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Lupus
Systemic Lupus Erythematosus
This booklet is for people who have systemic lupus erythematosus, commonly called SLE or lupus, as well as for their family and friends and others who want to better understand the disease. The booklet describes the disease and its symptoms and contains information about diagnosis and treatment as well as current research efforts supported by the National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS) and other components of the National Institutes of Health (NIH). It also discusses issues such as health care, pregnancy, and quality of life for people with lupus. If you have further questions after reading this booklet, you may wish to discuss them with your doctor.
Defining Lupus
Lupus is a type of immune system disorder known as an autoimmune disease. In autoimmune diseases, the body harms its own healthy cells and tissues. This leads to inflammation and damage of various body tissues. Lupus can affect many parts of the body, including the joints, skin, kidneys, heart, lungs, blood vessels, and brain. Although people with the disease may have many different symptoms, some of the most common ones include extreme fatigue, painful or swollen joints (arthritis), unexplained fever, skin rashes, and kidney problems. Lupus is also known as a rheumatic disease. The rheumatic diseases are a group of disorders that cause aches, pain, and stiffness in the joints, muscles, and bones.
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:
Systemic lupus erythematosus (SLE), which is the form of the disease that most people are referring to when they say "lupus." The word "systemic" means the disease can affect many parts of the body. The symptoms of SLE may be mild or serious. Although SLE usually first affects people between the ages of 15 and 45 years, it can occur in childhood or later in life as well. This booklet focuses on SLE.
Discoid lupus erythematosus primarily affects the skin. A red, raised rash may appear on the face, scalp, or elsewhere. The raised areas may become thick and scaly. The rash may last for days or years and may recur. A small percentage of people with discoid lupus later develop SLE.
Drug-induced lupus refers to a form of lupus caused by medication. It causes some symptoms similar to those of SLE (arthritis, rash, fever, and chest pain, but not kidney disease) that go away when the drug is stopped. Common medications that may cause drug-induced lupus include hydralazine (Apresoline), procainamide (Procan, Pronestyl), methyldopa (Aldomet), quinidine (Quinaglute), isoniazid (INH), and some anti-seizure medications such as phenytoin (Dilantin) or carbamazepine (Tegretol).
Neonatal lupus can affect some newborn babies of women with SLE or certain other immune system disorders. Babies with neonatal lupus may have a serious heart defect. Other affected babies may have a skin rash, liver abnormalities, or low blood counts. Physicians can now identify most at-risk SLE patients, allowing for prompt treatment of the infant at birth. Neonatal lupus is very rare, and most infants of mothers with SLE are entirely healthy.
Understanding What Causes Lupus
Lupus is a complex disease whose cause is unknown. It is likely that there is no single cause but rather a combination of genetic, environmental, and possibly hormonal factors that work together to cause the disease. The exact cause may differ from one person to another. Scientists are making progress in understanding the causes of lupus, as described here and in the Current Research section of this booklet. Research suggests that genetics plays an important role; however, no specific "lupus gene" has been identified. Instead, it appears that several genes may increase a person's susceptibility to the disease.
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
Each person's experience with lupus is different. Symptoms can range from mild to severe and may come and go over time. Common symptoms of lupus include extreme fatigue, painful or swollen joints, unexplained fever, and skin rashes. A characteristic skin rash may appear across the nose and cheeks - the so-called butterfly or malar rash. Other rashes occur elsewhere on the face and ears, upper arms, shoulders, chest, and hands.
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.
Common Symptoms of Lupus
Painful or swollen joints and muscle pain
Unexplained fever
Extreme fatigue
Red rash or color change on the face
Chest pain upon deep breathing
Unusual loss of hair
Pale or purple fingers or toes from cold or stress (Raynaud's phenomenon)
Sensitivity to the sun
Swelling (edema) in legs or around eyes
Swollen glands
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.
Kidneys:
Inflammation of the kidneys (nephritis) can impair their ability to effectively get rid of waste products and other toxins from the body. Because the kidneys are so important to overall health, lupus in the kidneys generally requires intensive drug treatment to prevent permanent damage. There is usually no pain associated with kidney involvement, although some patients may notice that their ankles swell. Most often the only indication of kidney disease is an abnormal urine test.
Central nervous system:
In some patients, lupus affects the brain or central nervous system. This can cause headaches, dizziness, memory disturbances, vision problems, stroke, or changes in behavior. Some of these symptoms, however, also can be caused by some treatments of lupus or by the emotional stress of dealing with the disease.
Blood vessels:
Blood vessels may become inflamed (vasculitis), affecting the way blood circulates through the body. The inflammation may be mild, and may not require treatment.
