Systemic Lupus Erythematosis

THE PHOTOS above are of a 36-year-old woman with a butterfly rash, membranous glomerulonephritis and hypertension. She had been previously diagnosed with Systemic lupus erythematosis with hematologic tests showing circulating immune complexes, antinuclear antibodies and antiphospholipid antibodies. She recently developed an episode of episcleritis on her right eye (photo above right) which resolved over a 2-3 day period. She then complained of visual loss in this same eye. Clinical examination revealed the presence of multiple cotton wool spots along with areas of active retinal vasculitis (photo above left) which were most evident angiographically.

Systemic lupus erythematosis (SLE) is an autoimmune disorder characterized by multiorgan involvement. The disease tends to fluctuate with clinical exacerbations and remissions. The typical patient with the disease is an African American woman in her mid 30's with a family history of lupus or an autoimmune disease. The systemic manifestations of lupus are well known and include fatigue, weight loss, fever, midfacial butterfly rash, discoid skin lesions, focal or diffuse neurologic disorders, pericarditis, and various forms of nephritis often leading to secondary hypertension. The ocular manifestations are often secondary to the systemic diseases or treatments associated with SLE. As a result of the renal dysfunction and secondary hypertension, SLE patients will often show retinal findings characteristic of hypertensive retinopathy.

The presence of circulating immune complexes is often contributory to the development of retinal vasculitis. The antiphospholipid antibodies make SLE patients prone to retinal vascular occlusions and optic nerve head infarctions. The systemic treatment SLE patients receive, such as systemic prednisone can also cause ocular toxicity in the form of cataracts, secondary glaucoma or central serous choroidopathy, a condition characterized by leakage beneath the retina. Finally, some patients with SLE are treated with chloroquine, a drug well known to cause a "bull's eye maculopathy."