Lupus (Systemic Lupus Erythematosus)

Topic Overview

What is systemic lupus erythematosus, or lupus?

Lupus is an autoimmune disease, which means that the body's natural defense system (immune system) attacks its own tissues instead of attacking foreign substances like bacteria and viruses. This causes inflammation. Inflammation causes swelling, pain, and tissue damage throughout the body. If you develop severe lupus, you may have problems with your kidneys, heart, lungs, nervous system, or blood cells. Lupus is the common name for systemic lupus erythematosus, also called SLE.

Although some people with lupus have only mild symptoms, the disease is lifelong and can become severe. But most people can control their symptoms and prevent severe damage to their organs. They do this by seeing their doctors often for checkups, getting enough rest and exercise, and taking medicines.

This topic focuses on systemic lupus erythematosus (SLE), the most common and most serious type of lupus. But there are four other types of lupus: discoid or cutaneous lupus, drug-induced systemic lupus, neonatal lupus, and subacute cutaneous lupus.

What causes lupus?

The exact cause of lupus is not known. Experts believe that some people are born with certain genes that affect how the immune system works and that they are more likely to get lupus. Then a number of other factors can trigger lupus attacks. These include viral infections, including the virus that causes mononucleosis, and sunlight.

Although these things can trigger lupus, they may affect one person but not another person.

What are the symptoms?

Lupus symptoms vary widely, and they come and go. The times when symptoms get worse are called relapses, or flares. The times when symptoms are not so bad are called remissions.

Common symptoms include feeling very tired and having joint pain or swelling (arthritis), a fever, and a skin rash . The rash often happens after you have been in the sun. Mouth sores and hair loss may occur. Over time, some people with lupus have problems with the heart, lungs, kidneys, blood cells, or nervous system.

How is lupus diagnosed?

There is no single test for lupus. Because lupus affects different people in different ways, it can be hard to diagnose.

Your doctor will check for lupus by examining you, asking you questions about common symptoms, and doing some blood tests. It is easier for your doctor to diagnose lupus if you have the most common symptoms and your blood has certain proteins. These proteins are called antinuclear antibodies, or ANAs. But other problems can cause your body to make ANAs, so doctors will use blood tests and other tests to find out if you have lupus.

How is it treated?

Lupus is treated by:

• Applying corticosteroid cream for rashes.
• Taking nonsteroidal anti-inflammatory drugs (NSAIDs) for mild joint or muscle pain and fever.
• Taking antimalarial medicines to treat fatigue, joint pain, skin rashes, and lung inflammation.
• Taking corticosteroids if other medicines are not controlling your symptoms.

Because corticosteroids are powerful medicines and can cause serious side effects, the doctor will recommend the lowest dose that will give the most benefit.

The doctor may also recommend other medicines that slow down the immune system.

How can you manage lupus?

One of the goals of controlling mild to moderate lupus symptoms is to prevent flares, the times when your symptoms are worse. Some of the other things you can do include:

• Rest to reduce stress.
• Avoid the sun. Wear sunscreen and protective clothing when you are outside.
• Exercise regularly to prevent fatigue and joint stiffness.
• Stop smoking.
• Learn the warning signs of a symptoms flare, such as fatigue, pain, and rash, and take steps to control them.

With good self-care, most people with lupus can keep doing their regular daily activities.

It is important to learn about lupus so that you can understand how it might affect your life and how you can best cope with it. Also, help your family and friends understand your limitations and needs when your symptoms flare. Develop a support system of family, friends, and health professionals.

Frequently Asked Questions

Cause

Lupus (systemic lupus erythematosus, or SLE) is an autoimmune disease, in which the immune system attacks the body's own tissues as though they were foreign substances. Lupus is not contagious.

No one knows exactly what causes the body to attack its own tissues. A person may be born with a certain genetic makeup that affects how the immune system functions or makes him or her at risk for lupus. A combination of factors can trigger the autoimmune process, some of which may affect one person but not another.¹

• Exposure to ultraviolet light, usually from sunlight, is known to trigger the disease process and symptom flares.
• Hormonal factors are linked to autoimmune disease, though the link is poorly understood. Some research shows an increased risk of lupus with higher levels of estrogen. But a separate study did not find evidence that the hormones estrogen or prolactin taken for hormone replacement therapy or birth control, or taken previously for fertility treatments, increased the risk of lupus.² Another study showed that women with stable lupus had no increased risk of symptom flares from taking birth control pills.³
• Smoking may increase the risk of getting lupus and may make the disease more severe.
• Some medications are suspected triggers of lupus and symptom flares.
• Some infections are suspected triggers. Some people who have cytomegalovirus (CMV), parvovirus (such as fifth disease), and hepatitis C infections eventually develop lupus. The Epstein-Barr virus has been linked to lupus in children.
• Chemical exposure has been known to trigger lupus. Suspected chemical toxins include trichloroethylene in well water and silica dust. Hair dyes and straighteners, linked to lupus in the past, are no longer considered to be lupus triggers.