Blood:
People with lupus may develop anemia or leukopenia (a decreased number of white blood cells). Lupus also may cause thrombocytopenia, a decreased number of platelets in the blood that contributes to an increased chance of bleeding. Some people with lupus may have an increased risk for blood clots.
Lungs:
Some people with lupus develop pleuritis, an inflammation of the lining of the chest cavity that causes chest pain, particularly with breathing. Patients with lupus also may get pneumonia.
Heart:
In some people with lupus, inflammation can occur in the arteries that supply blood to the heart (coronary vasculitis), the heart itself (myocarditis and endocarditis), or the membrane that surrounds it (pericarditis), causing chest pains or other symptoms.
Diagnosing Lupus
Diagnosing lupus can be difficult. It may take months or even years for doctors to piece together the symptoms to accurately diagnose this complex disease. Making a correct diagnosis of lupus requires knowledge and awareness on the part of the doctor and good communication on the part of the patient. Telling the doctor a complete, accurate medical history (for example, what health problems you have had and for how long) is critical to the process of diagnosis. This information, along with a physical examination and the results of laboratory tests, helps the doctor consider other diseases that may mimic lupus, or determine if the patient truly has the disease. Reaching a diagnosis may take time and occur gradually as new symptoms appear.
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.
Diagnostic Tools for Lupus
Medical history
Complete physical examination
Skin or kidney biopsy
Laboratory tests:
Complete blood count
Erythrocyte sedimentation rate (ESR) - an elevated ESR indicates inflammation in the body
Urinalysis
Blood chemistries
Complement levels - often low in people with lupus, especially during a flare
Antinuclear antibody test (ANA) - positive in most lupus patients, but a positive ANA test can have other causes
Other autoantibody tests (anti-DNA, anti-Sm, anti-RNP, anti-Ro [SSA], anti- La [SSB]): One or more of these tests may be positive in some people with lupus
Syphilis test - may be falsely positive in people with lupus
Treating Lupus
Diagnosing and treating lupus is often a team effort between the patient and several types of health care professionals. A person can go to his or her family doctor or internist, or can visit a rheumatologist. A rheumatologist is a doctor who specializes in arthritis and other diseases of the joints, bones, and muscles. Clinical immunologists (doctors specializing in immune system disorders) may also treat people with lupus. As treatment progresses, other professionals often help. These may include nurses, psychologists, social workers, and specialists such as nephrologists (doctors who treat kidney disease), hematologists (doctors specializing in blood disorders), dermatologists (doctors who treat skin disease), and neurologists (doctors specializing in disorders of the nervous system).
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.
NSAIDs Used To Treat Lupus*
Generic Name
Ibuprofen
Naproxen
Sulindac
Diclofenac
Piroxicam
Ketoprofen
Diflunisal
Nabumetone
Etodolac
Oxaprozin
Indomethacin
Brand Name
Motrin, Advil
Naprosyn, Aleve
Clinoril
Voltaren
Feldene
Orudis
Dolobid
Relafen
Lodine
Daypro
Indocin

* Brand names included in this fact sheet are provided as examples only and their inclusion does not mean that these products are endorsed by the National Institutes of Health or any other Government agency. Also, if a particular brand name is not mentioned, this does not mean or imply that the product is unsatisfactory.
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
Despite the symptoms of lupus and the potential side effects of treatment, people with lupus can maintain a high quality of life overall. One key to managing lupus is to understand the disease and its impact. Learning to recognize the warning signs of a flare can help the patient take steps to ward it off or reduce its intensity. Many people with lupus experience increased fatigue, pain, a rash, fever, stomach discomfort, headache, or dizziness just before a flare. Developing strategies to prevent flares can also be helpful, such as limiting exposure to the sun (intense sun exposure triggers flares in some patients) and scheduling adequate rest and quiet times.
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.
Warning Signs of a Flare
Increased fatigue
Pain
Rash
Fever
Stomach discomfort
Headache
Dizziness
Preventing a Flare
Learn to recognize warning signals
Maintain good communication with your doctor
Set realistic goals and priorities
Limit exposure to the sun
Maintain a healthy, balanced diet
Try to limit stress
Schedule adequate rest and quiet times
Participate in moderate exercise when possible
Develop a support system
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.
Tips for Working With Your Doctor
Find a doctor who will listen to and address your concerns.
Provide complete, accurate medical information.
Make a list of your questions and concerns in advance.
Be honest and share your point of view with the doctor.
Ask for clarification or further explanation if you need it.
Talk to other members of the health care team, such as nurses, therapists, or pharmacists.
Don't hesitate to discuss sensitive subjects (for example, birth control, sex) with your doctor.
Discuss any treatment changes with your doctor before making them.