Lupus is a chronic inflammatory disease that occurs when your body's immune system attacks your own tissues and organs. Inflammation caused by lupus can affect many different body systems, including your joints, skin, kidneys, blood cells, heart and lungs.
Lupus occurs more frequently in women than it does in men, though it isn't clear why. Four types of lupus exist — systemic lupus erythematosus, discoid lupus erythematosus, drug-induced lupus erythematosus and neonatal lupus. Of these, systemic lupus erythematosus is the most common and serious form of lupus.
The outlook for people with lupus was once grim, but diagnosis and treatment of lupus has improved considerably. With treatment, most people with lupus can lead active lives.
Signs and symptoms
No two cases of lupus are exactly alike. Signs and symptoms may come on suddenly or develop slowly, may be mild or severe, and may be temporary or permanent. Most people with lupus experience episodes — called "flares" — of worsening signs and symptoms that eventually improve or even disappear completely for a time.
The signs and symptoms of lupus that you experience will depend on which body systems are affected by the disease. But, in general, lupus signs and symptoms may include:
Fatigue
Fever
Weight loss or gain
Joint pain, stiffness and swelling
Butterfly-shaped rash (malar rash) on the face that covers the cheeks and bridge of the nose
Skin lesions that appear or worsen with sun exposure
Mouth sores
Fingers and toes that turn white or blue when exposed to cold or during stressful periods (Raynaud's phenomenon)
Shortness of breath
Chest pain
Dry eyes
Easy bruising
Anxiety
Depression
Memory loss
Causes
Lupus is an autoimmune disease, which means that instead of just attacking foreign substances, such as bacteria and viruses, your immune system also turns against healthy tissue. This leads to inflammation and damage to various parts of the body, including the joints, skin, kidneys, heart, lungs, blood vessels and brain.
Doctors don't know what causes autoimmune diseases, such as lupus. It's likely that lupus results from a combination of your genetics and your environment. Doctors believe that you may inherit a predisposition to lupus, but not lupus itself. Instead, people with an inherited predisposition for lupus may only develop the disease when they come into contact with something in the environment that can trigger lupus, such as a medication or a virus.
Types of lupus
Four types of lupus exist. Though similar, each type of lupus has a different prognosis and treatment.
Systemic lupus erythematosus can affect nearly any part of your body. Body systems most commonly involved include the skin, joints, lungs, kidneys and blood. When people talk about lupus, they're usually referring to systemic lupus erythematosus.
Discoid lupus erythematosus affects only the skin. People with discoid lupus, also called cutaneous lupus, experience a circular rash on the face, neck and scalp. A small number of people with discoid lupus may develop systemic lupus erythematosus, though it isn't possible to predict who will develop the more serious form of lupus.
Drug-induced lupus erythematosus occurs after you take certain prescription medications. Not everyone who takes these medications develops lupus. Drug-induced lupus affects a wide variety of body systems. Signs and symptoms usually go away when you stop taking the medication that caused your lupus.
Neonatal lupus is a rare form of lupus that affects newborn babies. A mother with certain antibodies that are linked to autoimmune diseases can pass them to the developing fetus — even if the mother has no signs or symptoms of an autoimmune disease. The antibodies can cause neonatal lupus. A baby with neonatal lupus may experience a rash in the weeks following birth. Neonatal lupus may last about six months before disappearing.
Risk factors
While doctors don't know what causes lupus in many cases, they have identified factors that may increase your risk of the disease, including:
Sex. Lupus is more common in women than in men.
Age. Although lupus affects people of all ages, including infants, children and older adults, it's most often diagnosed between the ages of 15 and 45.
Race. Lupus is more common in blacks and in Asians.
Sunlight. Exposure to the sun may bring on lupus skin lesions or trigger an internal response in susceptible people. Exactly why ultraviolet radiation has this effect isn't well understood, but scientists suspect that sunlight may cause skin cells to express certain proteins on their surface. Antibodies that are normally present in the body then latch onto these proteins, initiating an inflammatory response. Damaged skin cells also seem to die more frequently in people with lupus, leading to even more inflammation.
Certain prescription medications. Drug-induced lupus results from the long-term use of certain prescription drugs. Although many medications can potentially trigger lupus, examples of drugs most clearly linked with the disease include the antipsychotic chlorpromazine, high blood pressure medications such as hydralazine, the tuberculosis drug isoniazid and the heart medication procainamide, among others. It usually takes several months or years of therapy with these drugs before symptoms appear, and even then, only a small percentage of people will ever develop lupus.
Infection with Epstein-Barr virus. Almost everyone has been infected with a common human virus called Epstein-Barr virus. Epstein-Barr virus causes nonspecific signs and symptoms, such as fever and sore throat. Once the initial infection subsides, the virus remains dormant in the cells of your immune system unless something reactivates the virus. For reasons that aren't clear, recurrent Epstein-Barr infections seem to increase the risk of developing lupus.
Exposure to chemicals. It's difficult to prove that chemicals can cause or increase the risk of a disease. But some studies have shown that people who work in jobs that involve exposure to mercury and silica may have an increased risk of lupus.
When to seek medical advice
If you develop an unexplained rash, ongoing fever, persistent aching or fatigue, see your doctor.
If you've already been diagnosed with lupus, meet with your doctor on a regular basis so that your condition and treatment can be monitored. Make an appointment with your doctor if new symptoms arise.