Symptoms

If you have lupus (systemic lupus erythematosus, or SLE), you may be extremely tired, have skin rashes, or have joint pain. If the disease is more serious, you may have problems with your kidneys, heart, lungs, blood, or nervous system.

Lupus symptoms depend on what body organs are affected and how seriously they are affected.

• Fatigue: Nearly all people with lupus have mild to extreme fatigue. Even mild cases of lupus cause an inability to engage in daily activities and exercise. Increased fatigue is a classic sign that a symptom flare is about to occur.
• Joint and muscle pain: Most people with lupus have joint pain (arthritis) at some time. About 70% of people with lupus report that joint and muscle pain was their first sign of the disease. Joints may be red and warm, and may swell. Morning stiffness may also be felt. Lupus arthritis often occurs on both sides of the body at the same time, particularly in the wrists, small joints of the hands, elbows, knees, and ankles.
• Skin problems: Most people with lupus develop skin rashes. These rashes are often an important clue to the diagnosis. In addition to the butterfly rash over the cheeks and bridge of the nose, other common skin symptoms include skin sores or flaky red spots on the arms, hands, face, neck, or back; mouth or lip sores; and a scaly, red or purple raised rash on the face, neck, scalp, ears, arms, and chest.
• Sensitivity to light: Exposure to ultraviolet light (such as sunlight or tanning parlors) typically worsens the skin rash and can trigger lupus flares. Sensitivity to light affects many of those with lupus, with fair-skinned people with lupus tending to be more sensitive.
• Nervous system symptoms: The majority of people with lupus develop nervous system problems, most commonly headaches, depression, or anxiety. Memory loss is less common.
• Heart problems: People with lupus may develop inflammation of the heart sac (pericarditis), which may cause severe, sudden pain in the center of the left side of the chest that may spread to the neck, back, shoulders, or arms.
• Mental health problems: People with lupus may develop problems such as anxiety and depression. Such problems can be caused by lupus, the medications used to treat it, or the stress of coping with chronic illness.
• Fever: Most people with lupus will sometimes have a low-grade fever related to the disease. Fever is sometimes a first sign of the disease.
• Changes in weight: Many people with lupus lose weight when their disease is active (flaring).
• Hair loss: People with lupus may experience periods of hair loss, either in patches or spread evenly over the head. This hair loss is usually not permanent.
• Swollen glands: Many people with lupus eventually develop swollen lymph glands during a flare.
• Raynaud's phenomenon: Some people with lupus have this condition. It affects the small vessels that supply blood to the skin and the soft tissues under the skin of the fingers and toes, causing them to turn white and/or blue or red. The skin affected will feel numb, tingly, and cold to the touch.
• Inflammation of blood vessels in the skin (cutaneous vasculitis): Inflammation or bleeding from the blood vessels can lead to small or large blue spots or small reddish spots on the skin or nail beds.
• Swelling of the hands and feet: Some people with lupus have kidney problems, which can prevent extra fluids from being removed from the body tissues. As fluid collects, the hands and feet may swell.
• Anemia: Anemia is a decrease in the amount of the oxygen-carrying substance (hemoglobin) found in red blood cells. Many people with an ongoing disease such as lupus develop anemia because they don't have enough red blood cells.

There are other conditions with symptoms similar to lupus.

What Happens

The outlook for people with lupus has improved as better treatments have been found. Now, nearly 70% of people with lupus live 20 years or more after they are diagnosed with the condition.⁴

The course of lupus varies by individual and is hard to predict because symptoms come and go. Lupus usually develops so slowly that a person may not notice the symptoms for a long time.

Periods of time when you have lupus symptoms are called flares or relapses. Periods of time when your symptoms get better are called remissions. On occasion, lupus develops and progresses rapidly. Flares and remissions can occur abruptly, unexpectedly, and without clear cause. There is no way to predict when a flare will happen, how bad it will be, or how long it will last. When you have a lupus flare, you may have new symptoms in addition to those you have had in the past.

Children can get lupus, though it more commonly develops in the teen years or later. Lupus in children appears to be more severe than in adults when vital organs, such as the kidneys and heart, are involved. This may be due to age-related differences in the disease, a child's stage of development, or differences in access to treatment.

People with lupus commonly lead a less active lifestyle than do people who do not have lupus, due to the fatigue, joint pain, and decreased aerobic capacity caused by the disease.^{5, 6} Aerobic capacity is the ability to do exercise such as walking and swimming that pumps oxygen to your heart and muscles.

