| Handout on Health
Systemic Lupus Erythematosus This is a publication of the This publication is not copyrighted. Readers are encouraged to duplicate and distribute as many copies as needed. Printed copies of this booklet are available
from the Phone: (301) 495-4484 Systemic Lupus
Erythematosus Defining Lupus At present, there is no cure for lupus. However, the symptoms of lupus can be controlled with appropriate treatment, and most people with the disease can lead active, healthy lives. Lupus is characterized by periods of illness, called flares, and periods of wellness, or remission. Understanding how to prevent flares and how to treat them when they do occur helps people with lupus maintain better health. Intense research is underway and scientists funded by the NIH are continuing to make great strides in understanding the disease, which ultimately may lead to a cure. Two of the questions researchers are studying are who gets lupus and why. We know that many more women than men have lupus. Lupus is three times more common in black women than in white women and is also more common in women of Hispanic, Asian, and Native American descent. In addition, lupus can run in families, but the risk that a child or a brother or sister of a patient also will have lupus is still quite low. It is difficult to estimate how many people in the United States have the disease because its symptoms vary widely and its onset is often hard to pinpoint. Although "lupus" is used as a broad term, there actually are several kinds of lupus:
Understanding What Causes
Lupus The fact that lupus can run in families indicates that development of this disease has a genetic basis. In addition, studies of identical twins have shown that lupus is much more likely to affect both members of a pair of identical twins, who share the exact same set of genes, than two nonidentical twins or other siblings. Because the risk for identical twins is far less than 100 percent, however, scientists think that genes alone cannot account for who gets lupus. Other factors must also play a role. Some of the factors that scientists are studying include sunlight, stress, certain drugs, and infectious agents such as viruses. Even though a virus might trigger the disease in susceptible individuals, a person cannot "catch" lupus from someone else. In lupus, the body's immune system doesn't work as it should. A healthy immune system produces antibodies, which are special proteins that help fight and destroy viruses, bacteria, and other foreign substances that invade the body. In lupus, the immune system produces antibodies against the body's healthy cells and tissues. These antibodies, called autoantibodies ("auto" means self), contribute to the inflammation of various parts of the body, causing swelling, redness, heat, and pain. In addition, some autoantibodies join with substances from the body's own cells or tissues to form molecules called immune complexes. A buildup of these immune complexes in the body also contributes to inflammation and tissue injury in people with lupus. Researchers do not yet understand all of the factors that cause inflammation and tissue damage in lupus, and this is an active area of research. Symptoms of Lupus Other symptoms of lupus include chest pain, hair loss, sensitivity to the sun, anemia (a decrease in red blood cells), and pale or purple fingers and toes from cold and stress. Some people also experience headaches, dizziness, depression, or seizures. New symptoms may continue to appear years after the initial diagnosis, and different symptoms can occur at different times.
In some people with lupus, only one system of the body such as the skin or joints is affected. Other people experience symptoms in many parts of their body. Just how seriously a body system is affected also varies from person to person. Most commonly, joints and muscles are affected, causing arthritis and muscle pain. Skin rashes also are quite common. The following systems in the body also can be affected by lupus.
Diagnosing Lupus No single test can determine whether a person has lupus, but several laboratory tests may help the doctor to make a diagnosis. The most useful tests identify certain blood autoantibodies often present in people with lupus. For example, the antinuclear antibody (ANA) test is commonly used to look for autoantibodies that react against components of the nucleus, or "command center," of the patient's own cells. Many people with lupus test positive for ANA; however, some drugs, infections, and other diseases also can cause a positive result. The ANA test simply provides another clue for the doctor to consider in making a diagnosis. There are also blood tests for individual types of autoantibodies that are more specific to people with lupus, although not all people with lupus test positive for these. These antibodies include anti-DNA, anti-Sm, anti-RNP, anti-Ro (SSA), and anti-La (SSB). The doctor may use these antibody tests to help make a diagnosis of lupus. Some tests are used less frequently but may be helpful if the cause of a person's symptoms remains unclear. The doctor may order a biopsy of the skin or kidneys if those body systems are affected. Some doctors may order a syphilis test because some lupus antibodies in the blood may cause the test to be falsely positive. A positive test does not mean that a patient has syphilis. Again, all these tests merely serve as tools to give the doctor clues and information in making a diagnosis. The doctor will look at the entire picture - medical history, symptoms, and test results - to determine if a person has lupus. Other laboratory tests are used to monitor the progress of the disease once it has been diagnosed. A complete blood count (CBC), urinalysis, blood chemistries, and erythrocyte sedimentation rate (ESR) test can provide valuable information. (The ESR is a measure of inflammation in the body. It tests how quickly red blood cells drop to the bottom of a tube of unclotted blood.) Another common test measures the blood level of a group of proteins called complement. People with lupus often have low complement levels, especially during flares of the disease.
