By: Mary Terlaak-Smi
DIPOMATE, AMERICAN BOARD OF OPHTHALMOLOGY
Dr. Mary Terlaak-Smith is an eye physician and surgeon at The Associated Eyes & Surgeons Medi
cal Group at Scripps Memorial Hospital, La Jolla CA
Systemic Lupus Erythematosus is a chronic, systemic, immunologically mediated disease
of unknown etiology. The underlying abnormalities are the production of a number of pathogenic autoantibodies and immune complexes and the inability to suppress or clear them. Traditionally eleven diagnostic criteria have been referenced to diagnose SLE; more recently a twelfth has been proposed, Ophthalmologic Involvement. The diagnosis of SLE can be made if four of these criteria are met. This would
be rather straightforward if SLE did not have such a variable clinical course in terms of severity and rate of progression. In fact, serologic testing for autoantibodies can be negative at presentation. The constitutional symptoms of fever, malaise, arthralgias, headache and loss of appetite and weight can be prominent and yet the correct diagnosis can evade us.
Not infrequently, involvement of the visual system can be the manifesting sign of SLE. An ocular inflammatory problem may precede diagnosable SLE by five or more years, but more importantly for the patient with known disease, the eye is an especially sensitive barometer for activity in other systems. Periodic ophthalmologic exams can monitor for the emergence of potentially serious problems.
Keratoconjunctivitis Sicca (KCS), a condition of dry mucous membranes of the eye and mouth, is the most common ocular manifestation of SLE, occurring in approximately 25% of patients. The skin involvement of the malar rash seen in as many as 85% of patients is not infrequently detected by an ophthalmologist. Discoid lupus can appear on the eyelids as small scaly patches that do not respond to the usual therapy for blepharitis. Autoimmune reactions in other tissues of the wall of the eye, such as keratitis and scleritis, may be painful and threatening to vision.
Most significantly, however, SLE, can involve the retina. The classic lesion of SLE is a white fluffy appearing lesion within the retina known as a cotton wool spot. This may or may not be surrounded by hemorrhage or swelling. Lupus retinopathy appears to be microscopic areas of infarction, that is, mini strokes, with scattered spots of retinal detachment. Irregularities of the circulation can be seen with dilated capillaries and narrowed arteries. Similar changes are seen in the retinas of individuals with high blood pressure, which is not an uncommonly associated problem for SLE patients.<p>
Far fewer patients develop more severe retinal inflammation of the vessels, a sort of vasculitis with closure of small arterioles. In a progression not dissimilar to the retinopathy of diabetes, the retina may develop areas of poor circulation which result in the growth of abnormal vessels. Autoimmune complexes may cause the close of a large retinal vein or artery with severe loss of vision.
logic manifestations of SLE frequently involve the optic nerve or motor nerves to the eyes that would be detected on a complete eye exam. Changes of the pupils, double vision and migraine headache can all signal lupus activity in the nervous system. But all these warnings are not harbingers of doom, but opportunities for the eye to be a window into the patient’s immune system. These visible signs warn of potential problems and allow us to closely monitor and treat a disease that often progresses invisibly in other tissues. An aware patient with SLE can protect more than his precious sight by seeing an opthalmologist.