Some people with lupus develop complications such as:

• Pregnancy problems.
• Kidney problems.
• Heart problems.
• Lung problems.
• Blood problems.
• Nervous system problems.
• Mental health problems.

Living with lupus

Most people with lupus are able to continue their usual daily activities. You may find that you need to cut back on your activity level, get help with child care, or change the way you work because of fatigue, joint pain, or other symptoms. You may find that you have to take time off from daily activities entirely.

Most people with lupus can expect to live a normal or near-normal life span. This depends on how severe your disease is, whether it affects vital organs (such as the kidneys), and how severely these organs are affected.

Lupus usually does not cause joint damage or deformity, which may happen in people who have rheumatoid arthritis, another autoimmune disease.

Medications used to treat moderate to severe lupus have side effects. It can be difficult to tell what problems are part of the natural course of the disease and what problems are due to effects of medications used to control the disease.

In the past, lupus was not well understood. People who had lupus died younger, usually of problems with vital organs. Now that the disease can be treated more successfully, life expectancy with lupus has increased significantly. Up to 90% of people with lupus live at least 5 years after diagnosis. Nearly 70% live at least 20 years after diagnosis.⁴

What Increases Your Risk

The chances of developing lupus (systemic lupus erythematosus, or SLE) are higher in people who:

• Are female.
• Are black.
• Are between the ages 15 and 45.
• Have a family history of lupus.
• Take medications that are associated with drug-induced systemic lupus.

Results from studies are mixed on the effect that the hormone estrogen has on a woman's risk of lupus or of having lupus flares. For example, while most women do not have symptom flares during pregnancy, when a woman has a high level of estrogen, a few women do have flares during pregnancy. And although most women develop lupus when they are age 15 to 45, when estrogen levels are higher, a number of women develop lupus after menopause, when estrogen levels are low.

Hormone replacement therapy (HRT) and birth control pills (oral contraceptives) do not appear to affect a woman's risk of lupus.² Birth control pills also do not appear to increase the chance of symptom flares in women with moderate lupus that is inactive or under control.³

Some research suggests smoking may increase the risk of getting lupus.¹

When To Call a Doctor

Call 911 or other emergency services immediately if you have:

• Chest pain that is crushing or squeezing, occurs with sweating or nausea, and has not been previously diagnosed.
• A sudden increase in shortness of breath that makes it very difficult for you to breathe.
• One or more of the following signs of a stroke:
  • Sudden numbness, tingling, or weakness in or an inability to move (paralysis) part or all of one side of the body (such as the face, arm, and leg)
  • Vision changes that come on suddenly, such as dimness, blurring, double vision, or loss of vision in one or both eyes
  • A seizure
  • Sudden difficulty speaking or understanding speech
  • Sudden nausea or vomiting
  • A sudden, severe headache, different from previous headaches, that occurs without a known cause
  • Sudden dizziness, clumsiness, staggering, or fainting (loss of consciousness)

Call a doctor immediately if you:

• Have chest pain.
• Are short of breath.
• Have blood in your urine or are urinating less often and in smaller amounts than usual.
• Have a fever over 100.5 °F (38.1 °C), with or without headache and body aches, but you haven't recently been exposed to a cold or the flu.
• Experience depression or any changes in behavior or thinking.
• Have numbness or tingling in the hands or feet.
• Are dizzy or have muscle weakness.
• Have swelling of the lower legs or feet.

Call a doctor as soon as possible if you develop any new symptoms, such as fever, aching or swollen joints, increased fatigue, loss of appetite, hair loss, skin rashes, or new sores in your mouth or nose. Also call your doctor if any symptoms that you have had for a period of time get worse.

If you have not been diagnosed with lupus and you have symptoms such as joint pain, fatigue, or skin rashes, see your doctor or tell your doctor about your concerns at your next medical appointment.

Watchful Waiting

Lupus symptoms can be very vague. However, any time that fatigue, joint or muscle pain, fever, or other symptoms develop without clear cause and persist despite home treatment, it is appropriate to call your doctor. Some of the more serious problems of lupus, including kidney and heart disease, may have no symptoms until the organs have already been damaged. Regular doctor visits are essential.

Who To See

To evaluate initial symptoms and treat mild lupus, you can consult with:

• A family medicine doctor or an internist.
• A rheumatologist.
• An immunologist.

For long-term management of complicated lupus, consult with:

• A rheumatologist.
• An immunologist.

For more complicated cases of lupus, a rheumatologist is usually the primary doctor; other specialists are consulted as needed.

• For mental health problems such as depression, anxiety, psychosis, or other behavioral changes, see your family medical doctor or internist, or a psychiatrist.
• For the treatment of organ problems, a doctor who specializes in diseases of that particular organ system may work together with a rheumatologist or immunologist. The following practitioners typically treat vital organ problems caused by lupus:
  • Cardiologist
  • Dermatologist
  • Hematologist
  • Nephrologist
  • Neurologist
  • Pulmonologist

To prepare for your appointment, see the topic Making the Most of Your Appointment.

