Lupus and the Eye

Lupus and the Eye
Stephen H. Sinclair, MDErnesto L. Collazo, MD
Systemic lupus erythematosus is a disease which can involve every organ in the human body. It is no surprise, therefore, that it can involve not only the eyes but also the orbit (eye socket) around the eyes and the eyelids that house them. It may even involve the nerve connections from the eye to the brain. Before we can discuss eye problems in lupus, it is first necessary to understand the structure of the human eye. The eye can be compared to a photographic camera.

The camera contains a lens which focuses an image onto the film, located against the back wall of the camera. The eye is arranged in a similar fashion. It is protected by the eyelids which act as windshield wipers, spreading tears over the surface of the eye (the cornea) to keep the cornea moist and clear. The lids have a smooth inner lining known as the conjunctiva. The conjunctiva is essentially a membrane which also covers the front portion of the eyeball preventing objects such as dirt and contact lenses from getting behind the eye. The front surface of the eye is covered by the cornea, a transparent window similar to the crystal of a watch. Behind the cornea is the iris which may be of various colors (brown, blue, green, etc.) and acts similarly to the diaphragm of the camera by controlling the amount of light that enters the eye.

Behind the iris is the lens which, like the lens of the camera, focuses the image on the retina at the back of the eye. It is not composed of glass but rather is a bag of clear protein. The eye's outer wall or sclera, the white of the eye, provides structural strength. The inside layer of the eye, known as the retina, is the light sensitive layer that is similar to the film of the camera. It lies flat against the back inside wall of the eye. The nerve impulses which it produces are transmitted to the visual part of the brain through the optic nerve in the back of the eye.

Skin and Eyelids

Lupus can produce inflammatory lesions (or sores) of the eyelids. The lesions may be small and individual ("discoid") or they may b more widespread causing swelling, irritation and redness of the eyelids, and sometimes loss of the eyelashes. Over time these lesion, if untreated, may cause scarring which can distort the eyelids and prevent them from performing their function of blinking and wiping the front of the eye. Treatment usually consists of systemic (taken internally) or topical (applied locally) corticosteroids to decrease inflammation.

Conjunctiva

The conjunctiva may also become inflamed causing redness and tearing, although this does not occur often. The tear glands are located in the conjunctiva. When lupus affects the conjunctiva, causing inflammation and scarring, it may reduce tear production resulting in dryness of the eye (which is often associated with mouth and vaginal dryness and is known as sicca or Sjogren's Syndrome). With reduced wetting, the cornea can lose its clarity and become roughened and irritable as well as prone to infection (ulcers). Rarely inflammation of the sclera or iris may also occur. Again systemic or topical (eye drops in this case) corticosteroids are used to reduce the inflammation. In addition artificial tear preparations and ointments can be used to reduce the dryness and subsequent irritation. Infection or ulcers of the cornea require prompt and careful treatment with antibiotics.

Retina

By far the most common effect of systemic lupus erythematostis on the eye is injury to the blood vessels that supply the retina. Small retinal hemorrhages can result, as well as occlusions or blockages of the smaller retinal blood vessels. This causes damage to the retinal tissue due to lack of an adequate blood supply. Infrequently, larger arteries may also be occluded leading to loss of function in large areas of the retina. When this involves the center of the retina, known as the macula, a large area of central vision is lost. The optic nerve may also suffer from blockages of the blood vessels which supply it, resulting in loss of vision in the entire eye. In rare cases the blockages of blood vessels in the retina may be so severe and widespread that the retina may, in response to the blockages, attempt to develop new vessels. This process is termed "neovascularization." Such new vessels grow abnormally out from the surface of the retina into the central vitreous gel (the substance that fills the inside of the eye). They are prone to bleeding when stretched or pulled by the gel, resulting in severe vision loss. Although these abnormal blood vessels are rare in patients who are on adequate treatment for lupus, all lupus patients should be followed with periodic eye examinations because the blockages and abnormal vessel growth may occur without the patient noticing symptoms. If blockages or neovascularization are detected early, they may be treated with a laser. This procedure is usually done as an outpatient.

