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SYSTEMIC LUPUS ERYTHEMATOSUS OVERVIEW — Systemic lupus erythematosus (also called SLE or just "lupus") is a chronic disease that can affect various parts of the body. Lupus is an autoimmune condition, meaning that your body's immune system (which normally protects the body from infection) attacks your own tissues as though they were foreign. This can lead to pain, swelling, and damage to organs such as the kidneys. The cause of lupus is not well understood; there is likely to be a genetic component (meaning a person's risk is higher if they have family members with the disease), but environmental factors are thought to play a role as well. People with lupus most often start developing symptoms in young adulthood, although it can happen at any age.
People with lupus often have disease "flares," in which symptoms worsen, followed by periods of remission, in which symptoms improve. The frequency of these flares varies from person to person. Symptoms are mild in some people and life-threatening in others. However, treatments are available to relieve symptoms, reduce inflammation, and minimize organ damage.
This article discusses the symptoms, diagnosis, and treatments for lupus in adults. The care of pregnant people with lupus is discussed separately. (See "Patient education: Systemic lupus erythematosus and pregnancy (Beyond the Basics)".)
SYMPTOMS OF SYSTEMIC LUPUS ERYTHEMATOSUS — Symptoms of lupus can be general, affecting the whole body, or related to the effects of inflammation on specific organs.
Whole-body symptoms — Most people with lupus experience fatigue, fever, and weight changes at some point in their illness.
Fatigue — Fatigue is the most common symptom of lupus and can be debilitating. Almost everyone with lupus experiences fatigue at some point, even when there are no other symptoms. However, fatigue can also be due to other reasons that are unrelated to lupus, and your doctor may need to rule out other potential causes.
Fever — Many people with lupus get fevers when their disease flares. Fever is defined as a temperature over 100°F (37.8°C).
Muscle pain — Muscle pain or tenderness is common in people with lupus; rarely, some people also notice muscle weakness.
Weight changes — Lupus can sometimes cause weight loss or weight gain.
●Weight loss may be unintentional and due to decreased appetite or problems with the digestive system (see 'Digestive system' below). It can also be a side effect of some medications used to treat lupus.
●Weight gain may be related to salt and water retention associated with kidney disease (see 'Kidneys' below) or due to increased appetite (which can happen in people who take glucocorticoids).
Specific organ symptoms — Lupus can affect virtually any organ of the body, resulting in a variety of symptoms. It affects different parts of the body in different people.
Joint pain and stiffness — Joint pain and stiffness (arthritis) occur in almost all people with lupus at some point during the disease, and these are often the earliest symptoms. The pain and stiffness tends to move from one part of the body to another and does not usually affect both sides of the body in the same way. Only a few joints are affected at any time, most commonly the fingers, wrists, and knees. Joints may be swollen and warm to the touch.
Skin changes — Two-thirds of people with lupus have skin problems at some point. The most common is a rash, known as the "butterfly rash" because of its shape; this appears as redness over the cheeks and nose after being in the sun. The rash usually lasts only a few days but often comes back.
Some people develop circular patches of raised, scaly skin (called discoid lesions), which often leave a scar (picture 1). Some amount of hair loss (also called alopecia) is common, but total baldness is not. Many people develop ulcers or sores in the mouth, which are usually painless.
Raynaud phenomenon is a condition that causes blood vessels in the fingers and toes to narrow in response to cold temperatures, emotional stress, and/or cigarette smoking. As the blood vessels narrow, less blood flows to the area, causing the fingers or toes to turn pale and then blue, purple, and/or red (picture 2). Raynaud phenomenon is a frequent problem in people with lupus. However, most people who have Raynaud phenomenon do not have lupus. (See "Patient education: Raynaud phenomenon (Beyond the Basics)".)
Sensitivity to sunlight — Many people with lupus are sensitive to ultraviolet (UV) light. This "photosensitivity" causes a skin rash to develop after exposure to UV light from the sun.
You can do the following to help protect your skin:
●Use a broad-spectrum sunscreen with a sun-protection factor (SPF) of 50 or greater every day, even if you don't plan to spend a lot of time outdoors. The sunscreen should be applied 30 to 60 minutes before going outside and should be reapplied every four to six hours.
●Avoid sun exposure during the midday (between 10 AM and 4 PM).
●Avoid medications that may make photosensitivity worse. Your health care provider can talk to you about this if you have questions about your medications or whether a product is safe to use.
More information about sun protection is available separately. (See "Patient education: Sunburn prevention (Beyond the Basics)".)