Screening and diagnosis
Diagnosing lupus is difficult because signs and symptoms vary considerably from person to person. Signs and symptoms of lupus may change over time and overlap with those of many other disorders. For these reasons, doctors may not initially consider lupus until the signs and symptoms become more obvious. Even then, lupus can be challenging to diagnose because nearly all people with lupus experience fluctuations in disease activity. At times the disease may become severe and at other times subside completely.
American College of Rheumatology criteria for a lupus diagnosis
The American College of Rheumatology (ACR) has developed clinical and laboratory criteria to help physicians diagnose and classify lupus. If you have four of the 11 criteria at one time or individually over time, you probably have lupus. Your doctor may also consider the diagnosis of lupus even if you have fewer than four of these signs and symptoms. The criteria identified by the ACR include:
Face rash, which doctors call a malar rash, that is butterfly shaped and covers the bridge of the nose and spreads across the cheeks
Scaly rash, called a discoid rash, which appears as raised, scaly patches
Sun-related rash, which appears after exposure to sunlight
Mouth sores, which are usually painless
Joint pain and swelling that occurs in two or more joints
Swelling of the linings around the lungs or the heart
Kidney disease
A neurological disorder, such as seizures or psychosis
Low blood counts, such as low red blood count, low platelet count (thrombocytopenia), or a low white cell count (leukopenia)
Positive anti-nuclear antibody tests, which indicate that you may have an autoimmune disease
Other positive blood tests that may indicate an autoimmune disease, such as a positive double-stranded anti-DNA test, positive anti-Sm test, positive anti-phospholipid antibody test or false-positive syphilis test
Laboratory tests
Your doctor may order blood and urine tests to determine your diagnosis, including:
Complete blood count. This test measures the number of red blood cells, white blood cells and platelets as well as the amount of hemoglobin, a protein in red blood cells. Results may indicate you have anemia, which commonly occurs in lupus. A low white blood cell or platelet count may occur in lupus as well.
Erythrocyte sedimentation rate. This blood test determines the rate at which red blood cells settle to the bottom of a tube in an hour. A faster than normal rate may indicate a systemic disease, such as lupus. The sedimentation rate isn't specific for any one disease, but it may be elevated if you have lupus, another inflammatory condition or an infection.
Kidney and liver assessment. Blood tests can assess how well your kidneys and liver are functioning. Lupus can affect these organs.
Urinalysis. An examination of a sample of your urine may show an increased protein level or red blood cells in the urine, which may occur if lupus has affected your kidneys.
Antinuclear antibody (ANA) test. A positive test for the presence of these antibodies — produced by your immune system — indicates a stimulated immune system, which is common in lupus and other autoimmune diseases. A positive ANA doesn't always mean that you have lupus, however. ANA levels can be elevated if you have an infection or if you're taking certain medications. If you test positive for ANA, your doctor may advise more-specific antibody testing and refer you to a rheumatologist, a doctor who specializes in musculoskeletal and autoimmune disorders such as arthritis or lupus.
Chest X-ray. An image of your chest may reveal abnormal shadows that suggest fluid or inflammation in your lungs. It may also show an enlarged heart as a result of a buildup of fluid within the pericardium (pericardial effusion).
Electrocardiogram (ECG). This test measures the pattern of electrical impulses generated in your heart. It can help identify irregular rhythms or damage.
Syphilis test. A false-positive result on a syphilis test can indicate anti-phospholipid antibodies in your blood, another indication of lupus. The presence of anti-phospholipid antibodies has been associated with an increased risk of blood clots, strokes and recurrent miscarriages.
Complications
Inflammation caused by lupus can affect many areas of your body, including your:
Kidneys. Lupus can cause serious kidney damage, and kidney failure is one of the leading causes of death among people with lupus. A blood test called serum creatinine level is used to monitor kidney function. Signs and symptoms of kidney problems may include generalized itching, chest pain, nausea, vomiting and weight gain.
Central nervous system. If your central nervous system is affected by lupus, you may experience headaches, dizziness, memory problems, behavior changes, even seizures.
Blood and blood vessels. Lupus may lead to blood problems, including anemia and increased risk of bleeding or blood clotting. It can also cause inflammation of the blood vessels (vasculitis).
Lungs. Having lupus increases your chances of developing an inflammation of the chest cavity lining (pleurisy) that can make breathing painful. You may also be more susceptible to a noninfectious form of pneumonia.
Heart. Lupus can cause inflammation of your heart muscle (myocarditis and endocarditis), your arteries (coronary vasculitis) or heart membrane (pericarditis). Having lupus also greatly increases your risk of cardiovascular disease and heart attacks. Controlling high blood pressure and high blood cholesterol, not smoking, and getting regular exercise are essential to help reduce the risk of heart disease.
Infection. People with lupus are vulnerable to infection because both the disease and its treatments — corticosteroid and cytotoxic drugs, in particular — affect the immune system. And in a vicious cycle, infection can bring on a lupus flare, increasing the risk of infection even more.
Cancer. Having lupus appears to increase your risk of cancer — especially non-Hodgkin's lymphoma, which affects the lymph system; lung cancer; and liver and bile duct cancers. Immunosuppressant drugs that are sometimes used to treat lupus also can increase the risk of cancer.
