Systemic lupus erythematosus, also called lupus or SLE for short, belongs to the family of rheumatic diseases.

Rheumatic diseases affect joints, muscles and other parts of the body, and often involve abnormalities of the immune system. Lupus can affect not only joints and muscles but also skin, kidneys, nervous system, lungs, heart and the blood-forming organs.

Lupus is an autoimmune disease in which the tissues of the body are attacked by the overly active immune system and is treated by suppressing the immune system.

There are two common forms of lupus: discoid and systemic.

Discoid lupus is a disease of the skin which is often chronic and leads to scarring. It may be limited to the skin and not be associated with disease in any other body systems. In other cases, discoid lupus may be one of the features of the systemic form of lupus. This information focuses on the systemic form of the disease.

Lupus may be a mild disorder in some people, but for others it can lead to serious problems. Each person is different, and what is true about someone else's disease may not apply to you.

Lupus is a chronic, systemic, inflammatory disease. Chronic means it lasts a long time, probably for the rest of your life. However, nearly all people with lupus have fluctuations in disease activity known as flares and remissions. At times there may be no signs or symptoms of lupus at all (remissions). Some people have complete and long-lasting remissions. A systemic disease is one in which many different parts of the body may be affected. Inflammatory refers to a reaction of the body resulting in pain, heat, redness and swelling.

Although lupus is a chronic disease, this doesn't mean you can't live a full life. Chronic diseases can't be cured but they can be controlled through proper treatment.

Special concerns for people with lupus

People with lupus are more likely to develop infections. This is true partly because of the illness itself, and partly because of the side effects of medications, especially corticosteroids and immunosuppressive drugs. In some people with lupus, infections can trigger disease flares.

Exposure to the sun and fluorescent lighting is known to make the skin rashes associated with lupus worse in most people. Sun exposure may also cause generalized flares of lupus, resulting in fevers, joint pains, or even inflammation involving the heart, lungs, kidneys or nervous system. Of course, what is too much sun for one person may be fine for someone else. However, it's best to take simple precautions. These include regularly using a sunscreen lotion or sun block on your skin and avoiding outdoor activities during peak sunlight hours (usually 10 am to 4 pm). Sunscreens may be bought without a prescription, and are available in many different forms, some of which also include skin lubricants. Those with a sun protection factor of 15 or more are the best. Some chemicals in sunscreens may cause skin irritation or rashes, so it is important to try a different type of preparation if this occurs. During unavoidable times of sun exposure, it is important to wear a hat to shade you from direct sun and clothing to cover the arms, legs and chest.

Because of possible problems from sun exposure, you may wonder if it is safe to move to a warmer climate. As long as you protect yourself when you go out in the sun, your illness should not affect where you live.

Fertility and pregnancy

Pregnancy may mean special problems for women with lupus. Fertility, or the ability to conceive, may be decreased during periods of disease flares. This may be the result of hormonal changes caused by lupus or from side effects of medications used to treat lupus. Women may experience lupus for the first time or a worsening of their symptoms during pregnancy or several weeks to months after delivery.

Women with lupus have an increased chance of having a miscarriage. This can occur either early or late in the pregnancy. Certain abnormal antibodies present in the blood of some women with lupus may contribute to the chance of a miscarriage. Therefore it is important that you discuss your plan to become pregnant with your doctor, and that you are seen regularly by your doctor during the pregnancy. Blood tests to detect the presence of the abnormal antibodies and to measure other signs of lupus activity should be done regularly. With these precautions, many women with lupus can have normal pregnancies.

Occasionally, newborn babies of mothers with lupus have a mild illness caused by transfer of the mother's antibodies through the placenta to the baby. This illness may include a rash, low blood counts, or an enlarged liver. These features go away, generally by six months after birth. Rarely, babies may have a permanent problem called congenital heart block, which causes a slow heart beat. This may require treatment with a pacemaker.

If your doctor advises against pregnancy, or if you do not wish to have children, the safest method of contraception for women with lupus is the diaphragm used with contraceptive jelly. Some women with lupus are able to use birth control pills safely, either combination pills with low dose estrogen and progesterone or progesterone only (minipill) preparations. Intrauterine devices (IUD's) are not advisable because of the risk of infections connected with their use.

Incidence

Symptoms

There is no uniform pattern of symptoms at the onset of lupus.

Fever, weakness, fatigue, or weight loss may be among the first signs of illness. A skin rash may appear on the face, neck, or arms. When this rash involves the nose and cheeks, it is called a butterfly rash. In people who are very sensitive to ultraviolet light, the rash may appear or get worse after exposure to the sun. Sometimes the fingers are unusually sensitive to cold and will turn blue on exposure. This color change is called Raynaud's phenomenon.