Kidneys — Changes in kidney function are common in people with lupus. These changes usually develop during the first few years of the illness. Regular monitoring with urine and blood tests can help to detect these changes so they can be treated as soon as possible.
●Lupus can cause inflammation of the glomerulus, the part of the kidney that filters waste and excess fluids (which then exit the body in the form of urine). When this happens, it is called glomerulonephritis or lupus nephritis. Damage to the glomeruli can prevent the kidneys from filtering wastes from the bloodstream; as a result, waste products build up in the bloodstream. (See "Patient education: Glomerular disease (Beyond the Basics)".)
Lupus can also cause inflammation of the interstitium. This is the area around the glomeruli, tubules, vessels, and nerves in the kidney.
●When there is damage to the glomeruli, proteins in the bloodstream can "leak" into the urine. When this becomes severe, it can cause water retention, swelling in the feet and lower legs, and other changes referred to as the nephrotic syndrome. (See "Patient education: The nephrotic syndrome (Beyond the Basics)".)
If lupus nephritis is left untreated, it can lead to permanent scarring in the kidneys. If your kidneys become too damaged to filter the blood properly, you will need dialysis or a kidney transplant. Fortunately, treatments to prevent or minimize lupus-related kidney disease are available.
Digestive system — Digestive symptoms can occur if lupus-related inflammation affects the pancreas (pancreatitis), the lining of the abdomen (peritonitis), or the large intestine (colitis). These can include severe abdominal pain, nausea, and vomiting.
The digestive system can also be affected by medications often used to treat lupus, including glucocorticoids and nonsteroidal antiinflammatory drugs (NSAIDs). These medicines can cause upset stomach or, less commonly, stomach ulcers. (See "Patient education: Nonsteroidal antiinflammatory drugs (NSAIDs) (Beyond the Basics)".)
Lungs — Lung problems in lupus can include:
●Pain with breathing – Pleurisy is a type of chest pain that gets worse when you take a deep breath. This can occur if there is inflammation of the lining covering the lungs and inside of the chest.
●Shortness of breath – Shortness of breath can occur due to several different problems. Collection of fluid in the space around the lungs (called pleural effusion) can make the lung unable to inflate fully. Inflammation of the air sacs of the lung (pneumonitis) or damage and scarring of the supporting tissues between the air sacs (interstitial lung disease) can cause difficulty breathing.
Heart — Lupus can affect different parts of the heart. The most common part that can be affected is the tissue that surrounds the heart called the pericardium. Lupus can cause inflammation of the pericardium and lead to a condition known as pericarditis. People with pericarditis can have chest pain. Less commonly, the valves of the heart or the heart muscle itself can be affected. Lupus is also associated with an increased risk of coronary artery disease.
Nervous system — Lupus can affect the nervous system in many ways. These problems may be related to lupus itself or to treatments used for lupus. Some of the most common problems affecting the nervous system include:
●Difficulty concentrating and thinking clearly
●Confusion or memory loss
●Depression and anxiety
●Burning or tingling in the hands or feet (called peripheral neuropathy)
●Weakness or numbness
There are treatments that can help with lupus-related nervous system problems.
Eyes — Lupus can affect the eyes. The most common symptom is dryness of the eyes with a feeling of grittiness or a lack of tearing. This can be treated by using over-the-counter "artificial tears" (a liquid, gel, or ointment that can help relieve the symptoms of dry eye).
It's important to get routine eye exams if you take a medication called hydroxychloroquine to make sure you don't develop damage to the retina. In addition, if you need to take glucocorticoids for a long time, it is also important to have your eyes checked regularly to monitor for cataracts or glaucoma. (See 'Medication therapy' below.)
When to call your doctor — If you have lupus, you will see a specialist called a rheumatologist regularly (see 'Systemic lupus erythematosus treatment' below). They will monitor your health and response to treatment.
Your rheumatologist will also tell you when you should call or seek help between visits, for example, if you develop a fever or notice any new, different, or severe symptoms (such as a new rash or hair loss, mouth ulcers, significant joint swelling, swelling of the ankles, or shortness of breath).
SYSTEMIC LUPUS ERYTHEMATOSUS DIAGNOSIS — To diagnose lupus, an experienced doctor will take a history, do a physical exam, learn about your symptoms, and order blood and urine tests. Then they will determine whether your symptoms are likely due to lupus or another condition. If it's not clear, they may order additional tests, such as imaging tests or biopsy (for example, if the skin or kidneys are affected).
Professional medical societies have created specific criteria to classify patients with lupus. The criteria vary, but they all involve confirming that a person has a minimum number of symptoms common to lupus as well as certain laboratory test results. While these criteria were developed to aid in research, they are sometimes used in medical practice to help with the diagnostic process.