Bone tissue death (avascular necrosis). This occurs when the blood supply to a bone diminishes, often leading to tiny breaks in the bone and eventually to the bone's collapse. The hip joint is commonly affected, although avascular necrosis can occur in other bones as well. Avascular necrosis can be caused by lupus itself or by high doses of corticosteroids used to treat the disease.
Pregnancy complications. Women with lupus have an increased risk of miscarriage. Some women with lupus experience a flare during pregnancy. Lupus increases the risk of high blood pressure during pregnancy (preeclampsia) and preterm birth.
Treatment
Treatment for lupus depends on your signs and symptoms. Determining whether your signs and symptoms should be treated and what medications to use requires a careful discussion of the benefits and risks with your doctor. As your signs and symptoms flare and subside, you and your doctor may find that you'll need to change medications or dosages.
Common medications used to treat lupus
Three types of drugs are commonly used to treat lupus when your signs and symptoms are mild or moderate. More aggressive lupus may require more aggressive drugs. In general, when first diagnosed with lupus, your doctor may discuss these medications:
Nonsteroidal anti-inflammatory drugs. Aspirin or other nonsteroidal anti-inflammatory drugs (NSAIDs) such as naproxen sodium (Aleve) and ibuprofen (Advil, Motrin, others) may be used to treat a variety of signs and symptoms associated with lupus. NSAIDs are available over-the-counter, or stronger versions can be prescribed by your doctor. Check with your doctor before taking over-the-counter NSAIDs because some have been associated with serious side effects in people with lupus. Side effects of NSAIDs include stomach bleeding and an increased risk of heart problems.
Antimalarial drugs. Although there's no known relationship between lupus and malaria, these medications have proved useful in treating signs and symptoms of lupus. Antimalarials may also prevent flares of the disease. Hydroxychloroquine (Plaquenil) is the most commonly prescribed antimalarial. Side effects of antimalarial drugs include vision problems and muscle weakness.
Corticosteroids. These drugs counter the inflammation of lupus, but can have serious long-term side effects, including weight gain, easy bruising, thinning bones (osteoporosis), high blood pressure, diabetes and increased risk of infection. The risk of side effects increases with higher doses and longer term therapy. To help reduce these risks, your doctor will try to find the lowest dose that controls your symptoms and prescribe corticosteroids for the shortest possible time. Taking the drug every other day can also help reduce side effects. Corticosteroids are sometimes combined with another medication to help reduce the dose, and therefore the toxicity, of both drugs. Taking calcium and vitamin D supplements while using corticosteroids can reduce the risk of osteoporosis.
Treatment for specific signs and symptoms
What treatments you may consider depend on your signs and symptoms. Treatments for specific signs and symptoms include:
Joint pain and swelling. Pain in your joints may be initially controlled with NSAIDs. If you experience more significant joint pain, you and your doctor may consider antimalarial drugs or corticosteroids.
Skin rashes. Avoid skin rashes by staying out of the sun, wearing sunblock year-round and keeping your skin covered. Despite your best attempts to avoid the sun, even indoor fluorescent lighting can trigger skin rashes in people with lupus. Skin rashes are sometimes treated with topical corticosteroids. These creams are applied to the affected area to reduce the inflammation in your skin cells. Oral steroids or antimalarial drugs also can be used.
Fatigue. Fatigue is treated by determining the underlying cause. Your fatigue may be caused by difficulty sleeping, depression or poorly controlled pain. In these cases, your doctor would treat the underlying cause. If you and your doctor can't determine a cause for your fatigue, you may consider medications such as corticosteroids and antimalarial drugs.
Swelling around the heart and lungs. Swelling around your heart and lungs that causes chest pain may be controlled NSAIDs, antimalarial drugs or corticosteroids.
Treatment for aggressive lupus
Life-threatening cases of lupus — those including kidney problems, inflammation in the blood vessels, and central nervous system problems, such as seizures — may require more aggressive treatment. In these cases, you and your doctor may consider:
High-dose corticosteroids. High-dose corticosteroids can be taken orally or administered through a vein in your arm (intravenously). A high-dose regimen of corticosteroids may help control dangerous signs and symptoms quickly, but can also cause serious side effects, including infections, mood swings, high blood pressure and osteoporosis. To minimize side effects, your doctor will give you the lowest dose needed to control your signs and symptoms and then reduce the dosage over time.
Immunosuppressive drugs. Drugs that suppress the immune system may be helpful in serious cases of lupus, but can cause serious side effects. The most commonly used immunosuppressive drugs include cyclophosphamide (Cytoxan) and azathioprine (Imuran). The drug mycophenolate mofetil (CellCept), another immunosuppressant, can be used to treat lupus-related kidney problems. All three of these drugs can be taken orally, and cyclophosphamide can also be given intravenously. Side effects include an increased risk of infection, liver damage, infertility and an increased risk of cancer.
High-dose corticosteroids can be combined with immunosuppressive drugs to reduce the dosage of each drug, which may reduce the risk of side effects. Sometimes, even with aggressive treatment, your kidneys may fail. In that case, you may need kidney dialysis or, if kidney failure is permanent, a kidney transplant.
Clinical trials
Researchers are developing new treatments for lupus in clinical trials. These studies give people with lupus the chance to try new treatments, but they don't guarantee a cure. If you're interested in a clinical trial, discuss the options with your doctor to determine the benefits and risks.