Another early sign of lupus may be joint pain in the hands, wrists, elbows, knees or ankles. Although the joints may become red, warm or swollen, the development of deformities is very uncommon. A feeling of stiffness in the joints and muscles upon awakening in the morning may accompany the joint pains, or may even occur without joint pain. Other common symptoms of the illness include muscle aches, swollen glands, lack of appetite, low grade temperature, hair loss, and nausea and vomiting.

There may be an increased tendency to get infections or to bleed easily. You may develop anemia. This is a condition in which one type of blood cell may be decreased in number, causing weakness and paleness, or even shortness of breath. Some people with lupus may have an increased tendency to form blood clots.

Other frequent symptoms include inflammation of the lining of some body parts (for example, the heart and lungs) causing symptoms of pain on breathing or shortness of breath. Kidney problems commonly occur. In the early stages, there may be no symptoms of kidney involvement, although edema, a swelling of the legs, may occur if there is a leak of protein into the urine.

Sometimes people with lupus experience depression or inability to concentrate. Rapid changes in mood or abnormal behavior may occur. These changes may be due to the disease itself, or they may be a reaction to the changes in appearance and energy caused by the disease. In a very small percentage of people, more severe nerve and mental problems, especially convulsions like those in epilepsy, may develop, but they rarely persist.

The doctor will watch you closely for any signs of these problems, and treatment will be started quickly if they occur. Remember that lupus can take many forms. You may have only a few of these problems. Many people with lupus do very well without having major problems.

Conditions with similar symptoms

Causes

We don't know the cause of lupus. Doctors and scientists agree that it is an autoimmune disease. The immune system is your body's natural defense against disease. "Auto" means self. Thus, an autoimmune disease is one that is directed against the body's own tissue.

Under normal conditions, when harmful bacteria invade the body, protective substances in your blood called antibodies develop to fight off the bacteria. In lupus, antibodies may be formed even without the presence of foreign substances like bacteria. These antibodies are called autoantibodies because they attack the body's own tissues. This in turn causes inflammation and injury to body tissues and organs, and may result in the symptoms that people with lupus experience. What triggers the chain of events that leads to this abnormal autoimmune reaction is unknown.

Some studies suggest that certain people may inherit the tendency to get lupus. This conclusion stems from the fact that some new cases of lupus may be more common in a family in which one member already has the disease. However there is no evidence that lupus is directly passed on, for example, from mother to daughter. Some scientists believe that perhaps a virus may trigger the genetic tendency to develop lupus and bring on the symptoms of the disease.

Diagnosis

It is sometimes difficult to diagnose lupus, particularly in people with mild symptoms of the disease.

Your doctor will ask you many questions and do a complete physical examination. Next, certain laboratory tests will be performed. For example, a blood count is done to see if you have too few red cells, white cells, or platelets, blood cells that help to control bleeding and clotting.

Diagnostic tests

If your doctor thinks you may have lupus, a blood test will be done to determine if you have a type of antibody that is found in the blood of almost all people with lupus. This is called an antinuclear antibody or ANA. Other blood tests may be done for diagnosis and as an aid in following disease activity. Blood chemistry tests will help to determine whether organs such as the kidneys and liver are functioning normally.

Because kidney problems often occur, you may have a urinalysis, an examination of your urine. You may be asked to collect all the urine you pass in a 24-hour period so that kidney function tests can be done. If kidney involvement is suspected, you may have a biopsy performed, during which a small piece of tissue from one of your kidneys is removed and examined with special tests.

A chest X-ray may be taken to see whether the lungs or heart are involved. An electrocardiogram and echocardiogram may also be done to help determine if there is any heart involvement.

The treatment program for lupus includes taking medications, getting rest when the disease is active, and being careful about sun exposure.

Lupus is an unpredictable disease. Signs of the disease appear and disappear, sometimes for no apparent reason. Because lupus assumes so many different forms, finding the right balance of treatment for you may take time--but it is very important.

Once an effective treatment program has been started, continue it faithfully. If your symptoms change, let your doctor know so that you can work together to modify your program.

If you tire easily, you need a balance of rest and activity. Part of this balancing includes pacing yourself during the day, and also from day to day. Allow plenty of time to finish the things you start, so you won't feel rushed. Don't try to do too much at one time. Be realistic. Scheduling and pacing also includes doing the hardest things when you're feeling your best.

It's usually not necessary to give up your normal activities. However, you may need to limit tiring activities, especially if you feel a flare coming on. A flare is a period during which disease symptoms appear or become worse. During a flare, don't hesitate to ask others in the family to help out more at home. You might consider getting someone to come in to help you with housework.