SYSTEMIC LUPUS ERYTHEMATOSUS TREATMENT — Although there is no cure for lupus, a variety of treatments can reduce symptoms, limit damage to vital organs, and reduce the risk of recurrence.
It's important to see your lupus provider regularly. At these visits, your provider will ask about your symptoms, do an exam, and order laboratory tests. This way they can tell how active your disease is and whether you need any changes to your treatment plan. The frequency of these visits will depend on the frequency and severity of your current and past symptoms.
It is also important to maintain visits with your primary care provider for an annual physical as well as other routine checks for health problems. Depending on your situation, these may include bone density screening; screening for hyperlipidemia, hypertension, and diabetes; and/or screening for issues such as cervical and breast cancer.
Lifestyle changes and preventive interventions — There are a number of things you can do to help manage your disease.
Sun protection — Since exposure to ultraviolet (UV) light can cause or worsen lupus symptoms, it's important to protect yourself from the sun. This includes wearing sunscreen and avoiding direct sun exposure when possible. (See 'Sensitivity to sunlight' above.)
Diet and nutrition — Most people with lupus do not require a special diet but should instead eat a well-balanced diet. A well-balanced diet is one that is low in fat; high in fruits, vegetables, and whole grains; and contains a moderate amount of meat, poultry, and fish.
However, you may need to make changes to your diet depending upon how lupus has affected your body. Your health care provider can talk to you about your situation and whether you should modify your diet; do not make any drastic changes without speaking with your provider first.
●Glucocorticoids (such as prednisone), which are often used to treat lupus, increase appetite; this can lead to weight gain. If you notice this, try to avoid overeating and stay active.
●If you have high cholesterol or triglycerides, you may be advised to change your diet to help manage this. (See "Patient education: High cholesterol and lipids (Beyond the Basics)".)
●If you have swelling (edema) in your feet or lower legs, decrease the amount of salt and sodium in your diet. (See "Patient education: Low-sodium diet (Beyond the Basics)".)
●Extra vitamins are rarely needed if you eat a balanced diet. If you are not able to eat a balanced diet or are dieting to lose weight, you should take a daily multivitamin.
●If you take glucocorticoids every day or are a woman who has been through menopause, your provider will likely prescribe calcium and vitamin D supplements to minimize bone loss (which can increase the risk of fractures). (See "Patient education: Calcium and vitamin D for bone health (Beyond the Basics)".)
●Drinking a moderate amount of alcohol (no more than one drink per day for females or two drinks per day for males) is usually safe for people with lupus. However, alcohol can interact with medications used to treat lupus. Talk to your health care provider if you have questions.
●Herbal and other dietary supplements are not recommended and may even cause harm. If you are curious about a particular vitamin or supplement, talk with your provider.
Exercise — It can be challenging to exercise when your lupus causes fatigue and other symptoms (such as breathing problems). But being inactive can cause you to lose muscle strength, which can make you feel worse in the long term. Even small amounts of gentle movement can be beneficial for your health. Preferred activities include those that are lower impact and result in strengthening and stretching of muscles, such as walking, tai chi, yoga, Pilates, low-impact aerobics, and swimming. Advice about how to incorporate exercise into your life is available separately. (See "Patient education: Arthritis and exercise (Beyond the Basics)".)
Avoiding smoking — Cigarette smoking has been associated with symptom flares in people with lupus, and has many other negative health effects. Quitting smoking is difficult, but your health care provider can help. (See "Patient education: Quitting smoking (Beyond the Basics)".)
Vaccines — Vaccines to prevent pneumonia, flu, and coronavirus disease 2019 (COVID-19) are recommended for people with lupus. Some people should also get the shingles vaccine.
By contrast, vaccines that contain live viruses (eg, measles, mumps, rubella, polio, varicella, and smallpox) are not recommended for people with lupus, especially those taking immunosuppressive therapies such as prednisone. (See "Patient education: Vaccines for adults (Beyond the Basics)".)
Possible medication allergy — Allergies to antibiotics are more common in people with lupus, especially antibiotics that contain sulfonamide ("sulfa"). Make sure any health care provider who treats you for anything is aware that you have lupus so they can prescribe a medication that is safe for you.
Medication therapy — A number of different medications are used in the treatment of lupus, including nonsteroidal antiinflammatory drugs (NSAIDs), hydroxychloroquine, glucocorticoids (also called steroids), and immunosuppressive agents.