Treatments being studied in clinical trials include:
Stem cell transplant. A stem cell transplant uses your own adult stem cells to rebuild your immune system. Doctors hope this treatment can help people with life-threatening cases of lupus. Before a stem cell transplant you're given a drug that coaxes the adult stem cells out of your bone marrow and into your bloodstream. The stem cells are then filtered from your blood and frozen for later use. Strong immunosuppressive drugs are administered to wipe out your immune system. Then the adult stem cells are put back into your body where they can rebuild your immune system.
Dehydroepiandrosterone (DHEA). Some clinical trials have shown that a synthetic form of the hormone DHEA may improve quality of life in people with lupus, though others haven't found this. Your body uses DHEA to make male and female sex hormones. Researchers are conducting further clinical trials to better understand whether DHEA may be useful for people with lupus.
Rituximab (Rituxan). Rituximab decreases the number of B cells, a type of white blood cell, in your body and has shown some promise in treating lupus in people who haven't responded to other immunosuppressants. However, rituximab has been linked to a fatal brain infection in two people with lupus.
Self-care
Take steps to care for your body if you have lupus. Simple steps can help you prevent lupus flares and, should they occur, better cope with the signs and symptoms you experience. Try to:
Get adequate rest. People with lupus often experience persistent fatigue that's different from normal tiredness and that isn't necessarily relieved by rest. For that reason, it can be hard to judge when you need to slow down. Many experts recommend eight to 10 hours of sleep a night and naps or breaks during the day as needed. Friends and family members need to understand and respect your need for rest.
Be sun smart. Because ultraviolet light can trigger a flare, wear protective clothing such as a hat, long-sleeved shirt and long pants, and use sunscreens with a sun protection factor (SPF) of at least 15 every time you go outside, even if it's just a quick trip to the mailbox. Be sure that your ears, scalp and the backs of your hands are protected. Avoid tanning beds and stay out of the sun entirely when it's strongest, from 10 a.m. to 4 p.m. Because fluorescent and halogen lights also can emit UV rays and thus aggravate lupus, you may need to wear sunscreen and protective clothing indoors or use plastic devices that block UV emissions from indoor lights.
Get regular exercise. Exercise can help you recover from a flare, reduce your risk of heart attack, help fight depression and promote general well-being. Exercise as much as your body allows — aim for 30 minutes of activity most days of the week. You'll likely feel fatigued and not up to exercising sometimes, and that's OK. Rest when you need to. Time outdoor activities so that you avoid the sun when it's most intense, and if you're having a flare, stay out of the sun entirely.
Don't smoke. Smoking increases your risk of cardiovascular disease and can worsen the effects of lupus on your heart and blood vessels.
Eat a healthy diet. A healthy diet emphasizes fruits, vegetables and whole grains. Sometimes you may have dietary restrictions, especially if you have high blood pressure, kidney damage or gastrointestinal problems. And although no specific foods have been shown to cause or worsen lupus, it's best to avoid any food that seems to make your symptoms worse.
Coping
Coping with lupus can be stressful. People with lupus often experience anxiety, depression and frustration because the disease is unpredictable. Knowing you have a serious disease can also be scary. To help you cope with lupus, try to:
Learn all you can about lupus. Write down all the questions you have about lupus and ask them at your next appointment. Ask your doctor or nurse for reputable sources of further information. The more you know about lupus, the more confident you'll feel in your treatment choices.
Gather support among your friends and family. Talk about lupus with your friends and family. They may have questions about lupus and how it affects your life. Answer their questions honestly. Explain ways your friends and family can help out when you're having flares. Lupus can be frustrating for your loved ones because they usually can't see it and you may not appear sick. They can't tell if you're having a good day or a bad day unless you tell them. Be open about what you're feeling so that your friends and family know what to expect.
Take time for yourself. Cope with stress in your life by taking time for yourself. Use that time to read, meditate, listen to music or write in a journal. Find activities that calm and renew you.
Connect with others who have lupus. Talk to other people who have lupus. You can connect with other people who have lupus through support groups in your community or through online message boards. Though your friends and family love you, sometimes you'll feel as if they can't quite understand what you're feeling. Other people with lupus can offer unique support because they're facing many of the same obstacles and frustrations that you're facing.
Complementary and alternative medicine
If your medications aren't controlling all of your signs and symptoms or if you're frustrated by lupus flares, you might turn to complementary and alternative medicine for solutions. Mainstream doctors are becoming more open to discussing these options with their patients. But, since few of these treatments have been extensively studied in clinical trials, it's difficult to assess whether these treatments are helpful for lupus. In some cases, the risks of these treatments aren't known.
If you're interested in trying complementary and alternative medicine therapies, discuss these treatments with your doctor first. He or she can help you weigh the benefits and risks and tell you if the treatments will interfere with your current lupus medications.
Some common complementary and alternative treatments for lupus include:
Fish oil. Fish oil supplements contain omega-3 fatty acids that may be beneficial for people with lupus. Preliminary studies have found some promise, though more study is needed. Side effects of fish oil supplements can include nausea, belching and a fishy taste in the mouth. Fish oil can interfere with medications, so check with your doctor first.
Flaxseed. Flaxseed contains a fatty acid called alpha-linolenic acid, which may decrease inflammation in the body. Some studies have found that flaxseed may improve kidney function in people who have lupus that affects the kidneys, though more study is needed. Side effects of flaxseed include bloating and abdominal pain. Flaxseed can also interfere with medications, so check with your doctor first.
