Five-day history of decreased vision in a patient with systemic lupus erythematosus
Fundus exam of the right eye found a distinct intra-arteriolar plaque and a large flame-shaped hemorrhage overlying the proximal inferior arcade.
A 48-year-old white woman was referred to the ophthalmology clinic with a 5-day history of slightly decreased vision in her right eye. This was associated with a fixed “black line” running through the center of her vision.
The patient’s medical history included systemic lupus erythematosus (SLE) with lupus nephritis and resultant end stage renal disease. She had an ocular history of myopia. She did not take any medications due to noncompliance because most medications caused mild nausea.
She noted that her malar rash had returned in the past week but denied other signs or symptoms of a lupus flare, such as arthritis, fatigue or headache.
On examination, best corrected visual acuity was 20/40– in the right eye and 20/20 in the left, with manifest refraction of –1.25 D in both eyes. Applanation tensions were 15 mm Hg in the right eye and 14 mm Hg in the left eye. The patient had no relative afferent pupillary defect in either eye. Extraocular movements were full. On slit-lamp examination, lids were normal, conjunctivae were white, and corneas were clear. There were no cells present in the anterior chambers or vitreous. She had trace nuclear sclerotic cataracts.
Funduscopic examination of the right eye (Figure 1) showed a normal optic nerve with a sharp disc margin and vessels of normal caliber. At the disc, there was a distinct intra-arteriolar plaque, measuring approximately 200 µm in length, corresponding to a discrete area of retinal whitening of the inferior macula. There was a large flame-shaped hemorrhage overlying the proximal inferior arcade and a small intraretinal hemorrhage in the macula. There was no sheathing of the vessels or other signs of vasculitis. Fundus examination of the left eye was normal.
Fluorescein angiography was significant for early blockage in the area of the edematous infarcted retina in the right eye (Figure 2). Also, there was delayed filling of the small macular arteriole that contained the plaque (Figures 3a and 3b).
Images: Loman J, Das A
What is your diagnosis?
Patient with decreased vision
This patient has findings consistent with vaso-occlusive retinopathy. Classically, retinal artery occlusions are usually thought not to be associated with retinal hemorrhages; however, they can be present. The differential diagnosis of a branch retinal artery occlusion is related to diagnosing the source of the embolus. The embolus may be a cholesterol embolus from a carotid artery plaque, a calcific embolus from a calcified heart valve or a fibrin platelet thrombotic embolus formed from an atherosclerotic plaque or stenotic heart valve. Thrombus formation can also result from inflammatory causes such as SLE or temporal arteritis. If the patient had a history of intravenous drug use, septic embolus or talc embolus would also be considered. Hypercoagulable states that can cause thrombus formation and vaso-occlusive retinopathy are also on the differential; these include homocystinuria, anti-thrombin III, antiphospholipid Ab, factor V Leiden, protein C and S, and oral contraceptive use.
Clinical course and treatment
Due to the possible risk of recurrent emboli, this SLE patient was urgently admitted and received anti-inflammatory and anti-coagulation therapy. She received cyclophosphamide and heparin therapy and was then maintained on warfarin anti-coagulation. It was decided that there was no utility to attempt to dislodge the embolus by ocular massage or by acutely lowering eye pressure with a paracentesis because her symptoms had been present for 5 days.
She also had an extensive workup to delineate the source of her emboli. Her laboratory studies that would suggest a lupus flare were within normal limits (ANA=1:160, DS-DNA=1:10, C3=106, C4=25, ESR=40, CRP<0.4). her="" anti-ssa,="" ssb,="" sm="" and="" rnp="" were="" negative.="" her="" carotid="" ultrasound="" was="" negative="" for="" significant="" stenosis.="" her="" hypercoagulability="" workup="" was="" negative.="" her="" transthoracic="" echocardiogram="" demonstrated="" an="" extensive="" posterior="" mitral="" annular="" calcification,="" which="" was="" described="" as="" an="" irregular="" mass-like="" calcification="" measuring="" 1="" cm="" by="" 2="" cm.="" she="" was="" noncompliant="" with="" her="" follow-up="" transesophageal="" echocardiogram.="">0.4).>
Two months later, the patient’s vision and ophthalmologic exam remained stable. She was continued on anticoagulation and was on a taper of steroids but again failed to go for her transesophageal echocardiogram.