Treating Lupus The range and effectiveness of treatments for lupus have increased dramatically, giving doctors more choices in how to treat the disease. It is important for the patient to work closely with the doctor and take an active role in treatment. Once lupus has been diagnosed, the doctor will develop a treatment plan based on the patient's age, gender, health, symptoms, and lifestyle. Treatment plans are tailored to the individual's needs and may change over time. In developing a treatment plan, the doctor has several goals: to prevent flares, to treat them when they do occur, and to minimize complications. The doctor and patient should reevaluate the plan regularly to ensure that it is as effective as possible. Several types of drugs are used to treat lupus. The treatment the doctor chooses is based on the patient's individual symptoms and needs. For people with joint pain, fever, and swelling, drugs that decrease inflammation, referred to as nonsteroidal anti-inflammatory drugs (NSAIDs), are often used. While some NSAIDs are available over the counter, a doctor's prescription is necessary for others. NSAIDs may be used alone or in combination with other types of drugs to control pain, swelling, and fever. Even though some NSAIDs may be purchased without a prescription, it is important that they be taken under a doctor's direction because the dose for people with lupus may differ from the dose recommendations on the bottle. Common side effects of NSAIDs, including those available over the counter, can include stomach upset, heartburn, diarrhea, and fluid retention. Some lupus patients also develop liver and kidney inflammation while taking NSAIDs, making it especially important to stay in close contact with the doctor while taking these medications.
Antimalarials are another type of drug commonly used to treat lupus. These drugs were originally used to treat the symptoms of malaria, but doctors have found that they also are useful treatments for lupus. Exactly how antimalarials work in lupus is unclear, but scientists think that they may work by suppressing parts of the immune response. Specific antimalarials used to treat lupus include hydrochloroquine (Plaquenil), chloroquine (Aralen), and quinacrine (Atabrine). They may be used alone or in combination with other drugs and generally are used to treat fatigue, joint pain, skin rashes, and inflammation of the lungs. Research doctors have found that continuous treatment with antimalarials may prevent flares from recurring. Side effects of antimalarials can include stomach upset and, extremely rarely, damage to the retina of the eye. The mainstay of lupus treatment involves the use of corticosteroid hormones, such as prednisone (Deltasone), hydrocortisone, methylprednisolone (Medrol), and dexamethasone (Decadron, Hexadrol). Corticosteroids are related to cortisol, which is a natural anti-inflammatory hormone. They work by rapidly suppressing inflammation. Corticosteroids can be given by mouth, in creams applied to the skin, or by injection. Because they are potent drugs, the doctor will seek the lowest dose with the greatest benefit. Short-term side effects of corticosteroids include swelling, increased appetite, weight gain, and emotional ups and downs. These side effects generally stop when the drug is stopped. It can be dangerous to stop taking corticosteroids suddenly, so it is very important that the doctor and patient work together in changing the corticosteroid dose. Sometimes doctors give very large amounts of corticosteroid by vein ("bolus" or "pulse" therapy). With this treatment, the typical side effects are less likely and slow withdrawal is unnecessary. Long-term side effects of corticosteroids can include stretch marks on the skin, excessive hair growth, weakened or damaged bones, high blood pressure, damage to the arteries, high blood sugar, infections, and cataracts. Typically, the higher the dose of corticosteroids, the more severe the side effects. Also, the longer they are taken, the greater the risk of side effects. Researchers are working to develop alternative strategies to limit or offset the use of corticosteroids. For example, corticosteroids may be used in combination with other, less potent drugs, or the doctor may try to slowly decrease the dose once the disease is under control. People with lupus who are using corticosteroids should talk to their doctors about taking supplemental calcium and vitamin D to reduce the risk of osteoporosis (weakened, fragile bones). For patients whose kidneys or central nervous systems are affected by lupus, a type of drug called an immunosuppressive may be used. Immunosuppressives, such as azathioprine (Imuran) and cyclophosphamide (Cytoxan), restrain the overactive immune system by blocking the production of some immune cells and curbing the action of others. These drugs may be given by mouth or by infusion (dripping the drug into the vein through a small tube). Side effects may include nausea, vomiting, hair loss, bladder problems, decreased fertility, and increased risk of cancer and infection. The risk for side effects increases with the length of treatment. As with other treatments for lupus, there is a risk of relapse after the immunosuppressives have been stopped. In special circumstances, patients may require stronger drugs to combat the symptoms of lupus. For patients who cannot take corticosteroids, a type of immunosuppressive drug called methotrexate (Folex, Mexate, Rheumatrex) may be used to help control the disease. Patients who have many body systems affected by the disease may receive intravenous gamma globulin (Gammagard, Gammar, Gamine), a blood protein that increases immunity and helps fight infection. Gamma globulin also may be used to control acute bleeding in patients with thrombocytopenia or to prepare a person with lupus for surgery. Working closely with the doctor helps ensure that treatments for lupus are as successful as possible. Because some treatments may cause harmful side effects, it is important to promptly report any new symptoms to the doctor. It is also important not to stop or change treatments without talking to the doctor first. Because of the nature and cost of the medications used to treat lupus, their potentially serious side effects, and the lack of a cure, many patients seek other ways of treating the disease. Some alternative approaches that have been suggested include special diets, nutritional supplements, fish oils, ointments and creams, chiropractic treatment, and homeopathy. Although these methods may not be harmful in and of themselves, no research to date shows that they help. Some alternative or complementary approaches may help the patient cope or reduce some of the stress associated with living with a chronic illness. If the doctor feels the approach has value and will not be harmful, it can be incorporated into the patient's treatment plan. However, it is important not to neglect regular health care or treatment of serious symptoms. Lupus and Quality of