Other complementary and alternative medicine treatments are available. Discuss the options with your doctor.
By Mayo Clinic Staff
Oct 20, 2007
Lupus occurs more frequently in women than it does in men, though it isn't clear why. Four types of lupus exist — systemic lupus erythematosus, discoid lupus erythematosus, drug-induced lupus erythematosus and neonatal lupus. Of these, systemic lupus erythematosus is the most common and serious form of lupus.
The outlook for people with lupus was once grim, but diagnosis and treatment of lupus has improved considerably. With treatment, most people with lupus can lead active lives.
Signs and symptoms
No two cases of lupus are exactly alike. Signs and symptoms may come on suddenly or develop slowly, may be mild or severe, and may be temporary or permanent. Most people with lupus experience episodes — called "flares" — of worsening signs and symptoms that eventually improve or even disappear completely for a time.
The signs and symptoms of lupus that you experience will depend on which body systems are affected by the disease. But, in general, lupus signs and symptoms may include:
Fatigue
Fever
Weight loss or gain
Joint pain, stiffness and swelling
Butterfly-shaped rash (malar rash) on the face that covers the cheeks and bridge of the nose
Skin lesions that appear or worsen with sun exposure
Mouth sores
Fingers and toes that turn white or blue when exposed to cold or during stressful periods (Raynaud's phenomenon)
Shortness of breath
Chest pain
Dry eyes
Easy bruising
Anxiety
Depression
Memory loss
Causes
Lupus is an autoimmune disease, which means that instead of just attacking foreign substances, such as bacteria and viruses, your immune system also turns against healthy tissue. This leads to inflammation and damage to various parts of the body, including the joints, skin, kidneys, heart, lungs, blood vessels and brain.
Doctors don't know what causes autoimmune diseases, such as lupus. It's likely that lupus results from a combination of your genetics and your environment. Doctors believe that you may inherit a predisposition to lupus, but not lupus itself. Instead, people with an inherited predisposition for lupus may only develop the disease when they come into contact with something in the environment that can trigger lupus, such as a medication or a virus.
Types of lupus
Four types of lupus exist. Though similar, each type of lupus has a different prognosis and treatment.
Systemic lupus erythematosus can affect nearly any part of your body. Body systems most commonly involved include the skin, joints, lungs, kidneys and blood. When people talk about lupus, they're usually referring to systemic lupus erythematosus.
Discoid lupus erythematosus affects only the skin. People with discoid lupus, also called cutaneous lupus, experience a circular rash on the face, neck and scalp. A small number of people with discoid lupus may develop systemic lupus erythematosus, though it isn't possible to predict who will develop the more serious form of lupus.
Drug-induced lupus erythematosus occurs after you take certain prescription medications. Not everyone who takes these medications develops lupus. Drug-induced lupus affects a wide variety of body systems. Signs and symptoms usually go away when you stop taking the medication that caused your lupus.
Neonatal lupus is a rare form of lupus that affects newborn babies. A mother with certain antibodies that are linked to autoimmune diseases can pass them to the developing fetus — even if the mother has no signs or symptoms of an autoimmune disease. The antibodies can cause neonatal lupus. A baby with neonatal lupus may experience a rash in the weeks following birth. Neonatal lupus may last about six months before disappearing.
Risk factors
While doctors don't know what causes lupus in many cases, they have identified factors that may increase your risk of the disease, including:
Sex. Lupus is more common in women than in men.
Age. Although lupus affects people of all ages, including infants, children and older adults, it's most often diagnosed between the ages of 15 and 45.
Race. Lupus is more common in blacks and in Asians.
Sunlight. Exposure to the sun may bring on lupus skin lesions or trigger an internal response in susceptible people. Exactly why ultraviolet radiation has this effect isn't well understood, but scientists suspect that sunlight may cause skin cells to express certain proteins on their surface. Antibodies that are normally present in the body then latch onto these proteins, initiating an inflammatory response. Damaged skin cells also seem to die more frequently in people with lupus, leading to even more inflammation.
Certain prescription medications. Drug-induced lupus results from the long-term use of certain prescription drugs. Although many medications can potentially trigger lupus, examples of drugs most clearly linked with the disease include the antipsychotic chlorpromazine, high blood pressure medications such as hydralazine, the tuberculosis drug isoniazid and the heart medication procainamide, among others. It usually takes several months or years of therapy with these drugs before symptoms appear, and even then, only a small percentage of people will ever develop lupus.
Infection with Epstein-Barr virus. Almost everyone has been infected with a common human virus called Epstein-Barr virus. Epstein-Barr virus causes nonspecific signs and symptoms, such as fever and sore throat. Once the initial infection subsides, the virus remains dormant in the cells of your immune system unless something reactivates the virus. For reasons that aren't clear, recurrent Epstein-Barr infections seem to increase the risk of developing lupus.
Exposure to chemicals. It's difficult to prove that chemicals can cause or increase the risk of a disease. But some studies have shown that people who work in jobs that involve exposure to mercury and silica may have an increased risk of lupus.
When to seek medical advice
If you develop an unexplained rash, ongoing fever, persistent aching or fatigue, see your doctor.
If you've already been diagnosed with lupus, meet with your doctor on a regular basis so that your condition and treatment can be monitored. Make an appointment with your doctor if new symptoms arise.
