Lupus is a chronic, autoimmune disease which causes inflammation of various parts of the body, especially the skin, joints, blood and kidneys. The body’s immune system normally makes proteins called antibodies to protect the body against viruses, bacteria and other foreign materials. These foreign materials are called ANTIGENS. In an autoimmune disorder such as Lupus, the immune system loses its ability to tell the difference between foreign substances (antigens) and its own cells and tissues. The immune system then makes antibodies directed against “self.” These antibodies, called “auto-antibodies” react with the “self” antigens to form immune complexes. The immune complexes build up in the tissues and cause inflammation, injury to tissues, and pain.
More people have Lupus than AIDS, cerebral palsy, multiple sclerosis, sickle-cell anemia, and cycsic fibrosis combined. Lupus Foundation of America market research data show that between 1.4 and 2 MILLION people reported to have been diagnosed with Lupus. For most people, Lupus in a mild disease affecting only a few organs, but for others, it can be serious, or even life threatening.
“Lupus” is Latin for the word “wolf,” and it is common medical lore that the “butterfly rash” seen on the cheeks of many Lupus patients is so similar to the markings of a wolf. The medical name for the disease that we know of as Lupus-Lupus Erythmatosus-was first applied to a skin disorder by a Frencham, Pierre Cazenave, in 1851, though descriptive articles detailing the condition date back to Hippocrates in ancient greece.
Lupus is often called a woman’s disease despite the fact that men can be affected as well. Lupus can occur, at any age, in any sex, although it occurs 10-15 times more frequently among adult females than adult males. The symptoms of the disease are the same in men and in women.
Lupus is a chronic inflammatory condition caused by an autoimmune disease. Autoimmune diseases are illnesses which occur when the body’s tissues are attacked by its own immune system. The immune system is a complex organization within the body that is designed normally to fight infections and other foreign invaders. Patients with lupus have unusual antibodies in their blood that target their own body tissues. Lupus can cause disease of the skin, heart, lungs, kidneys, joints, and nervous system. When only the skin is involved, the condition is called discoid lupus. When internal organs are involved, the condition is called systemic lupus erythematosus.
Both discoid and systemic lupus are more common in women than men (about eight times more). The disease can affect all ages, but most commonly begins from age 20 to 45 years. It is more frequent in African-Americans and people of Chinese and Japanese descent.
Factors which activate autoimmune disturbances that cause lupus are not known. Genetics, viruses, ultraviolet light, and drugs may all play some role. Genetic factors increase the tendency of developing autoimmune diseases. Autoimmune diseases such as lupus, rheumatoid arthritis, and thyroid disorders are more common among relatives of patients with lupus than the general population. Some scientists postulate that the immune system in lupus patients is more easily activated by external factors like viruses or ultraviolet light. Sometimes, symptoms of lupus can be precipitated or aggravated by only a brief period of sun exposure.
Certain medications used in treating high blood pressure (such as hydralazine), abnormal heart rhythms (such as procainamide), and epilepsy (such as phenytoin) have been known to activate immune systems and cause lupus. Fortunately, this “drug-induced” lupus is infrequent (less than 5%) and usually resolves when the medications are discontinued.
It is also known that women patients with systemic lupus not infrequently experience worsening of symptoms prior to their menstrual periods. This phenomenon, together with the female predominance of those with the disease, suggest that female hormones play an integral role in the expression of systemic lupus. This hormonal relationship is an active area of ongoing study by research scientists.
In discoid lupus, only the skin is involved. Over time, 5 to 10% of patients with discoid lupus may develop systemic lupus. The skin involvement in discoid lupus is often found on the face and scalp. It is usually red, may have raised borders, and can scar. Discoid lupus skin rashes are usually painless and do not itch, but scarring of discoid skin lesions can cause permanent hair loss. Patients with systemic lupus can develop different combinations of symptoms and organ involvement. Common complaints and symptoms include fatigue, low-grade fever, loss of appetite, muscle aches, arthritis, ulcers of the mouth and nose, facial rash (”butterfly rash”), unusual sensitivity to sunlight (photosensitivity), inflammation of the lining of the lung (pleuritis) and the heart (pericarditis), and poor circulation to the fingers and toes with cold exposure (Raynaud’s phenomenon).