The choice of medications for lupus is highly individualized and guided by which organs are affected as well as how severe symptoms are.
Hydroxychloroquine — Everyone with lupus should be treated with daily hydroxychloroquine (brand name: Plaquenil) unless this medication is otherwise contraindicated. The benefits of this medication in lupus are broad and include relief of whole-body symptoms (such as fatigue and fever), musculoskeletal symptoms, and skin symptoms, as well as a reduction in the number of flares.
Nonsteroidal antiinflammatory drugs (NSAIDs) — NSAIDs can relieve joint pain caused by lupus-related pain and inflammation. NSAIDs include ibuprofen (sample brand names: Advil, Motrin) and naproxen (sample brand names: Aleve, Naprosyn).
Immunosuppressives — People with lupus affecting the internal organs (eg, involvement of the kidney, heart, lungs, or central nervous system) usually require treatment with glucocorticoids (steroids).
Glucocorticoids may be used alone or in combination with medications that suppress the immune system (called "immunosuppressive" medications). Examples of immunosuppressive medicines include mycophenolate, cyclophosphamide, azathioprine, and methotrexate.
The benefit of these medications must be weighed against the risks because these treatments have potentially serious side effects. Glucocorticoids can cause weight gain, worsened diabetes, thinning of bones (osteoporosis), and an increased risk of infection. Providers usually try to avoid long-term glucocorticoid use when possible.
Other medications — In some cases, doctors prescribe "targeted therapies" such as belimumab (brand name: Benlysta) or rituximab (brand name: Rituxan). These medications are generally reserved for people with significant organ damage, particularly of the kidneys, blood, lungs, or central nervous system, or if glucocorticoids and immunosuppressive therapy are not effective.
A number of other treatment approaches for lupus are being actively investigated as well.
Pregnancy — Pregnant people with lupus are at increased risk of miscarriage; however, the majority of people with lupus who get pregnant are able to carry to term. Miscarriage occurs more often in people with active disease, especially if there has been related organ damage; other issues sometimes associated with lupus, such as the presence of certain proteins in the body (called "antiphospholipid antibodies"), also increase the risk of miscarriage. Your doctors can guide you on how to maximize your chances of having a healthy pregnancy and baby. In most cases, a person should wait until their lupus has been under control (ie, no flares) for at least six months before trying to get pregnant.
More detailed information about pregnancy in people with lupus is available separately. (See "Patient education: Systemic lupus erythematosus and pregnancy (Beyond the Basics)".)
Family planning and contraception — Family planning is an important consideration since many people with lupus are of reproductive age. It is important to discuss with your provider whether or not you want to become pregnant, and whether you think you might want to get pregnant in the future. Also, there are some medications used to treat lupus that are not safe to take during pregnancy. The best choice of contraception depends on your symptoms, risk factors, medications, and personal preference. Your gynecologist and rheumatologist can help you find something that works for you. Options may include:
●An intrauterine device (IUD) – An IUD is a small device that is placed in the uterus to prevent pregnancy. While a number of IUDs are available, those that release the hormone progestin are often recommended for people with lupus who want a long-term method of birth control. (See "Patient education: Long-acting methods of birth control (Beyond the Basics)".)
●Birth control pills – Most birth control pills contain two hormones, estrogen and progestin. These are safe for many people with mild forms of lupus. However, some people (eg, those at an increased risk of blood clots) probably should not take any medicines that contain estrogen. For these people, the progestin-only birth control pill (sometimes called the "mini pill") may be a good option. (See "Patient education: Hormonal methods of birth control (Beyond the Basics)".)
●Injectable birth control – The birth control shot (brand name: Depo-Provera) contains progestin and is injected into a muscle (usually the buttock or upper arm). It may be an option for some people with lupus, although it should not be used long-term in people who have osteoporosis or are taking glucocorticoids. (See "Patient education: Hormonal methods of birth control (Beyond the Basics)".)
Certain other birth control methods, including the skin patch, implant, and vaginal ring, are not usually recommended for people with lupus because the amount of estrogen they contain may increase the risk of blood clots.
Some medications often used to treat lupus can interfere with birth control, making one or both medications less effective. Your providers can review your current medications with you in order to help you choose a birth control option that will be safe and effective.
WHAT WILL MY LIFE BE LIKE? — It is hard to predict exactly how lupus will affect an individual's health and life. Lupus can cause a wide spectrum of symptoms; in some people, it is relatively mild, while in others, it is a severe, debilitating illness.
The life expectancy for people with lupus has dramatically increased over the last several decades. The improvement in survival is probably due to multiple factors, including the ability to recognize the disease earlier with more sensitive diagnostic tests, to begin treatment earlier, and to promptly treat complications.