Screening and diagnosis
Diagnosing lupus is difficult because signs and symptoms vary considerably from person to person. Signs and symptoms of lupus may change over time and overlap with those of many other disorders. For these reasons, doctors may not initially consider lupus until the signs and symptoms become more obvious. Even then, lupus can be challenging to diagnose because nearly all people with lupus experience fluctuations in disease activity. At times the disease may become severe and at other times subside completely.
American College of Rheumatology criteria for a lupus diagnosis
The American College of Rheumatology (ACR) has developed clinical and laboratory criteria to help physicians diagnose and classify lupus. If you have four of the 11 criteria at one time or individually over time, you probably have lupus. Your doctor may also consider the diagnosis of lupus even if you have fewer than four of these signs and symptoms. The criteria identified by the ACR include:
Face rash, which doctors call a malar rash, that is butterfly shaped and covers the bridge of the nose and spreads across the cheeks
Scaly rash, called a discoid rash, which appears as raised, scaly patches
Sun-related rash, which appears after exposure to sunlight
Mouth sores, which are usually painless
Joint pain and swelling that occurs in two or more joints
Swelling of the linings around the lungs or the heart
Kidney disease
A neurological disorder, such as seizures or psychosis
Low blood counts, such as low red blood count, low platelet count (thrombocytopenia), or a low white cell count (leukopenia)
Positive anti-nuclear antibody tests, which indicate that you may have an autoimmune disease
Other positive blood tests that may indicate an autoimmune disease, such as a positive double-stranded anti-DNA test, positive anti-Sm test, positive anti-phospholipid antibody test or false-positive syphilis test
Laboratory tests
Your doctor may order blood and urine tests to determine your diagnosis, including:
Complete blood count. This test measures the number of red blood cells, white blood cells and platelets as well as the amount of hemoglobin, a protein in red blood cells. Results may indicate you have anemia, which commonly occurs in lupus. A low white blood cell or platelet count may occur in lupus as well.
Erythrocyte sedimentation rate. This blood test determines the rate at which red blood cells settle to the bottom of a tube in an hour. A faster than normal rate may indicate a systemic disease, such as lupus. The sedimentation rate isn't specific for any one disease, but it may be elevated if you have lupus, another inflammatory condition or an infection.
Kidney and liver assessment. Blood tests can assess how well your kidneys and liver are functioning. Lupus can affect these organs.
Urinalysis. An examination of a sample of your urine may show an increased protein level or red blood cells in the urine, which may occur if lupus has affected your kidneys.
Antinuclear antibody (ANA) test. A positive test for the presence of these antibodies — produced by your immune system — indicates a stimulated immune system, which is common in lupus and other autoimmune diseases. A positive ANA doesn't always mean that you have lupus, however. ANA levels can be elevated if you have an infection or if you're taking certain medications. If you test positive for ANA, your doctor may advise more-specific antibody testing and refer you to a rheumatologist, a doctor who specializes in musculoskeletal and autoimmune disorders such as arthritis or lupus.
Chest X-ray. An image of your chest may reveal abnormal shadows that suggest fluid or inflammation in your lungs. It may also show an enlarged heart as a result of a buildup of fluid within the pericardium (pericardial effusion).
Electrocardiogram (ECG). This test measures the pattern of electrical impulses generated in your heart. It can help identify irregular rhythms or damage.
Syphilis test. A false-positive result on a syphilis test can indicate anti-phospholipid antibodies in your blood, another indication of lupus. The presence of anti-phospholipid antibodies has been associated with an increased risk of blood clots, strokes and recurrent miscarriages.
Complications
Inflammation caused by lupus can affect many areas of your body, including your:
Kidneys. Lupus can cause serious kidney damage, and kidney failure is one of the leading causes of death among people with lupus. A blood test called serum creatinine level is used to monitor kidney function. Signs and symptoms of kidney problems may include generalized itching, chest pain, nausea, vomiting and weight gain.
Central nervous system. If your central nervous system is affected by lupus, you may experience headaches, dizziness, memory problems, behavior changes, even seizures.
Blood and blood vessels. Lupus may lead to blood problems, including anemia and increased risk of bleeding or blood clotting. It can also cause inflammation of the blood vessels (vasculitis).
Lungs. Having lupus increases your chances of developing an inflammation of the chest cavity lining (pleurisy) that can make breathing painful. You may also be more susceptible to a noninfectious form of pneumonia.
Heart. Lupus can cause inflammation of your heart muscle (myocarditis and endocarditis), your arteries (coronary vasculitis) or heart membrane (pericarditis). Having lupus also greatly increases your risk of cardiovascular disease and heart attacks. Controlling high blood pressure and high blood cholesterol, not smoking, and getting regular exercise are essential to help reduce the risk of heart disease.
Infection. People with lupus are vulnerable to infection because both the disease and its treatments — corticosteroid and cytotoxic drugs, in particular — affect the immune system. And in a vicious cycle, infection can bring on a lupus flare, increasing the risk of infection even more.
Cancer. Having lupus appears to increase your risk of cancer — especially non-Hodgkin's lymphoma, which affects the lymph system; lung cancer; and liver and bile duct cancers. Immunosuppressant drugs that are sometimes used to treat lupus also can increase the risk of cancer.
Bone tissue death (avascular necrosis). This occurs when the blood supply to a bone diminishes, often leading to tiny breaks in the bone and eventually to the bone's collapse. The hip joint is commonly affected, although avascular necrosis can occur in other bones as well. Avascular necrosis can be caused by lupus itself or by high doses of corticosteroids used to treat the disease.
