Clinical Care and Practice Advancement
Systemic Lupus Erythematosus and the Eyes
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 cause of lupus remains unknown, although genetic, hormonal, immunologic, and environment factors have been implicated. There is no cure for lupus; however, the disease can be effectively managed in most instances with corticosteroids and/or immunosuppressive therapy. Lupus patients are also encouraged to maintain an active and healthy life style.
The effects lupus may have in and around the eyes include2:
- Changes in the skin around the eyelids
- Dry eyes
- Inflammation of the white outer layer of the eyeball (scleritis)
- Blood vessel changes in the retina – the light sensitive lining inside the eye
- Damage to nerves in the muscles controlling eye movement and the nerves affecting vision
1. Involvement of the skin around the eyelids
- This is most often related to the discoid lupus erythematosus3 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.
2. Dry eyes
- Approximately 20 percent of people with lupus also have Sjögren’s syndrome, a condition in which the tear glands do not produce sufficient tears to lubricate and nourish the eye.
- 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.2,4
- This painful red eye condition is caused by inflammation in the white scleral (outer) layer of the eye.
- Scleritis occurs in approximately 1 percent of people with lupus and may be the first sign of the disease.
4. Retinal Vascular Lesions (blood vessel changes in the retina)
- This is the most common form of eye involvement in lupus.2
- The occurrence in lupus can vary depending on the population studied. The lowest incidence reported is 3%, seen in outpatient clinics, and the highest is 28% in those hospitalized for lupus-related complications.2
- 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 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.
5. Neuro-ophthalmic Involvement (nerve damage)
- Cranial nerve palsies can result in double vision, poor eye movement and alignment, poor pupil reflexes, and droopy eye lids.
- Lupus optic neuropathy occurs in 1-2% of people with lupus;2 slow progressive vision loss also can result in more rapid loss of vision from lupus optic neuropathy.6
- Damage to the visual nerve fibers in the brain may cause hallucination and loss of peripheral vision and/or central vision.
In addition, some of the medications used in the treatment of lupus may have ocular side effects. For example, hydroxychloroquine (Plaquenil) can cause retinal toxicity if taken on large doses over time. Regular annual comprehensive eye examinations are recommended by the American Optometric Association for people with lupus, especially for anyone taking Plaquenil.
- 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 lupus erythematosus. Arch Ophthalmol 1986; 104:564-568
- Lessell S. The neuron-ophthalmology of systemic lupus erythematosus. Doc Ophthalmol 1979; 47:13-42.