More serious organ damage involves inflammation of the brain tissue, hepatitis, and kidney damage and failure. Certain blood cells and blood clotting factors can also be affected in systemic lupus, thereby increasing the risk of infection and bleeding.
Over half of the patients with systemic lupus develop a characteristic red, flat facial rash over the bridge of the nose. Because of its shape, it is frequently referred to as the “butterfly rash” of lupus. The rash is painless and does not itch. The facial rash, along with inflammation in other organs, can be precipitated or worsened by exposure to sunlight, a condition called photosensitivity. This photosensitivity can be accompanied by a worsening of inflammation throughout the body, called a “flare” of disease.
With an increase in the activity of systemic lupus, many patients start to experience arthritis, inflammation of the lungs, kidney inflammation, and hair loss to name a few. Most systemic lupus patients will develop arthritis some time during the course of their illness. Arthritis in systemic lupus commonly involves swelling, pain, stiffness, and even deformity of the small joints of the hands, wrists, and feet. Sometimes, arthritis of systemic lupus can mimic that of rheumatoid arthritis (another autoimmune disease). Inflammation of the lining of the lungs (pleuritis) and of the heart (pericarditis) can cause sharp chest pain. The chest pain is aggravated by coughing, deep breathing, and certain changes in body posture. Kidney inflammation in systemic lupus can cause leakage of protein in the urine, fluid retention, high blood pressure, and even kidney failure. With kidney failure, machines are needed to cleanse the blood of accumulated poisons in a process called dialysis. Brain tissue involvement can cause personality changes, thought disorders (psychosis), seizures, and even coma. Damage to nerves can cause numbness, tingling, and weakness of the involved body parts or extremities. Some patients with systemic lupus have Raynaud’s phenomenon. In these patients, blood supply to the fingers and toes becomes interrupted upon cold exposure, causing blanching, bluish discoloration, and pain in the exposed fingers and toes.
Since patients with systemic lupus can have a wide variety of symptoms and different combinations of organ involvement, no single test establishes the diagnosis of systemic lupus. To help doctors improve the accuracy and confidence in the diagnosis of systemic lupus, eleven criteria were established by the American Rheumatism Association. These eleven criteria are closely related to the symptoms discussed above.
Some patients suspected of having systemic lupus may never develop enough criteria for a definite diagnosis. Other patients accumulate enough criteria only after months or years of observation. When a person has four or more of these criteria, the diagnosis of systemic lupus erythematosus is suggested.
The eleven criteria used for diagnosing systemic lupus erythematosus are:
malar (over the cheeks of the face) “butterfly” rash
discoid skin rash: patchy redness that can cause scarring
photosensitivity: skin rash in reaction to sunlight exposure
mucus membrane ulcers: ulceration of the lining of the mouth, nose or throat
arthritis: 2 or more swollen, tender joints of the extremities
pleuritis/pericarditis: inflammation of the lining tissue around the heart or lungs, usually associated with chest pain with breathing
kidney abnormalities: abnormal amounts of urine protein or cellular elements
brain irritation: manifested by seizures (convulsions) and/or psychosis
blood count abnormalities: low counts of white or red blood cells, or platelets
immunologic disorder: abnormal immune tests include anti-DNA or anti-Sm (Smith) antibodies, or positive LE prep test
antinuclear antibody: positive ANA antibody testing
In addition to the eleven criteria, other tests can be helpful in evaluating patients with systemic lupus to determine the severity of organ involvement. These include routine testing of the blood to detect inflammation (for example, a test called “sedimentation rate”), blood chemistry testing, direct analysis of internal body fluids, and tissue biopsies. Abnormalities in body fluids and tissue samples (kidney, skin, and nerve biopsies) can further support the diagnosis of systemic lupus erythematosus. The appropriate test procedures are selected for the patient individually by the doctor.
There is no permanent cure for systemic lupus. The goal of treatment is to relieve symptoms by decreasing inflammation and/or the level of autoimmune activity in the body. Many patients with mild symptoms may need no treatment or only intermittent courses of antiinflammatory medications. Those with more serious illness involving damage to internal organ(s) may require high doses ofsymptoms by decreasing inflammation and/or the level of autoimmune activity in the body.
