Retinopathy in systemic lupus erythematosus. A case report and review of the literature

Abstract

Retinal lesions occur frequently in systemic lupus erythematosus, however, they rarely cause visual impairment. A patient btinded by severe lupus retinopathy is described, and the pertinent literature is reviewed.Systemic lupus erythematosus not infrequently causes retinopathy (1-4). Subsequent visual impairment, however, is not common and blindness due to lupus retinopathy is rare (2). Clinically observed retinal lesions include cotton wool exudates in 9 4 4 % of cases (2, 3, 5) and superficial flame-shaped, or preretinal hemorrhages in about 10% (6). Less common are retinal arteritis and phlebitis, which when severe may lead to perivascular sheathing and fibrosis (7). Papilledema with optic atrophy and retinal detachment are seen only rarely and most often terminally (6,7).Severe retinopathy was more commonly seen in the presteroid era and, at present, occurs most frequently in patients whose generalized lupus activity has not been well controlled. It is of interest then to report a case of probable lupus erythematosus in which the eyes were involved early in the course and were the organs most severely affected by the disease process. CASE HISTORYThe patient was a 23-year-old black woman, well until one month prior to admission when she developed a malar eruption, anorexia, weight loss, malaise, myalgias, low grade fever and fatigue. She denied a history of arthritis, chest pain, headaches, abdominal pain or change in bowel habits. She had used oral contraceptives for three years prior to the onset of her illness.Initial examination revealed a thin black woman in minimal physical distress, with a blood pressure of 110/60 mmHg, regular pulse of 84, and oral temperature of 101°F. Other pertinent findings included a discoidal skin eruption in a malar distribution; medullated nerve fibers in the left eye; bilateral, tender, easily movable 3 X 3 cm axillary lymph nodes, and several small petechiae on the right foot.Laboratory findings included a persistent pancytopenia with WBC of 2,800-3,500/cu mm, hematocrit of 33-38%, and platelet counts between 75,000 and 160,OOO/cu mm. ESR on multiple occasions was between 55-110 mm/hr. Urinalyses showed 3-25 WBC/hpf, 3+ proteinuria, occasional RBCs and no casts. A 24-hour urine contained 600 mg of protein.Total serum protein was 8.2 8% with an albumin of 3.4 g%. Serum protein electrophoresis showed a nonspecific y globulin elevation. Antinuclear antibody (using mouse liver as the substrate), was 4+ with a speckled pattern, LE preparations were negative X 5, serum complements were 173 and 186 hemolytic units (normal greater than 150 hemolytic units) : latex fixation test was negative. Serum electrolytes, CO,, fasting blood sugar, creatinine clearance, SGOT, SGPT, Ca and P were normal. Serology was nonreactive and the EKG was within normal limits. T h e chest X-ray demonstrated mini-

Combined Arterial and Venous Retinal Occlusion as a Presenting Sign of Systemic Lupus erythematosus

“…We presume that the vascular mecha nism of the ocular complications of SLE seen in our patient is similar to that of the patients described above [16][17][18][19], We assume that the focal arteriolar narrowing, found secondary to endothelial and medial proli feration due to vasculitis, was aggravated in our patient by the hypercoagulability state of SLE [20.27], or by the secondary effects of the oral contraception which the patient used. The latter probably aggravated the venous stasis, and contributed to the severe wall damage of the vascular bed.…”

“…The visual acuity improved in both eyes after intravenous administration of a gold compound. Bishko [17] described a similar case ofearly-onset lupus retinopathy in 1972, which did not respond to steroid treatment. In 1978, Silverman et al [21] reported central retinal vein occlusion as an initial complication in the course of active SLE.…”

“…The clas sic SLE retinopathy, which is related to the focal occlusions of the smallest arterioles, includes the cotton wool spots, the location of which corresponds to that of the avascular zones seen on fluorescein angiography. Su perficial hemorrhages may be associated with these changes [17]. Disc edema and hyperemia [21] accompanied by focal or gen eralized retinal edema may occur.…”

“…Clinically, the patient had retinal edema, cotton wool spots, and dilated veins. Bishko [17] described a normotensive woman with SLE who was receiving corti costeroids and oral contraceptives and who developed retinal vasculitis with retinal ed ema and hemorrhages. Another case of mul tifocal retinal arterial occlusions in SLE was reported by Hammami and Streiff [ 18], who observed branch arterial obstructions, cot ton wool spots, retinal hemorrhages and pa pilledema in a 13-year-old boy.…”

Cerebral disease in systemic lupus erythematosus

“…Although two SLE patients may have the same neurological symptom, findings at autopsy may be quite different. For example, visual defects may result from any of a number of causes, including retinal vascular lesions [7,51,52], central retinal arterial [7,31] or venous occlusion [66], or ischaemia of visual pathways posterior to the optic chiasm and of the occipital cortex [15]. On the other hand, visual defects may be secondary to optic neuritis, where demyelination of the optic nerve may occur [2,46,69].…”