Exams and Tests

Lupus (systemic lupus erythematosus, or SLE) can be hard to recognize, sometimes taking weeks to years to diagnose. Lupus affects different people in different ways, and it can take time to develop the symptoms that suggest this disease. Your health professional will record your medical history and perform a physical examination, checking for the presence of certain criteria to help diagnose lupus. These criteria are used to separate lupus from other similar diseases. A person with 4 of these 11 conditions can be classified as having lupus. These conditions may be present all at once, or they may appear in succession over a period of time.¹

Classification criteria for systemic lupus erythematosus:

• Butterfly (malar) rash on cheeks
• Rash on face, arms, neck, torso (discoid rash)
• Skin rashes that result from exposure to sunlight or ultraviolet light (photosensitivity)
• Mouth or nasal sores (ulcers), usually painless
• Joint swelling, stiffness, pain involving two or more joints (arthritis)
• Inflammation of the membranes surrounding the lungs (pleuritis) or heart (pericarditis)
• Abnormalities in urine, such as increased protein in the urine or clumps of red blood cells or kidney cells, called cell casts, in the urine
• Nervous system problems, such as seizures or psychosis, without known cause
• Problems with the blood, such as reduced numbers of red blood cells (anemia), platelets, or white blood cells
• Laboratory tests indicating increased autoimmune activity (antibodies against normal tissue)
• Positive antinuclear antibody (ANA) test

Initial diagnosis and disease monitoring

If you have physical signs of lupus and a positive ANA test result, further testing may not be necessary. If your doctor feels that further testing is necessary to clarify your diagnosis, you may have one or more of the following tests:

• Other antibody blood tests
• Complement test
• Erythrocyte sedimentation rate (ESR, or sed rate) or C-reactive protein (CRP)
• Complete blood count (CBC)
• Urinalysis

Evaluating possible organ damage

As part of ongoing treatment for lupus, you may have a:

• Urinalysis to check for protein and cells, signs of possible kidney problems.
• Kidney biopsy, if your doctor sees signs of kidney inflammation. This test may help your doctor determine the best treatment for you. Only a small number of people with lupus need a kidney biopsy.

To evaluate other possible causes of symptoms, imaging tests are sometimes done, depending on which organ systems are involved. Imaging tests include computed tomography (CT) scan, echocardiogram, magnetic resonance imaging (MRI), and X-rays.

Treatment Overview

Your treatment choices for lupus (systemic lupus erythematosus, or SLE) depend on how severe your symptoms are, whether your organs are affected, and how much your symptoms are affecting your daily life. Your treatment plans should be tailored to your individual needs and will change over time, as the disease flares or ebbs. There currently is no cure for lupus.

You may be able to control your symptoms with self-care and medication. Self-care includes learning as much as possible about lupus, maintaining good communication with your health professional, and developing a healthy lifestyle. Medications that may be used to treat lupus include nonsteroidal anti-inflammatory drugs (NSAIDs), corticosteroids, antimalarials, and immunosuppressants.

Initial treatment

The goal of treatment for mild lupus is to prevent symptom flares-when fatigue, joint pain, and rash get worse. Maintain a schedule of regular checkups with your health professional, instead of waiting until your disease flares. When flares do occur, the goal is to treat them rapidly to limit any damage to body organs.

Treatment for mild lupus includes:

• Avoiding the sun. If you must be in the sun, cover your arms and legs, wear a hat, and apply broad-spectrum sunscreen (covering both UVA and UVB rays) with a high sun protection factor (such as SPF 50) to protect your skin.
• Applying corticosteroid cream for rashes.
• Taking nonsteroidal anti-inflammatory drugs (NSAIDs) and getting plenty of rest for mild joint or muscle pain and fever.
• Taking antimalarial medications to treat fatigue, joint pain, skin rashes, and lung inflammation.
• Taking low-dose corticosteroids if NSAIDs aren't effective in controlling your symptoms.

For more severe cases of lupus, treatment may include:

• Higher-dose corticosteroids, either in pills or by injection.
• Medications that suppress the immune system (immunosuppressants).

Good self-care is essential to managing lupus. A healthy lifestyle may reduce the frequency and severity of flares, resulting in an improved quality of life. Self-care includes:

• Regular exercise.
• Education about lupus and self-care.
• Not smoking.
• Eating a healthful, balanced diet.
• Developing a support system of family, friends, and health professionals.