Pregnancy complications. Women with lupus have an increased risk of miscarriage. Some women with lupus experience a flare during pregnancy. Lupus increases the risk of high blood pressure during pregnancy (preeclampsia) and preterm birth.
Treatment
Treatment for lupus depends on your signs and symptoms. Determining whether your signs and symptoms should be treated and what medications to use requires a careful discussion of the benefits and risks with your doctor. As your signs and symptoms flare and subside, you and your doctor may find that you'll need to change medications or dosages.
Common medications used to treat lupus
Three types of drugs are commonly used to treat lupus when your signs and symptoms are mild or moderate. More aggressive lupus may require more aggressive drugs. In general, when first diagnosed with lupus, your doctor may discuss these medications:
Nonsteroidal anti-inflammatory drugs. Aspirin or other nonsteroidal anti-inflammatory drugs (NSAIDs) such as naproxen sodium (Aleve) and ibuprofen (Advil, Motrin, others) may be used to treat a variety of signs and symptoms associated with lupus. NSAIDs are available over-the-counter, or stronger versions can be prescribed by your doctor. Check with your doctor before taking over-the-counter NSAIDs because some have been associated with serious side effects in people with lupus. Side effects of NSAIDs include stomach bleeding and an increased risk of heart problems.
Antimalarial drugs. Although there's no known relationship between lupus and malaria, these medications have proved useful in treating signs and symptoms of lupus. Antimalarials may also prevent flares of the disease. Hydroxychloroquine (Plaquenil) is the most commonly prescribed antimalarial. Side effects of antimalarial drugs include vision problems and muscle weakness.
Corticosteroids. These drugs counter the inflammation of lupus, but can have serious long-term side effects, including weight gain, easy bruising, thinning bones (osteoporosis), high blood pressure, diabetes and increased risk of infection. The risk of side effects increases with higher doses and longer term therapy. To help reduce these risks, your doctor will try to find the lowest dose that controls your symptoms and prescribe corticosteroids for the shortest possible time. Taking the drug every other day can also help reduce side effects. Corticosteroids are sometimes combined with another medication to help reduce the dose, and therefore the toxicity, of both drugs. Taking calcium and vitamin D supplements while using corticosteroids can reduce the risk of osteoporosis.
Treatment for specific signs and symptoms
What treatments you may consider depend on your signs and symptoms. Treatments for specific signs and symptoms include:
Joint pain and swelling. Pain in your joints may be initially controlled with NSAIDs. If you experience more significant joint pain, you and your doctor may consider antimalarial drugs or corticosteroids.
Skin rashes. Avoid skin rashes by staying out of the sun, wearing sunblock year-round and keeping your skin covered. Despite your best attempts to avoid the sun, even indoor fluorescent lighting can trigger skin rashes in people with lupus. Skin rashes are sometimes treated with topical corticosteroids. These creams are applied to the affected area to reduce the inflammation in your skin cells. Oral steroids or antimalarial drugs also can be used.
Fatigue. Fatigue is treated by determining the underlying cause. Your fatigue may be caused by difficulty sleeping, depression or poorly controlled pain. In these cases, your doctor would treat the underlying cause. If you and your doctor can't determine a cause for your fatigue, you may consider medications such as corticosteroids and antimalarial drugs.
Swelling around the heart and lungs. Swelling around your heart and lungs that causes chest pain may be controlled NSAIDs, antimalarial drugs or corticosteroids.
Treatment for aggressive lupus
Life-threatening cases of lupus — those including kidney problems, inflammation in the blood vessels, and central nervous system problems, such as seizures — may require more aggressive treatment. In these cases, you and your doctor may consider:
High-dose corticosteroids. High-dose corticosteroids can be taken orally or administered through a vein in your arm (intravenously). A high-dose regimen of corticosteroids may help control dangerous signs and symptoms quickly, but can also cause serious side effects, including infections, mood swings, high blood pressure and osteoporosis. To minimize side effects, your doctor will give you the lowest dose needed to control your signs and symptoms and then reduce the dosage over time.
Immunosuppressive drugs. Drugs that suppress the immune system may be helpful in serious cases of lupus, but can cause serious side effects. The most commonly used immunosuppressive drugs include cyclophosphamide (Cytoxan) and azathioprine (Imuran). The drug mycophenolate mofetil (CellCept), another immunosuppressant, can be used to treat lupus-related kidney problems. All three of these drugs can be taken orally, and cyclophosphamide can also be given intravenously. Side effects include an increased risk of infection, liver damage, infertility and an increased risk of cancer.
High-dose corticosteroids can be combined with immunosuppressive drugs to reduce the dosage of each drug, which may reduce the risk of side effects. Sometimes, even with aggressive treatment, your kidneys may fail. In that case, you may need kidney dialysis or, if kidney failure is permanent, a kidney transplant.
Clinical trials
Researchers are developing new treatments for lupus in clinical trials. These studies give people with lupus the chance to try new treatments, but they don't guarantee a cure. If you're interested in a clinical trial, discuss the options with your doctor to determine the benefits and risks.
