Systemic lupus is a chronic autoimmune disease that can affect any part of the body, including the eyes. Lupus most often affects the heart, joints, skin, lungs, blood vessels, kidneys and central nervous system (CNS). The clinical course is unpredictable and is characterized by periods of remissions and flares, which may be acute or chronic.

The effects lupus may have in and around the eyes include: changes in the skin around the eyelids, dry eyes, inflammation of the white outer layer of the eyeball, blood vessel changes in the retina, and damage to nerves controlling eye movement and affecting vision.

Involvement of the skin around the eyelids

• This is most often related to the discoid lupus erythematosus form of cutaneous lupus.
• The skin lesion is well-defined, slightly raised, scaly, and misformed (atrophic).
• There are typically no symptoms, but occasional burning and itching may occur.
• Scarring may result in deformities along the edge of the eyelids.

Dry eyes

• Approximately 20 percent of people with lupus also have secondary Sjogren’s syndrome, a condition in which the tear glands do not produce sufficient tears to lubricate and nourish the eyes; the other moisture-producing glands are similarly affected. (Primary Sjogren’s syndrome is a systemic disease that, like lupus, can affect many parts of the body.)
• Typical symptoms are irritated, gritty, scratchy, or burning eyes, a feeling of something in the eyes, excess watering, and blurred vision.
• Advanced cases of dry eyes may result in damage to the front surface of the eye and impaired vision.
• The dry eye that is seen in lupus cannot be distinguished from other dry eye conditions.

Scleritis

• This painful red eye condition is caused by inflammation in the white scleral (outer) layer of the eye.
• Scleritis occurs in approximately one percent of people with lupus and may be the first sign of the disease.

Retinal vascular lesions (blood vessel changes in the retina)

• This is the most common form of eye involvement in lupus.
• The occurrence in lupus can vary depending on the population studied. The lowest incidence reported is three percent, seen in outpatient clinics, and the highest is 28 percent in those hospitalized for lupus-related complications.
• The presence of these lesions seems to correlate with active disease.
• Retinal blood vessel changes are due to lack of adequate blood supply to this delicate tissue, and may cause decreased vision ranging from mild to severe. For individuals with severe retinal vascular disease, the prognosis for vision is poor.
• Retinal vein occlusions (blockages) and retinal artery occlusions have been reported, but these complications are rare and seem to be more related with CNS lupus.
• The choroidal layer of the eye -- the nourishing tissue underneath the retina -- can also be affected by lupus, but this is very uncommon. This involvement can appear as excess fluid between the retinal layers. There is an association between lupus choroidal disease and blood vessel disease in the rest of the body, which may be related to kidney disease and blood vessel disease complications seen in lupus.

Neuro-ophthalmic involvement (nerve damage)

• Cranial nerve palsies can result in double vision, poor eye movement and alignment, poor pupil reflexes, and droopy eyelids.
• Lupus optic neuropathy occurs in one-two percent of people with lupus. Slow progressive vision loss also can result in more rapid loss of vision from lupus optic neuropathy.
• Damage to the visual nerve fibers in the brain may cause hallucination and loss of peripheral vision and/or central vision.

Side-effects of certain lupus medications

In addition, some of the medications used in the treatment of lupus may have ocular side effects. In particular, hydroxychloroquine (Plaquenil®) can cause retinal toxicity over time, particularly at high dosages. 

Annual comprehensive eye examinations are recommended by the American Optometric Association for people with lupus, especially for anyone taking Plaquenil. As a precaution, people treated with Plaquenil should get a baseline eye exam before (or soon after) starting the drug and visit an eye doctor (ophthalmologist) annually. Long term plaquenil users on high doses will need to monitor eye health regularly to prevent retinal toxicity from long-term use.

For further information, consult these references:

• Schur PH. Systemic lupus erythematosus. In: Goldman L, Ausiello D. Cecil Textbook of Medicine. 22nd ed., Philadelphia, Saunders, 2004:1660-1670.
• Thorne JE, Jabs DA. Rheumatic Disease. In: Ryan SJ, Schachat AP, eds. Retina, Vol. II—Medical Retina. 4th ed. St. Louis: Mosby, 2006:1390-1408.
• Huey C, Jakobiec FA, Iwamoto T et al. Discoid lupus erythematosus of the eyelids. Ophthalmology 1983; 90:1389-1398.
• Hochberg MC, Boyd RF, Aheran JM et al. Systemic lupus erythematosus: a review of clinico-laboratory features and immunogenic markers in 150 patients with emphasis on demographic subsets. Medicine (Baltimore) 1985; 64:285-295.
• Jabs DA, Miller NR, Newman SA et al. Optic neuropathy in systemic lupus erythematosus. Arch Ophthalmol 1986; 104:564-568
• Lessell S. The neuron-ophthalmology of systemic lupus erythematosus. Doc Ophthalmol 1979; 47:13-42.

The Lupus Foundation of America would like to thank the American Optometric Association and Mary Beth Rhomberg, O.D., for this information.