Treatment for the skin rash that many people develop with lupus starts with sunscreens, sun avoidance, and clothing to protect skin from the sun. If needed, medications may include antimalarials, corticosteroid creams and pills, and retinoids such as acitretin. Some treatments work for some people but not for others, and some treatments may have long-term side effects. More research is needed to determine which of these treatments is safest and most effective.

Ongoing treatment

Progression of lupus varies by the individual. Flares and remissions can occur abruptly, unexpectedly, and without clear cause. The major goal for ongoing treatment of lupus is prevention or management of damage to the body organs, including the arteries, kidneys, bones, and brain.

To control mild but continuing symptoms of lupus, treatment includes:

• Avoiding the sun. If you must be in the sun, cover your arms and legs, wear a hat, and apply broad-spectrum sunscreen (covering both UVA and UVB rays) with a high sun protection factor (such as SPF 50) to protect your skin.
• Applying corticosteroid cream for rashes.
• Taking nonsteroidal anti-inflammatory drugs (NSAIDs) and getting plenty of rest for mild joint or muscle pain and fever.
• Taking antimalarial medications to treat fatigue, joint pain, skin rashes, and lung inflammation.
• Taking corticosteroids if NSAIDs aren't effective in controlling your symptoms.

If your lupus symptoms are more severe and damage to organs is threatened, treatment may include:

• Corticosteroids in higher dose, for serious complications needing longer-term treatment.
• Medication that suppresses your immune system (immunosuppressants).

Good self-care is essential to managing lupus. A healthy lifestyle may reduce the frequency and severity of flares, resulting in an improved quality of life. Self-care includes:

• Regular exercise.
• Education about lupus and self-care.
• Not smoking.
• Eating a healthful, balanced diet.
• Developing a support system of family, friends, and health professionals.

Treatment if the condition gets worse

If you have severe complications of lupus that seriously impact your quality of life or are life-threatening or causing serious organ damage, you will likely need aggressive treatment, which may include:

• High-dose corticosteroids.
• Medication that suppresses your immune system (immunosuppressants), necessary to prevent permanent damage to your organs and possible death.

Proper self-care is essential for the successful management of lupus. It improves your quality of life and may help delay flares (times when your symptoms get worse). For example, you may be able to limit symptom flares by protecting against sun exposure, avoiding and promptly treating infections, and keeping your stress level as low as you can. Stress reduction techniques include exercise and simplifying your schedule. Getting plenty of rest may offset the fatigue that is common in lupus.

Some people with lupus produce a protein (antibody) that attacks certain blood-clotting factors, which can cause the blood to clot easily. This condition, called antiphospholipid antibody syndrome, can lead to mild or severe blood-clotting complications. If you develop this condition, you may need anticoagulant medication to slow blood clotting. This is especially important if you have already developed blood clots. If you have antiphospholipid antibody syndrome but have not had any blood clots, you may be able to take aspirin. Aspirin is a nonsteroidal anti-inflammatory drug (NSAID) that is sometimes used to slow blood clotting.

If you develop serious kidney disease that cannot be controlled with medication, you may need dialysis or a kidney transplant.

What To Think About

Corticosteroid treatment and physical inactivity put people with lupus at great risk of bone thinning (osteoporosis). Getting an adequate supply of calcium and vitamin D may slow the bone thinning process. Your health professional may also prescribe bisphosphonates, a type of medication that is also used for the prevention and treatment of osteoporosis. For more information, see the topic Osteoporosis.

Lupus treatment is complicated by several factors:

• The course and pattern of lupus symptoms vary widely.
• Flares and remissions can occur at any time, making it hard to tell how you are responding to treatment or which treatments are most helpful.
• Some treatment side effects can be as troubling as the symptoms of lupus.

It may not be possible to completely eliminate all of your symptoms for long periods of time, especially without the side effects from medications. Work closely with your health professional to reach a balance between reasonably controlling your symptoms, preventing damage to your organs, and minimizing side effects of long-term drug treatment. For example, you may take a dose of medication that will control lupus enough to prevent organ damage, but you may still have symptoms such as mild skin rash, muscle aches, and joint pain. Using higher doses of medications for a long time increases the risk of serious side effects. Your health professional will prescribe a dose that controls only the most serious, life-threatening symptoms and balances the risks of the medications with the benefits of controlling your symptoms.

The hormones in birth control pills (oral contraceptives) have not proven to be harmful in women with stable, moderate lupus.³ Women with lupus that is not well controlled may choose to use nonhormonal birth control methods, such as a condom or a diaphragm. For more information, see the topic Birth Control, and discuss your options with your health professional.

Prevention

There is currently no way to prevent lupus (systemic lupus erythematosus, or SLE). But people who smoke may be more likely to develop lupus.⁷ Avoiding smoking and perhaps other tobacco products may decrease your risk of developing lupus.