Given her clinical course, the patient’s branch retinal artery occlusion was thought to be due to a calcific or thrombotic embolus from a calcified mitral heart valve. Her calcific heart valve was thought to be due to recurrent inflammation from her SLE.
As noted earlier, branch retinal artery occlusions have various etiologies, but only 62% will demonstrate noticeable emboli. Alternatively, the Beaver Dam study showed a 1.3% incidence of retinal emboli in an asymptomatic population. Seventy-five percent of branch retinal artery occlusions have good long-term visual outcomes with better than 20/40 visual acuity, as in our patient. However, recurrent embolic events are frequent and the mortality rate after the first year is 13%. Ocular massage and lowering IOP to dislodge the embolus in the first 24 hours is noted to have limited success. Of note, combined branch vein and artery occlusions are thought to be more common in cases of inflammatory processes such as SLE, hypercoagulable states and AIDS.
It is important to perform a complete workup to determine the cause of an embolus. SLE patients are known to develop SLE related vaso-occlusive retinopathy, but delineating the exact cause of an embolus in this population is equally important. SLE retinopathy can be categorized into three types. The more common type 1 SLE retinopathy (3% to 29% of those with SLE) results in cotton wool spots and intraretinal heme. This demonstrates small vessel disease that is similar to Purtscher-like retinopathy. Retinal artery and vein occlusions fall into the category of type 2 SLE retinopathy. This type demonstrates the accelerated arteriosclerosis, antiphospholipid positivity or calcific heart valve patients found in the spectrum of SLE. Severe type 3 SLE retinopathy is rare (4% of those with SLE retinopathy) and includes vasculitis with sheathing, capillary non-perfusion, attenuated arterioles, neovascularization and choroidopathy. This is thought to be due to immune complex deposition within the vessels. These findings are usually bilateral but can first present unilaterally and progress. Severe retinopathy is associated with central nervous system lupus and cerebritis.
In our patient, it is thought that the embolus was derived from her calcified mitral heart valve. Calcified retinal emboli are characteristically found in arterioles at or near the disc and usually originate from SLE, rheumatic fever or senile heart valves. A high percentage of SLE patients (21%) are noted to have thick or calcified heart valves. Histologic examination shows degenerative changes of the mitral valve due to chronic inflammation that causes proliferating fibrous changes and calcification. The presence of a valvular abnormality correlates with the duration of SLE but not the severity of the disease. Of note, there is an increased incidence of calcified heart valves in patients on hemodialysis, as in this patient. The treatment of calcific heart valves causing emboli is surgical. If a calcific stenotic valve results in a thrombotic embolus, anticoagulation may be warranted.
For more information:
- Jane Loman, MD, can be reached at New England Eye Center, Tufts University School of Medicine, 750 Washington St., Box 450, Boston, MA 02111; 617-636-4219; fax: 617-636-4866; Web site: www.neec.com.
- Arup Das, MD, PhD, can be reached at University of New Mexico School of Medicine, 2211 Lomas Blvd. NE, Albuquerque, NM 87131; 505-272-6120; fax: 505-272-6125; e-mail: email@example.com.
- Edited by Jane Loman, MD, and Zinaria Williams, MD. Drs. Loman and Williams can be reached at New England Eye Center, Tufts University School of Medicine, 750 Washington St., Box 450, Boston, MA 02111; 617-636-4219; fax: 617-636-4866; Web site: www.neec.com. Drs. Loman and Williams have no direct financial interest in the products mentioned in this article, nor are they paid consultants for any companies mentioned.
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