Central Nervous System

Involvement of the central nervous system is uncommon but, when it occurs, may be due to blood vessel inflammation and blockage within the brain. The resulting damage may affect the visual system in a variety of ways. These include defects or gray areas of the visual field in one or both eyes, eye movement abnormalities which can lead to double vision, disturbances in the function of the pupils, and damage to the optic nerve leading to loss of vision in one eye.

Complications of Systemic Drugs

Treatments for reducing the systemic inflammation of lupus (inflammation which can affect any area of the body) are vitally important to the well-being of the patient and also lessen the effect of inflammation on the eye, eyelids, or orbit. Some of the drugs which are used in the treatment of systemic lupus, however, can have side effects on the eyes. The long-term use of steroids, either systemic or topical, may cause the formation of cataracts. A cataract is a change in the lens of the eye which can affect the ability of the lens to focus. It can produce opacities or defects that create glare and haziness of vision. Most mild cataracts are treated with corrective eyeglasses or special lens filters. When the glare or haziness become such a problem that patients can no longer see well enough to go about their daily lives, then the lens with the cataract can be removed surgically and replaced with an artificial lens which restores the vision.

In addition, corticosteroids can lead to the development of' glaucoma, a condition in which an increase in the pressure within the eye causes damage to the optic nerve and an irreversible loss of' vision. This condition can be treated with medications to lower the pressure within the eye, therefore preventing further damage to the optic nerve. Periodic eye examinations are vital to the early detection and treatment of glaucoma. The use of antimalarials has become widespread in the treatment of lupus patients. One of the first of these, chloroquine, was found to have a significant toxic effect on the eye. Over the years the drug has a tendency to accumulate in the layer between the retina and the sclera, and in some cases this leads to severe destruction of the central retina with central vision loss.

A drug similar to chloroquine is more commonly used today. It is called hydroxychloroquine (Plaquenil). This drug is apparently not as toxic and vision loss is extremely rare, even with relatively high doses taken over several years. Lupus patients who are on Plaquenil are still checked carefully with periodic vision and field testing and evaluation of the retina. If toxic side effects to the retina do occur, they are detected early, and the drug can be stopped without any further retinal damage and no injury to vision.

=========================================================== This information is for "informational purposes" and is not meant to be used for medical diagnosis. Always consult your physician on matters such as this.

Lupus and Anemia

Lupus and Anemia
Douglas B. Cines, MD

Anemia is the condition in which an individual has less than the normal number of red cells in the blood. Anemia occurs frequently in patients with lupus and some of these patients may require special attention. How do you know if you are anemic? Patients with anemia may have no symptoms or may feel fatigued because not enough red blood cells are available to carry oxygen to muscles and other tissues. However, similar feelings of fatigue may be caused by lupus itself. Occasionally, patients who are anemic may feel dizzy, experience a rapid heart beat or chest discomfort. These symptoms are more likely to develop following exercise when the body's demand for oxygen is increased.

If you experience increasing fatigue or related symptoms, your physician may check to see if you are anemic. Your doctor may use the term "hematocrit" to describe the number of red cells in your blood. The hematocrit refers to the proportion of whole blood occupied by the red cells themselves. Usually about 40% of the blood is composed of red cells, but in patients with anemia, this proportion may be reduced to 20-30%.If you are found to be anemic, your physician may order other tests to find out the cause. There are four common causes of anemia that patients with lupus experience.

Anemia of Chronic Illness. Normally, as red blood cells age, approximately 1% of them are removed from the blood each day. The bone marrow, where all blood cells are made, replaces these cells with new ones. When lupus is active, many patients develop anemia because the bone marrow produces fewer red blood cells than are needed. The exact reason why fewer cells are produced is unknown, but in some way this is related to the tissue inflammation caused by lupus. In most patients, this form of anemia is mild and produces no symptoms. This form of anemia does not respond to changes in diet, vitamins, etc., but improves when the lupus has been controlled.