Home Treatment

Good self-care is essential to managing lupus (systemic lupus erythematosus, or SLE). Learn to recognize your body's warning signs of a flare. Warning signs may include increased fatigue, joint pain, rash, or fever. When you notice any of these signs, take steps to control your symptoms.

Stress may trigger lupus symptoms. Keep your stress level as low as you can.

• Keep your daily schedule as simple as possible.
• Keep your list of obligations to others to a bare minimum.
• Delegate to others.
• Exercise regularly. A daily walk, for example, can reduce stress, clear your head, improve your mood, and help fight fatigue.
• Use relaxation techniques such as meditation, yoga, and guided imagery to calm your body and mind.

Fatigue is common in people with lupus. To fight fatigue:

• Get plenty of rest. Some people with lupus need up to 12 hours of sleep every night.
• Pace yourself. Limit tiring activities.
• Ask others for help. Don't try to do everything yourself.
• Take short breaks from your usual daily activities. Consider cutting down on work hours or getting help with parenting responsibilities, at least during periods when lupus symptoms are severe.
• Exercise regularly. Physical activity boosts energy and helps you stay in good condition. Walking and swimming are good forms of exercise for people with lupus.
• If you suspect that depression is contributing to your fatigue, get prompt treatment from your doctor, a mental health professional, or both.

Take care of your skin. Ask your doctor about the use of corticosteroid creams to relieve skin symptoms that are particularly troublesome. If you are bothered by the way a lupus rash looks on your face or if you have scars from lupus, you can try makeup, such as Covermark, to cover the rash or scars.

Ultraviolet (UVA and UVB) light triggers disease flares in up to 70% of people with lupus.⁸ Exposure to ultraviolet light, as from sunlight, can trigger or start skin rash, joint pain, or fatigue, or it can make these symptoms worse. To minimize your exposure to ultraviolet light:

• Avoid the sun. If you must be in the sun, cover your arms and legs, wear a hat, and apply broad-spectrum sunscreen (covering both UVA and UVB rays) with a high sun protection factor (50 SPF or higher) to protect your skin. Reapply sunscreen after swimming, sweating, or toweling off. Experiment with sunscreens. Some may irritate your skin or wash off too easily.
• Avoid going out when the sun's rays are the strongest. In most areas, this is between the hours of 10 a.m. and 4 p.m., especially during the summer.

Good general care is essential. A healthy lifestyle not only improves your quality of life but may also reduce your chances of having more frequent and severe flares. Good care includes:⁹

• Getting vaccinations to help protect you from illnesses such as pneumonia and the flu.
• Treating high blood pressure.
• Taking medicine to help prevent osteoporosis caused by corticosteroids.
• Preventing plaque in the arteries (atherosclerosis) that can be made worse by corticosteroids.
• Protecting yourself against infections you can get more easily due to decreased immune system function.

Other good health habits that will help protect you include:

• Regular exercise.
• Education about lupus and self-care.
• Not smoking. Studies show that smoking makes symptoms worse in people with lupus and may decrease the effectiveness of some medicines. Experts suggest that people with lupus avoid all tobacco products.¹⁰
• Eating a healthful, balanced diet.
• Regular dental care.
• Regular eye examinations by an ophthalmologist.
• Developing a support system of family, friends, and health professionals.

Some people with lupus are sensitive to antibiotic drugs called sulfonamides (sulfa drugs). These include Bactrim, Septra, and many others. Your doctor can prescribe drugs that do not contain sulfa, if needed.

If you have lupus and are a woman in your childbearing years, pay special attention to pregnancy-related concerns, both before conceiving and while pregnant. Most women with well-controlled lupus can take birth control pills if they choose that method of birth control, and for most women lupus will not interfere with becoming pregnant or with pregnancy. But some women with lupus, especially those with active disease, are at higher risk of complications with pregnancy. All women of childbearing age should check with their rheumatologist when they are planning to become pregnant.

Home treatment and regular checkups are sometimes sufficient for managing mild lupus or for periods of remission. Be sure to have your doctor monitor your condition on a regular basis. These regular checks are important to detecting and treating progressive organ damage.

It is important that the people in your life understand what lupus is, how it affects your life, and how you can best cope with it. Help them understand your limitations and needs when your symptoms flare. Support groups are great places to learn coping strategies from others. For information about support groups, see the Other Places to Get Help section of this topic.

Medications

Medications cannot cure lupus (systemic lupus erythematosus, or SLE), but they can control many symptoms and often can prevent or slow organ damage.

Because most lupus symptoms are caused by inflammation, nonsteroidal anti-inflammatory drugs (NSAIDs) and antimalarial medications are often enough to reduce symptoms.

Severe lupus may be treated with more aggressive medications that suppress the immune system, such as corticosteroids and immunosuppressive medications. Because these medications can cause serious side effects of their own, doctors prescribe and monitor them carefully.

