ocular Disease Residency
New York Harbor Healthcare System (Brooklyn/St. Albans VAMC); SUNY Optometry
Pertinent Findings
- Physical
o Patient presented with malar rash (erythematosus skin lesion extending
from cheek to cheek crossing the nose)
- Clinical
o Most recent laboratory studies
 C3/C4 levels: 57/12.5 (75-181/16-47; reference range)
 BUN: 15 mg/dl (2.3 – 6.6)
 Creatinine: 1.6 mg/dl (0.4 to 1.2)
 ESR 10 mm/hr
 Anti double stranded DNA negative
o Entering distance acuity
 Spectacle prescription:
o
o
o
o
OD -3.00
20/40+
OS -3.00
20/40+
Refraction and best corrected acuity
 OD: -4.00-0.50x075 20/20
 OS: -3.50
20/20
 Near VA
20/20
External/Auxiliary testing
 Confrontation visual fields full to finger counting OD/OS
 Full and smooth extra ocular motility OU
 11/11 OD/OS Ishihara color vision
 full/(-) metamorphopia OD/OS with red Amsler grid
 Pupils: PERRL (-) APD
Biomicroscopy
 Lids/lashes: clear OU
 Conjunctiva: clear OU
 Cornea:
decreased TBUT OU
 AC:
deep and quiet OU
 Iris:
flat/intact OU
 Lens:
gr 1 PSC (diffuse) OU
Intraocular pressures
 19/19 GAT @ 1:30 PM
Case Report Submission for Residents Day (Academy 2012)
Christopher Lee, O.D.
Ocular Disease Residency
New York Harbor Healthcare System (Brooklyn/St. Albans VAMC); SUNY Optometry
o Dilated fundus exam (pictures/description)
Retinal Photographs—posterior pole




Description
Vitreous:
clear OU
Optic Nerve: 0.50 H/V OD, 0.40 H/V OS, pink rims OU
Macula:
clear/flat/(+)FR OU
Periphery: attenuated, sclerosed vessels, arterial sheathing,
scattered hemorrhages OU
Case Report Submission for Residents Day (Academy 2012)
Christopher Lee, O.D.
Ocular Disease Residency
New York Harbor Healthcare System (Brooklyn/St. Albans VAMC); SUNY Optometry
Differential Diagnoses
- Lupus retinopathy
- Hypertensive retinopathy
- Diabetic retinopathy
- Central and branch retinal vein occlusion
- Central and branch retinal arterial occlusion
- Bechet’s Disease
- Wegener’s Granulomatosis
- Sarcoidosis
- Syphilis
- Lyme Disease
- HIV retinopathy
- Cytomegalovirus retinitis
Diagnosis/Treatment/Management
Upon examination of the patient and given all other associated factors, the leading
diagnosis was bilateral peripheral perivascular inflammation with scattered hemorrhages
secondary to SLE. The patient was referred to the specialty retina clinic for a consult and
fluorescein angiography.
At the retina consult, the diagnosis was confirmed. Fluorescein angiography revealed only
areas of perivascular staining consistent with vasculitis, but no areas of capillary nonperfusion and no neovascularization.
Patient was educated about her most recent ocular manifestations and the association to
SLE being uncontrolled and possible risk of blindness should progression occur. Patient’s
rheumatologist was contacted and reprised of these findings and the need for better
control of her SLE.
The patient is currently scheduled for follow up with her rheumatologist and is scheduled
to return to the eye clinic for visual field testing. In the interim, the patient was instructed
to monitor vision with home Amsler grid and to return to the clinic should any changes in
vision occur.
Discussion
SLE is a chronic autoimmune condition that affects multiple organs including the eye. It is
characterized by a relapsing and remitting clinical course. There is currently no cure and
therapy is targeted towards control of symptoms. Ocular manifestations occur in 30% of
patients, with 5% attributed to retinal vasculitis and the other 25% attributed to dry eye.
Although not as common as dry eye, the presence of retinal vasculitis secondary to SLE can
be very significant. Its occurrence has been shown to be an accurate indicator of systemic
Case Report Submission for Residents Day (Academy 2012)
Christopher Lee, O.D.
Ocular Disease Residency
New York Harbor Healthcare System (Brooklyn/St. Albans VAMC); SUNY Optometry
disease activity. Also, it has been noted that individuals with active retinopathy tend to
have poorer survival rates compared to those who do not.
Retinal manifestations of SLE include cotton wool spots, perivascular hard exudates, retinal
hemorrhages, vascular tortuosity, arteriolar constriction, and in severe cases occlusion of
the retinal arterioles and retinal ischemia.
Fortunately, the patient’s retinopathy was limited in the periphery and vision was not
affected. Furthermore, fluorescein angiography revealed no indication for more aggressive
management that would include pan retinal photocoagulation, intravitreal antivascular
endothelial growth factor agents, or vitrectomy.
Conclusions/Clinical Pearls
Although not as common as dry eye, retinal vasculitis secondary to SLE may occur and can
be a sight threatening complication. Furthermore, it can be an indicator of systemic disease
activity. Early detection can lead to prompt referral to an individual’s rheumatologist for
better control. This case illustrates the unique vantage point eye care providers have in
caring for these patients—simultaneously monitoring retinal health and systemic disease
activity. Furthermore, it points out the importance of the communication between eye care
providers and other medical specialists.
Case Report Submission for Residents Day (Academy 2012)
Christopher Lee, O.D.
Ocular Disease Residency
New York Harbor Healthcare System (Brooklyn/St. Albans VAMC); SUNY Optometry
Works Cited
Palejwala, Neal V., Harpreet S. Walia, and Steven Yeh. "Ocular Manifestations of Systemic
Lupus Erythematosus: A Review of the Literature." Autoimmune Diseases 2012 (2012): n.
pag. Web.
Read, Russell W. "Clinical Mini-review: Systemic Lupus Erythematosus and the Eye." Ocular
Immunology and Inflammation 12.2 (2004): 87-99. Web.
Sivaraj, R. R., O. M. Durrani, A. K. Denniston, P. I. Murray, and C. Gordon. "Ocular
Manifestations of Systemic Lupus Erythematosus." Rheumatology 46.12 (2007): 1757-762.
Print
Thomann, Kelly H., Esther S. Marks, and Diane T. Adamczyk. Primary
Eyecare in Systemic Disease. New York, NY: McGraw-Hill, Medical Pub. Div., 2001. Print.