Patients with systemic lupus need more rest during periods of active disease. During these periods, carefully prescribed exercise is still important to maintain muscle tone and range of motion in the joints. Nonsteroidal antiinflammatory drugs (NSAIDs) are helpful in reducing inflammation and pain in muscles, joints, and other tissues. Examples of NSAIDs include aspirin, ibuprofen (MOTRIN), naproxen (NAPROSYN), and sulindac (CLINORIL). Since the individual response to NSAIDs varies among patients, it is common for a doctor to try different NSAIDs to find the most effective one with the fewest side effects for the patient. The most common side effects are stomach upset, abdominal pain, ulcers, and even ulcer bleeding. NSAIDs are usually taken with food to reduce side effects. Sometimes, anti-ulcer medications, such as CYTOTEC, are given simultaneously.
Corticosteroids are more potent than NSAIDs in reducing inflammation and restoring function when the disease is active. Corticosteroids are particularly helpful when internal organs are involved. Corticosteroids can be given by mouth, injected directly into the joints and other tissues, or administered intravenously. Unfortunately, corticosteroids have serious side effects when given in high doses over prolonged periods. Side effects of corticosteroids include weight gain thinning of the bones and skin, infection, diabetes, facial puffiness, cataracts, and death (necrosis) of large joints. Hydroxychloroquine (PLAQUENIL) is an antimalarial medication found to be particularly effective for lupus patients with fatigue, skin, and joint diseases. Side effects include diarrhea, upset stomach, and eye pigment changes. Eye pigment changes are rare but require monitoring by an ophthalmologist (eye specialist) during treatment with PLAQUENIL.
Medications that suppress immunity (immunosuppressive medications) are also called cytotoxic drugs. Immunosuppressive medications are used for treating severe cases of systemic lupus with damage to internal organ(s). Examples include methotrexate (RHEUMATREX), azathioprine (IMURAN), cyclophosphamide (CYTOXAN), chlorambucil (LEUKERAN), and cyclosporine (SANDIMMUNE). All immunosuppressive medications can seriously depress blood cell counts and increase risks of infection and bleeding. RHEUMATREX can cause liver toxicity, while SANDIMMUNE can impair kidney function.
In systemic lupus patients with serious brain and kidney disease, plasmapheresis is sometimes used to remove antibodies and other immune substances from the blood to suppress immunity. Some systemic lupus
patients can develop seriously low platelet levels, thereby increasing the risk of excessive and spontaneous bleeding. Since the spleen is believed to be the major site of platelet destruction, surgical removal of the spleen is sometimes performed to improve platelet levels. Other treatments have included the use of male hormones.
Systemic lupus is undoubtedly a potentially serious illness with numerous organ system involvements. However, it is important to recognize that most patients with systemic lupus lead full, active, healthy lives. Periodic increases in disease activity (flares) can usually be managed by varying medications. Since ultraviolet light can precipitate and worsen flares, patients with systemic lupus should avoid sun exposure. Sunscreens and clothing covering the extremities can be helpful. Abruptly stopping medications, especially corticosteroids, can
also cause flares and should be avoided. Patients with systemic lupus are at increased risk of infections, especially if they are taking corticosteroids or immunosuppressive medications. Therefore, any unexpected fevers should be reported and evaluated.
Patients with systemic lupus who become pregnant are considered “high risk.” Lupus patients who are pregnant require close observation during pregnancy and delivery. These patients can have an increased risk of
miscarriages (spontaneous abortions) and can have flares of the disease activity during pregnancy. Presence of cardiolipin antibodies in the blood can identify patients at risk for miscarriages. Cardiolipin antibodies are associated with a tendency toward blood clotting. Patients with systemic lupus who have cardiolipin antibodies may need blood thinning medications during pregnancy to prevent miscarriages. Lupus antibodies can be transferred from the mother to the fetus and result in lupus illness in the newborn (”neonatal lupus”). Neonatal lupus usually clears within six months of birth as the mother’s antibodies are slowly metabolized by the baby.
The role of the immune system in causing diseases is becoming better understood by scientific research. This knowledge will be applied to design safer and more effective treatment methods. Overall the outlook for patients with systemic lupus is improving each decade with the development of more accurate monitoring tests and treatments. Individuals with systemic lupus can improve their prognosis by learning about the many aspects of the illness as well as closely monitoring their own health with their doctors.
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