Treatment for the skin rash that many people develop with lupus may include sunscreens, protective clothing, and avoiding sun exposure, as well as medications. Some medications work for some people but not for others, and some treatments have long-term side effects. More research is needed to determine which treatments are safest and most effective for skin rash.

Some lupus medications, like acetaminophen and prednisone, are considered safe during pregnancy. Others may not be. You may not be able to stop taking lupus medications after becoming pregnant, or you may need to start taking medication for a symptom flare. If possible, talk to your health professional before becoming pregnant about the effect lupus may have on your pregnancy.

Medication Choices

If you have mild disease or symptoms that affect your quality of life but don't have organ-threatening problems, your doctor may prescribe:

• Nonsteroidal anti-inflammatory drugs (NSAIDs), sometimes in combination with antimalarial drugs.
• Antimalarial drugs such as hydroxychloroquine (Plaquenil).
• Low-dose corticosteroids and/or topical corticosteroid creams or ointments.

If you have more severe disease, your doctor may prescribe:

• Corticosteroids, such as prednisone.
• Immunosuppressive medications, such as azathioprine, cyclophosphamide, cyclosporine, mycophenolate mofetil, or methotrexate.

If you have previously had blood clots in a vein or artery (venous or arterial thrombosis), or have antiphospholipid antibody syndrome, which increases your risk of developing blood clots, your doctor may prescribe a blood thinner (anticoagulant). This is especially important if you have already developed blood clots. If you have antiphospholipid antibody syndrome but have not had any blood clots, you may be able to take aspirin. Aspirin is a nonsteroidal anti-inflammatory drug (NSAID) that is sometimes used to slow blood clotting in antiphospholipid antibody syndrome.

What To Think About

Medication treatment for lupus often involves reaching a balance between preventing severe, possibly life-threatening organ damage, maintaining an acceptable quality of life, and minimizing side effects.

Lupus treatment requires frequent monitoring of disease activity and medication side effects. Depending on how you respond to medications, your health professional may vary the dose and combinations of medications until you reach the best possible balance.

It may not be possible to completely eliminate all your symptoms for long periods of time, especially without medication side effects. For example, you may take a dose of medication that will control lupus enough to prevent organ damage, but you may still have symptoms such as mild skin rash, muscle aches, and joint pain. While higher doses of medications may relieve your symptoms, using them for a long time increases your risk of serious side effects. Your health professional will prescribe a dose that controls only the most serious, life-threatening symptoms and balances the risks of the medications with the benefits of controlling your symptoms.

People with lupus can go into spontaneous remission. If you experience an apparent remission, your doctor may taper or stop your medication.

Surgery

Surgery is not used to treat mild or moderate symptoms of lupus (systemic lupus erythematosus, or SLE). Surgery may be considered for people with lupus who have permanent, life-threatening kidney damage. A kidney transplant or kidney dialysis may be done instead of continuing long-term treatment with high doses of medications that have serious side effects.

• If kidney disease from lupus does not respond to high-dose corticosteroids and other immunosuppressive medications, kidney dialysis or transplant are reasonable options.
• For unknown reasons, overall lupus disease activity is often less severe during dialysis and after kidney transplant.

Other Treatment

While some people with lupus (systemic lupus erythematosus, or SLE) try alternative or complementary therapies (such as special diets, fish oils, or chiropractic treatment), these are not proven treatments for lupus.

Some therapies that focus on relaxation can help you cope with having a long-lasting (chronic) disease and may significantly improve your quality of life. These relaxation therapies include:

• Massage.
• Yoga.
• Guided imagery.

Experimental therapies for lupus

Medications and therapies currently under investigation are meant to alter immune system function, thus preventing the disease from progressing. These newer treatments include stem cell transplantation and biologic treatment.

Immunoablation with or without stem cell transplantation is being studied as a treatment for severe lupus. Immunoablation uses powerful drugs to wipe out the damaged immune system. After immunoablation, either the bone marrow is allowed to replace itself, or it is partially replaced through a stem cell transplant. The transplant replaces damaged or destroyed bone marrow cells with healthy cells, or stem cells. Stem cells are immature cells that are produced in the bone marrow. They can divide to produce more stem cells or can mature into red blood cells, white blood cells, and platelets. More study of these treatments for lupus is needed.¹¹

Biologic treatment blocks specific steps in the lupus autoimmune process without suppressing the entire immune system. Researchers are currently experimenting with very specific substances, such as antibodies and nucleotides, that block the earliest steps of the autoimmune process. Studies have tested several biologic agents in the last few years. Examples of these substances are CTLA-4, anti-C5B, and CD154.⁴ Another biologic substance under study, LJP-394, has shown some effectiveness in reducing lupus disease activity.¹² Rituximab is an antibody directed against certain immune cells that may have a role in lupus. It is approved for treating rheumatoid arthritis. Studies are looking at the use of rituximab for lupus. It is beginning to be used for lupus flares that have not responded to other immunosuppressive therapies. In some cases, rituximab has been associated with serious side effects such as breathing difficulty, heart problems, or severe infection. So, the use of rituximab is closely watched.