Some people go into remission and require no treatment, sometimes even for years.
CLINICAL TRIALS — Researchers are continually conducting clinical trials of lupus treatments to find better ways of treating the disease. A clinical trial is a carefully controlled way to study the effectiveness of new treatments or new combinations of known therapies. For more information about clinical trials, visit http://clinicaltrials.gov or http://clinicalstudies.info.nih.gov.
WHERE TO GET MORE INFORMATION — Your healthcare provider is the best source of information for questions and concerns related to your medical problem.
This article will be updated as needed on our web site (www.uptodate.com/patients). Related topics for patients, as well as selected articles written for healthcare professionals, are also available. Some of the most relevant are listed below.
Patient level information — UpToDate offers two types of patient education materials.
The Basics — The Basics patient education pieces answer the four or five key questions a patient might have about a given condition. These articles are best for patients who want a general overview and who prefer short, easy-to-read materials.
Patient education: Lupus (The Basics)
Patient education: Lupus and pregnancy (The Basics)
Patient education: Pericarditis in children (The Basics)
Patient education: Lupus and kidney disease (The Basics)
Patient education: Antinuclear antibodies (The Basics)
Patient education: Disease-modifying antirheumatic drugs (DMARDs) (The Basics)
Patient education: Interstitial lung disease (The Basics)
Patient education: Discoid lupus (The Basics)
Beyond the Basics — Beyond the Basics patient education pieces are longer, more sophisticated, and more detailed. These articles are best for patients who want in-depth information and are comfortable with some medical jargon.
Patient education: Systemic lupus erythematosus and pregnancy (Beyond the Basics)
Patient education: Raynaud phenomenon (Beyond the Basics)
Patient education: Sunburn prevention (Beyond the Basics)
Patient education: Glomerular disease (Beyond the Basics)
Patient education: The nephrotic syndrome (Beyond the Basics)
Patient education: Nonsteroidal antiinflammatory drugs (NSAIDs) (Beyond the Basics)
Patient education: High cholesterol and lipids (Beyond the Basics)
Patient education: Low-sodium diet (Beyond the Basics)
Patient education: Calcium and vitamin D for bone health (Beyond the Basics)
Patient education: Arthritis and exercise (Beyond the Basics)
Patient education: Vaccines for adults (Beyond the Basics)
Professional level information — Professional level articles are designed to keep doctors and other health professionals up-to-date on the latest medical findings. These articles are thorough, long, and complex, and they contain multiple references to the research on which they are based. Professional level articles are best for people who are comfortable with a lot of medical terminology and who want to read the same materials their doctors are reading.
Antibodies to double-stranded (ds)DNA, Sm, and U1 RNP
Lupus nephritis: Therapy of lupus membranous nephropathy
Coronary heart disease in systemic lupus erythematosus
Clinical manifestations and diagnosis of systemic lupus erythematosus in adults
Kidney transplantation in adults: Issues related to lupus nephritis
Epidemiology and pathogenesis of systemic lupus erythematosus
Gastrointestinal manifestations of systemic lupus erythematosus
Hematologic manifestations of systemic lupus erythematosus
Overview of cutaneous lupus erythematosus
Arthritis and other musculoskeletal manifestations of systemic lupus erythematosus
Neonatal lupus: Epidemiology, pathogenesis, clinical manifestations, and diagnosis
Manifestations of systemic lupus erythematosus affecting the peripheral nervous system
Neurologic and neuropsychiatric manifestations of systemic lupus erythematosus
Non-coronary cardiac manifestations of systemic lupus erythematosus in adults
Overview of the management and prognosis of systemic lupus erythematosus in adults
Pregnancy in women with systemic lupus erythematosus
Pulmonary manifestations of systemic lupus erythematosus in adults
Lupus nephritis: Initial and subsequent therapy for focal or diffuse lupus nephritis
Lupus nephritis: Treatment of focal or diffuse lupus nephritis resistant to initial therapy
Lupus nephritis: Diagnosis and classification
Clinical manifestations and diagnosis of systemic lupus erythematosus in adults, section on 'Information for patients'
The following organizations also provide reliable health information.
●National Library of Medicine
●National Institute of Arthritis and Musculoskeletal and Skin Disease
●American College of Rheumatology
●Lupus Foundation of America
●Lupus Research Alliance
ACKNOWLEDGMENT — The editorial staff at UpToDate would like to acknowledge Peter Schur, MD, who contributed to an earlier version of this topic review.
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