Life It is also important for people with lupus to receive regular health care, instead of seeking help only when symptoms worsen. Having a medical exam and lab work on a regular basis allows the doctor to note any changes and may help predict flares. The treatment plan, which is tailored to the individual's specific needs and circumstances, can be adjusted accordingly. If new symptoms are identified early, treatments may be more effective. Other concerns also can be addressed at regular checkups. The doctor can provide guidance about such issues as the use of sunscreens, stress reduction, and the importance of structured exercise and rest, as well as birth control and family planning. Because people with lupus can be more susceptible to infections, the doctor may recommend yearly influenza vaccinations for some patients.
People with lupus should receive regular preventive health care, such as gynecological and breast examinations. Regular dental care will help avoid potentially dangerous infections. If a person is taking corticosteroids or antimalarial medications, a yearly eye exam should be done to screen for and treat eye problems. Staying healthy requires extra effort and care for people with lupus, so it becomes especially important to develop strategies for maintaining wellness. Wellness involves close attention to the body, mind, and spirit. One of the primary goals of wellness for people with lupus is coping with the stress of having a chronic disorder. Effective stress management varies from person to person. Some approaches that may help include exercise, relaxation techniques such as meditation, and setting priorities for spending time and energy. Developing and maintaining a good support system is also important. A support system may include family, friends, medical professionals, community organizations, and organized support groups. Participating in a support group can provide emotional help, boost self-esteem and morale, and help develop or improve coping skills. (For more information on support groups, see the Additional Resources section at the end of this booklet). Learning more about lupus may also help. Studies have shown that patients who are well informed and participate actively in their own care experience less pain, make fewer visits to the doctor, build self confidence, and remain more active.
Pregnancy For Women With
Lupus Some women may experience a mild to moderate flare during or after their pregnancy; others do not. Pregnant women with lupus, especially those taking corticosteroids, also are more likely to develop high blood pressure, diabetes, hyperglycemia (high blood sugar), and kidney complications, so regular care and good nutrition during pregnancy is essential. It is also advisable to have access to a neonatal (newborn) intensive care unit at the time of delivery in case the baby requires special medical attention. About 25 percent (one in four) of babies of women with lupus are born prematurely, but do not suffer from birth defects. It is important to consider treatment options during pregnancy. The woman and her doctor must weigh the potential risks and benefits of each option to both mother and baby. Some drugs used to treat lupus should not be used at all during pregnancy because they may harm the baby or cause a miscarriage. A woman with lupus who becomes pregnant needs to work closely with both her obstetrician and her lupus doctor. They can work together to evaluate her individual needs and circumstances. The fear of miscarriage is very real for many pregnant women with lupus. Researchers have now identified two closely related lupus autoantibodies, anticardiolipin antibody and lupus anticoagulant (together called the antiphospholipid antibodies), that are associated with risk of miscarriage. One-third to one-half of women with lupus have these antibodies, which can be detected by blood tests. Identifying women with these antibodies early in the pregnancy may help doctors take steps to reduce the risk of miscarriage. Pregnant women who test positive for these antibodies and who have had previous miscarriages are generally treated with baby aspirin or the drug heparin throughout their pregnancy. In a small percentage of cases, babies of women who have specific antibodies called anti-Ro (SSA) and anti-La (SSB) have symptoms of lupus such as a rash or low blood count. This is not the same as systemic lupus erythematosus and is almost always temporary. Most babies with symptoms of neonatal lupus need no treatment at all. Current Research To help answer these questions, scientists are developing new and better ways to study the disease. They are doing laboratory studies that compare various aspects of the immune systems of people with lupus with those of other people both with and without lupus. They also use mice with disorders resembling lupus to explore how the immune system functions in the disease and to identify possible new therapies. The National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS), a component of the National Institutes of Health (NIH), funds many individual researchers across the United States who are studying lupus. To help scientists gain new knowledge, NIAMS