DHEA (also called prasterone in the United States) is an androgenic dietary supplement that is derived from the wild yam. Experts suggest only using pharmaceutical-grade (versus "natural") DHEA. Results of research are mixed but suggest that DHEA may lessen the need for corticosteroids and improve bone density, as well as reduce symptoms for people with lupus.¹² The most common side effects of DHEA are acne and facial hair growth in women and hair loss in men. Because this supplement is a hormonal substance, consult your doctor before using it, and have your DHEA blood levels checked every 6 months.

Plasmapheresis, the removal of some parts of the blood, is rarely used as treatment for lupus except for severe cases in which other treatments are not effective. Studies continue on plasmapheresis as treatment for lupus; some studies show benefit while others show no benefit.^{12, 11}

Intravenous immune globulin (IVIG) may be used to treat lupus that is associated with destruction of blood platelets.¹³

Other Places To Get Help

Organizations

References

Citations

1. Petri MA (2005). Systemic lupus erythematosus: Clinical aspects. In WJ Koopman, LW Moreland, eds., Arthritis and Allied Conditions: A Textbook of Rheumatology, 15th ed., vol. 2, pp.1473-1496. Philadelphia: Lippincott Williams and Wilkins.

2. Cooper GS, et al. (2002). Hormonal and reproductive risk factors for development of systemic lupus erythematosus. Arthritis and Rheumatism, 46(7): 1830-1839.

3. Petri MA, et al. (2005). Combined oral contraceptives in women with systemic lupus erythematosus. New England Journal of Medicine, 353(24): 2550-2558.

4. Lockshin MD (2005). Systemic lupus erythematosus. In DC Dale, DD Federman, eds., ACP Medicine, section 15, chap. 4. New York: WebMD.

5. Keyser RE, et al. (2003). Evidence for aerobic insufficiency in women with systemic lupus erythematosus. Arthritis and Rheumatism, 49(1): 16-22.

6. Bruce IN, et al. (2003). Risk factors for coronary heart disease in women with systemic lupus erythematosus. Arthritis and Rheumatism, 48(11): 3159-3167.

7. Costenbader KH, et al. (2004). Cigarette smoking and the risk of systemic lupus erythematosus. Arthritis and Rheumatism, 50(3): 849-857.

8. Hahn BH, et al. (2005). Pathogenesis of systemic lupus erythematosus. In ED Harris et al., eds., Kelley's Textbook of Rheumatology, 7th ed., vol. 2, pp. 1174-1200. Philadelphia: Elsevier Saunders.

9. Wofsy D (2005). Therapy of systemic lupus erythematosus. In WJ Koopman, LW Moreland, eds., Arthritis and Allied Conditions: A Textbook of Rheumatology, 15th ed., vol. 2, pp. 1561-1574. Philadelphia: Lippincott Williams and Wilkins.

10. Ghaussy NO, et al. (2003). Cigarette smoking and disease activity in systemic lupus erythematosus. Journal of Rheumatology, 30(6): 1215-1221.

11. Hahn BH (2005). Management of systemic lupus erythematosus. In ED Harris et al., eds., Kelley's Textbook of Rheumatology, 7th ed., vol. 2, pp. 1225-1247. Philadelphia: Elsevier Saunders.

12. Wallace DJ (2002). Management of lupus erythematosus: Recent insights. Current Opinion in Rheumatology, 14(3): 212-219.

13. Petri M (2001). Systemic lupus erythematosus (including pregnancy and antiphospholipid antibody syndrome). In MH Weisman et al., eds., Treatment of the Rheumatic Diseases: Companion to Kelley's Textbook of Rheumatology, 2nd ed., pp. 274-288. Philadelphia: W.B. Saunders.

Other Works Consulted

• Bertsias GK, et al. (2007). EULAR recommendations for the management of systemic lupus erythematosus (SLE) report of a task force of the European Standing Committee for International Clinical Studies Including Therapeutics (ESCISIT). Annals of the Rheumatic Diseases, 67(2): 195-205.

• Madhok R, Wu O (2006). Systemic lupus erythematosus, search date April 2006. Online version of Clinical Evidence (15): 1-12.

• McMurray RW, May W (2003). Sex hormones and systemic lupus erythematosus: Review and meta-analysis. Arthritis and Rheumatism, 48(8): 2100-2110.

Credits