While on the job, try to take short breaks and alternate activities. You might need to cut down on the number of hours you work. If cutting down your hours presents a problem with your current job, a vocational rehabilitation counselor may be able to help you get a job which allows more flexible hours or is less physically demanding.

Diet

Eating a balanced diet is an important part of the treatment plan. At times when your lupus is active or your appetite is poor, it may be helpful to take a daily multivitamin. Your doctor may prescribe special vitamins such as folic acid for specific lupus problems. It is important to remember, however, that excessive doses of vitamins can have serious side effects. You should be sure that your doctor is aware of any vitamin preparations you take.

At times you may be placed on a special diet because of problems caused by features of your lupus such as kidney disease. A low salt diet will help to prevent the accumulation of edema fluid. When the kidneys do not function normally, it may be necessary to limit the amount of protein in the diet.

Some scientists have suggested that diets rich in fish oil may have a beneficial effect on the course of lupus by preventing inflammation from developing. Clinical trials of fish oil in people with lupus are still in the early stages.

Exercise and therapy

It is equally important to do proper exercises on a daily basis. This may be easier to do when the disease is not in an active stage and you are feeling better. However, even during a flare, doing gentle range of motion and strengthening exercises is important to keep you from getting stiff. Exercise can also help you avoid muscle weakness. Working under your doctor's guidance, a physical therapist can help you set up an exercise program that fits your schedule and physical condition.

Medications

Medication is a necessary part of treatment for most people with lupus.

Because of the changing character of lupus, the type and amount of drugs may be changed often. Changing how much medicine you take or how often you take it is a decision to be made only after talking with your doctor, however. Changing the schedule on your own, or not taking any drug your doctor has prescribed, could have harmful effects. It will also make it more difficult for your doctor to evaluate the course of your disease and the effectiveness of the medication. If you start taking a drug without medical advice, it could cause you harm, either by interacting with other medications prescribed for you, or by causing side effects which might even mimic some of the symptoms of your lupus.

Aspirin and other anti-inflammatory drugs

Aspirin is sometimes the only medication the doctor will prescribe. Because aspirin is commonly used for minor problems, you may not think it is special enough. However, aspirin is more than a painkiller. It is also an anti-inflammatory drug, which makes it helpful in treating the joint discomfort of lupus. But it can only do its job if you take it exactly as directed. This may mean taking it at regular internals, and often in large doses.

Some people are bothered by stomach problems when taking high doses of aspirin. One way to lessen this problem is to take the tablets with a meal or with milk. You might try using enteric-coated aspirin tablets. These do not dissolve until they have passed through the stomach. Another approach is to take antacids about one-half hour after meals and at bedtime to help protect the lining of your stomach.

Non-steroidal anti-inflammatory drugs (NSAIDs) have properties similar to aspirin. Some examples of brand name NSAIDs are Clinoril, Feldene, Indocin, Meclomen, Motrin, Rufen, Nalfon, Naprosyn, Orudis and Tolectin. In addition to these prescription-only medications, ibuprofen may be obtained over-the-counter. For some people, these drugs may be more effective or better tolerated than aspirin. They all have the potential for causing stomach irritation, and some may cause specific side effects such as dizziness or diarrhea. Some NSAIDs are quite long-acting, which may be an important consideration in finding a regimen that compliments your lifestyle.

Aspirin and most NSAIDs can damage the liver. NSAIDs may also cause an alteration in kidney function. Usually, however, the damage can be reversed when the medication is stopped or given in smaller doses.

Antimalarial drugs

Some doctors prescribe drugs similar to quinine which are used to treat malaria. The most commonly used antimalarial drug in lupus is Plaquenil. There is no known relationship between lupus and malaria, and no one knows just how the antimalarial drugs work in lupus. They are, however, helpful in treating discoid lupus and some of the manifestations of systemic lupus, especially fever, joint pains and pleuritis (inflammation of the lining of the lungs). These drugs increase resistance to sun exposure and can help to prevent lupus rashes and systemic problems if you are exposed to ultraviolet light. The most common side effects are mild nausea, vomiting and diarrhea.

Although it is rare, sometimes antimalarial drugs can affect your eyes if taken in large doses for a long time. If you are taking these drugs, you need to have regular eye examinations by an ophthalmologist, a medical doctor specializing in eye care. If you notice any changes in vision such as blurring or blind spots, contact your doctor right away for advice.

Corticosteroids

Corticosteroids, or cortisone-like drugs, are commonly used in the treatment of severe lupus. They are synthetic forms of hormones naturally produced by your adrenal glands, which are located on top of your kidneys.

Corticosteroids are the strongest anti-inflammatory drugs available. They can dramatically reduce pain and inflammation in a matter of hours. They also can control many of the signs and symptoms of lupus.