Treatments being studied in clinical trials include:
Stem cell transplant. A stem cell transplant uses your own adult stem cells to rebuild your immune system. Doctors hope this treatment can help people with life-threatening cases of lupus. Before a stem cell transplant you're given a drug that coaxes the adult stem cells out of your bone marrow and into your bloodstream. The stem cells are then filtered from your blood and frozen for later use. Strong immunosuppressive drugs are administered to wipe out your immune system. Then the adult stem cells are put back into your body where they can rebuild your immune system.
Dehydroepiandrosterone (DHEA). Some clinical trials have shown that a synthetic form of the hormone DHEA may improve quality of life in people with lupus, though others haven't found this. Your body uses DHEA to make male and female sex hormones. Researchers are conducting further clinical trials to better understand whether DHEA may be useful for people with lupus.
Rituximab (Rituxan). Rituximab decreases the number of B cells, a type of white blood cell, in your body and has shown some promise in treating lupus in people who haven't responded to other immunosuppressants. However, rituximab has been linked to a fatal brain infection in two people with lupus.
Self-care
Take steps to care for your body if you have lupus. Simple steps can help you prevent lupus flares and, should they occur, better cope with the signs and symptoms you experience. Try to:
Get adequate rest. People with lupus often experience persistent fatigue that's different from normal tiredness and that isn't necessarily relieved by rest. For that reason, it can be hard to judge when you need to slow down. Many experts recommend eight to 10 hours of sleep a night and naps or breaks during the day as needed. Friends and family members need to understand and respect your need for rest.
Be sun smart. Because ultraviolet light can trigger a flare, wear protective clothing such as a hat, long-sleeved shirt and long pants, and use sunscreens with a sun protection factor (SPF) of at least 15 every time you go outside, even if it's just a quick trip to the mailbox. Be sure that your ears, scalp and the backs of your hands are protected. Avoid tanning beds and stay out of the sun entirely when it's strongest, from 10 a.m. to 4 p.m. Because fluorescent and halogen lights also can emit UV rays and thus aggravate lupus, you may need to wear sunscreen and protective clothing indoors or use plastic devices that block UV emissions from indoor lights.
Get regular exercise. Exercise can help you recover from a flare, reduce your risk of heart attack, help fight depression and promote general well-being. Exercise as much as your body allows — aim for 30 minutes of activity most days of the week. You'll likely feel fatigued and not up to exercising sometimes, and that's OK. Rest when you need to. Time outdoor activities so that you avoid the sun when it's most intense, and if you're having a flare, stay out of the sun entirely.
Don't smoke. Smoking increases your risk of cardiovascular disease and can worsen the effects of lupus on your heart and blood vessels.
Eat a healthy diet. A healthy diet emphasizes fruits, vegetables and whole grains. Sometimes you may have dietary restrictions, especially if you have high blood pressure, kidney damage or gastrointestinal problems. And although no specific foods have been shown to cause or worsen lupus, it's best to avoid any food that seems to make your symptoms worse.
Coping
Coping with lupus can be stressful. People with lupus often experience anxiety, depression and frustration because the disease is unpredictable. Knowing you have a serious disease can also be scary. To help you cope with lupus, try to:
Learn all you can about lupus. Write down all the questions you have about lupus and ask them at your next appointment. Ask your doctor or nurse for reputable sources of further information. The more you know about lupus, the more confident you'll feel in your treatment choices.
Gather support among your friends and family. Talk about lupus with your friends and family. They may have questions about lupus and how it affects your life. Answer their questions honestly. Explain ways your friends and family can help out when you're having flares. Lupus can be frustrating for your loved ones because they usually can't see it and you may not appear sick. They can't tell if you're having a good day or a bad day unless you tell them. Be open about what you're feeling so that your friends and family know what to expect.
Take time for yourself. Cope with stress in your life by taking time for yourself. Use that time to read, meditate, listen to music or write in a journal. Find activities that calm and renew you.
Connect with others who have lupus. Talk to other people who have lupus. You can connect with other people who have lupus through support groups in your community or through online message boards. Though your friends and family love you, sometimes you'll feel as if they can't quite understand what you're feeling. Other people with lupus can offer unique support because they're facing many of the same obstacles and frustrations that you're facing.
Complementary and alternative medicine
If your medications aren't controlling all of your signs and symptoms or if you're frustrated by lupus flares, you might turn to complementary and alternative medicine for solutions. Mainstream doctors are becoming more open to discussing these options with their patients. But, since few of these treatments have been extensively studied in clinical trials, it's difficult to assess whether these treatments are helpful for lupus. In some cases, the risks of these treatments aren't known.
If you're interested in trying complementary and alternative medicine therapies, discuss these treatments with your doctor first. He or she can help you weigh the benefits and risks and tell you if the treatments will interfere with your current lupus medications.
Some common complementary and alternative treatments for lupus include:
Fish oil. Fish oil supplements contain omega-3 fatty acids that may be beneficial for people with lupus. Preliminary studies have found some promise, though more study is needed. Side effects of fish oil supplements can include nausea, belching and a fishy taste in the mouth. Fish oil can interfere with medications, so check with your doctor first.
Flaxseed. Flaxseed contains a fatty acid called alpha-linolenic acid, which may decrease inflammation in the body. Some studies have found that flaxseed may improve kidney function in people who have lupus that affects the kidneys, though more study is needed. Side effects of flaxseed include bloating and abdominal pain. Flaxseed can also interfere with medications, so check with your doctor first.
Other complementary and alternative medicine treatments are available. Discuss the options with your doctor.
By Mayo Clinic Staff
Oct 20, 2007
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