Iron Deficiency Anemia. Sometimes anemia is caused by iron deficiency. Iron deficiency is also a common cause of anemia in healthy women who do not have lupus. It usually results from loss of iron over many years due to normal menstrual bleeding. Medications used to treat lupus, such as aspirin, may cause irritation of the stomach lining leading to bleeding and iron deficiency. If you are anemic, you should get a blood test to determine if you are iron deficient before taking iron pills, because iron has no beneficial effect if the anemia is caused bv another problem.
Anemia of Kidney Disease. Some patients with lupus develop kidney damage. The kidney makes a hormone called erythropoietin that is needed by the bone marrow to produce red blood cells.

If the kidney is damaged and does not produce this hormone, the bone marrow will produce fewer red blood cells. Your doctor will be able to tell by urine and blood tests if kidney damage is responsible for the anemia. If kidney function improves with treatment, the production of erythropoietin increases and the anemia improves.

Autoimmune Hemolytic Anemia. Anemia may also result from the production of an antibody that binds to an individual's own red blood cells. 'I'his form of anemia, called autoimmune hemolytic anemia (AHA), occurs in approximately 5% of patients with lupus. An antibody is a type of protein made by the body, usually in response to infection. Patients with lupus commonly produce antibodies that bind to their own cells. In some cases, these antibodies bind to the red cells. These red cells coated with antibodies are quickly removed from the blood and destroyed by white blood cells, called macrophages, that normally destroy antibody coated viruses or bacteria as part of the body's defenses against infection. If the production of new red cells cannot keep up with this destruction, the patient becomes anemic.

Your physician can tell if you have autoimmune hemolytic anemia by looking at your blood under the microscope. If you do, the physician will see both red cells whose shape has been altered by these macrophages and a large number of newly produced, young red blood cells. The diagnosis of AHA is confirmed using the direct antiglobulin test, or Coombs' test, which measures both the presence and type of antibody bound to the red cell. The treatment of AHA depends upon its severity. If the anemia is mild, there may be no need for treatment. In one variety of uncommon AHA, called "cold-agglutinin disease", simple measures such as preventing exposure to cold may be sufficient. Periodic transfusions of warmed blood are often useful for patients with cold-agglutinin disease who have mild symptoms.

Patients with the more common, "warm-antibody" type AHA often require additional measures to correct the anemia. In most cases, the doctor will recommend the drug, prednisone, which both decreases the rate of red cell destruction and the amount of antibody produced. Sometimes prednisone must be taken for several weeks before its effect becomes apparent. Patients who do not respond to prednisone, or who require high doses of prednisone to control the anemia, may be advised to have an operation to remove their spleen. The reason why this procedure is so often recommended is that in many patients, most of the red cells to which antibodies have become attached are trapped and destroyed by macrophages within the spleen. In some patients the hormone, Danazol, or other drugs such as cyclophosphamide (Cytoxan) may also be recommended.

Transfusions are used only as a temporary measure since the transfused red cells will also quickly become coated with antibodies and will be destroyed. AHA can be managed Successfully in practically all patients with systemic lupus, but the specific recommendations for treatment vary among different patients for several reasons. First, the doctor must be sure of the diagnosis. Other causes of anemia may also be present and may contribute to the anemia. Second, the doctor must determine if the anemia is actually contributing to the patient's symptoms, since one of the major reasons to treat a patient is to improve how she/he feels. Third, the amount of medicine needed to treat AHA may vary from time to time. Patients vary widely in their ability to tolerate anemia and their ability to tolerate each form of treatment. Therefore, treatments must be custom tailored to fit each individual.

=========================================================== This information is for "informational purposes" and is not meant to be used for medical diagnosis. Always consult your physician on matters such as this.