It is common for a doctor to prescribe high doses of corticosteroids when your lupus is very active, and especially when it affects your kidneys or nervous system. Pulse steroids may be prescribed at such times. This means that a very high dose of steroid is given directly into the veins, usually every day for a three-day period. As the symptoms and abnormal laboratory tests improve, the steroid dose is usually tapered slowly over a period of months. For some people, an alternate, or every other day, dose of steroids may be prescribed. This method of taking steroids may help to decrease some of the side effects of the drug. If you have skin rashes, you may be given creams or ointments containing corticosteroids.

Common side effects of the cortisone-like drugs include weight gain, rounding of the face and easy bruising. Large doses of corticosteroids may cause mood swings from extreme nervousness and insomnia to depression. Other serious effects include the development of fluid retention with swelling of the legs and high blood pressure, the triggering or worsening of diabetes, an increase in the risk of infection and rarely, bleeding from the stomach. Use of corticosteroids over a long period of time may lead to thinning of the bones (osteoporosis), and to cataracts.

If you are taking these powerful drugs, be sure to inform any doctor or dentist before you have surgery or undergo any other type of stressful procedure. Your body may need more steroids at such times.

NEVER change your dose of corticosteroids without first discussing it with your doctor. Stopping them or changing the dose quickly may make you very ill.

Immunosuppressive drugs

Immunosuppressive drugs are used to treat autoimmune diseases. They weaken the body cells that produce the immune and inflammatory responses. Immunosuppressive drugs are used only with active disease, especially with severe kidney problems. They are almost always taken along with corticosteroids, to treat people who have not responded to other types of drug therapy. They may also be used to help in cutting down the dose of corticosteroids.

The most commonly used immunosuppressive drugs are Imuran and Cytoxan. Chlorambucil, Methotrexate and a new immunosuppressive drug Cyclosporine, which is also given to people with kidney transplants, may be occasionally prescribed.

These drugs can have serious side effects. If you are taking an immunosuppressive, regular blood tests will be done because the drug can interfere with the formation of blood cells. The development of infection is also more likely when you are taking an immunosuppressive, so it is important to notify your doctor if you develop a fever or any other new symptoms.

Sometimes, even with the use of corticosteroids and immunosuppressive drugs, the kidneys fail. Fortunately, dialysis is available to cleanse waste products from the blood. The most common form of dialysis is hemodialysis, in which catheters are placed in an artery and vein, and the blood is run through a cleansing machine.

When kidney failure is permanent, kidney transplantation may be performed. This procedure can often allow a person with lupus to return to a normal lifestyle, even though medication is generally required to prevent rejection of the new kidney.

Strategies for coping

There's no question that social and emotional problems often come with having a chronic disease.

However, not everyone experiences them to the same degree, and there can be long periods of time when you aren't bothered at all by the disease.

There may also be times when you feel angry and depressed over the loss of your former good health. You may be in pain, and there may be changes in your appearance. None of these things are easy to accept.

Talking about your fears, anger and depression usually helps. At times it may be hard to confide in family or friends or in your doctor. The chance to talk about problems and frustrations with others who have lupus can be just what you need to improve your spirits, and it can help you to find solutions to your problems too. It is important to remember, though, that you may never experience some of the problems you learn about in others with lupus. In some areas, people who have lupus have formed clubs or support groups. The groups are an effort by these people to help themselves through a better understanding of their disease.

Most support groups have regular meetings and often a medical specialist is invited to give a talk. A very important aspect of these groups is the opportunity to share experiences with others who have lupus. Meetings give everyone a chance to express their feelings, and to learn from listening about how others cope. Many practical tips about daily activities are also exchanged. Family members also have a lot to gain from such sessions. If they can understand your problems and your feelings better, they can be more sympathetic and helpful.

There may be times when you feel overwhelmed. At such times counseling may be helpful. Your doctor or the Lupus Foundation of America can give you suggestions about social workers and other professionals and agencies who may be able to offer their support.

Condition research

Scientific and clinical research slowly but continually provide us with a clearer understanding of lupus. Better tests have been found to help diagnose it, and many improvements have been made in treatment. This new knowledge has led to increased life expectancy and improved quality of life for most people with lupus.

Lupus research is a worldwide effort. There is every reason to believe that there will be even more progress to look forward to in the future.

Credits

Some of this material may also be available in an Arthritis Foundation brochure. Contact the Washington/Alaska Chapter Helpline: (800) 542-0295. If dialing from outside of WA and AK, contact the National Helpline: (800) 283-7800.

Adapted from the pamphlet originally prepared for the Arthritis Foundation by Ellen M. Ginzler, M.D. This material is protected by copyright.