also has established Specialized Centers of Research devoted specifically to lupus research. In addition, NIAMS is funding several lupus registries that will gather medical information as well as blood and tissue samples from patients and their relatives. This will give researchers across the country access to information and materials they can use to help identify genes that determine susceptibility to the disease. Identifying genes that play a role in the development of lupus is an active area of research. For example, researchers suspect a genetic defect in a cellular process called apoptosis, or "programmed cell death" in people with lupus. Apoptosis allows the body to safely get rid of damaged or potentially harmful cells. If there is a problem in the apoptosis process, harmful cells may stay around and do damage to the body's own tissues. For example, in a mutant mouse strain that develops a lupus-like illness, one of the genes that controls apoptosis, called the fas gene, is defective. When it is replaced with a normal fas gene, the mice no longer develop signs of the disease. Scientists are studying what role genes involved in apoptosis may play in human disease development. Studying genes for complement, a series of proteins in the blood that play an important part in the immune system, is another active area of lupus research. Complement acts as a backup for antibodies, helping them destroy foreign substances that invade the body. If there is a decrease in complement, the body is less able to fight or destroy foreign substances. If these substances are not removed from the body, the immune system may become overactive and begin to make autoantibodies. Research to identify genes that predispose some people to the more serious complications of lupus, such as kidney disease, is producing significant findings. NIAMS-supported researchers have identified a gene associated with an increased risk of lupus kidney disease in African Americans. Variations in this gene affect the immune systemıs ability to remove potentially harmful immune complexes from the body. Researchers are also making progress in identifying other genes that play a role in lupus. Researchers also are studying other factors that may affect a person's susceptibility to lupus. For example, because lupus is more common in women than in men some researchers are investigating the role of hormones and other male-female differences in the development and course of the disease.
A current study funded by the NIH is focusing on the safety and effectiveness of oral contraceptives (birth-control pills) and hormone replacement therapy in women with lupus. Doctors have worried about the wisdom of prescribing oral contraceptives or estrogen replacement therapy for women with lupus because of a widely held view that estrogens can make the disease worse. However, recent limited data suggest these drugs may be safe for some women with lupus. Researchers hope this study will yield options for safe, effective methods of birth control for young women with lupus and enable postmenopausal women with lupus to benefit from estrogen replacement therapy. Researchers are also focusing on finding better treatments for lupus. A primary goal of this research is to develop treatments that can effectively minimize the use of corticosteroids. Scientists are trying to identify combination therapies that may be more effective than single-treatment approaches. Researchers are also interested in using male hormones, called androgens, as a possible treatment for the disease. Another goal is to improve the treatment and management of lupus in the kidneys and central nervous system. For example, a 20-year study supported by NIAMS and NIH found that combining cyclophosphamide with prednisone helped delay or prevent kidney failure, a serious complication of lupus. On the basis of new information about the disease process, scientists are using novel "biologic agents" to selectively block parts of the immune system. Development and testing of these new drugs, which are based on compounds that occur naturally in the body, is an exciting and promising new area of lupus research. The hope is that these treatments not only will be effective but also will have fewer side effects. Other treatment options currently being explored include reconstructing the immune system by bone marrow transplantation. In the future, gene therapy also may play an important role in lupus treatment. Hope for the
Future Additional
Resources or your local chapter, listed in the telephone directory Web address: http://www.lupus.org/lupus This is the main voluntary organization devoted to lupus. The LFA assists local chapters in providing services to people with lupus, works to educate the public about lupus, and supports lupus research. Through a network of more than 500 branches and support groups, the chapters provide education through information and referral services, health fairs, newsletters, publications, and seminars. Chapters provide support to people with lupus, their families, and friends through support group meetings, hospital visits, and telephone help lines. Acknowledgments About NIAMS and NAMSIC National Arthritis and Musculoskeletal and Skin
Diseases The text of the booklet is also available 24 hours a day by fax. Using the phone on a fax machine, call NIAMS Fast Facts at (301) 881-2731. Listen to the instructions and dial 09601; the text will print to the fax machine. The National Arthritis and Musculoskeletal and Skin Diseases Information Clearinghouse (NAMSIC) is a public service sponsored by the NIAMS that provides health information and information sources. The NIAMS, a part of the National Institutes of Health (NIH), leads the Federal medical research effort in arthritis and musculoskeletal and skin diseases. The NIAMS supports research and research training throughout the United States as well as on the NIH campus in Bethesda, Maryland, and disseminates health and research information. Additional information and research updates can be found on the NIAMS Web site at http://www.nih.gov/niams/ | |||||||||||||