Systemic lupus erythematosus (SLE)

Systemic lupus erythematosus (SLE)

Author
Peter H Schur, MD
Section Editor
Robert H Shmerling, MD
Deputy Editor
Paul L Romain, MD

Disclosures

SYSTEMIC LUPUS ERYTHEMATOSUS OVERVIEW — Systemic lupus erythematosus (also known as lupus or SLE) is a chronic inflammatory disease that can affect various parts of the body. Lupus is an autoimmune condition, meaning that your body’s immune system attacks your own tissues, thinking that they are foreign. This can lead to pain, swelling, and damage to organs such as the kidneys. The cause of lupus is not clear.

People with lupus often have disease flares, when symptoms worsen, followed by a period of remission, when symptoms improve. Lupus is mild in some people and life-threatening in others. However, treatments are available to reduce symptoms, reverse inflammation, and minimize organ damage.

This article discusses the symptoms, diagnosis, and treatments for lupus in adults. The treatment of lupus in pregnant women is discussed in a separate article. (See “Patient information: Systemic lupus erythematosus and pregnancy”.)

LUPUS CAUSES — The exact cause of lupus is not known. What is known is that the disease tends to run in families and affects certain groups more than others, suggesting that genetic factors are important. Although research has been done to identify environmental factors and infections that might cause the disease, no cause has been consistently found.

A person who develops lupus probably inherits the risk from one or both parents and then develops the disease when exposed to a trigger. Triggers may include exposure to sunlight, being ill with an infection, having surgery, or being pregnant.

SYMPTOMS OF SYSTEMIC LUPUS ERYTHEMATOSUS — Symptoms of lupus can be caused by inflammation, which affects the whole body. Other lupus symptoms are caused by damage to a particular organ system.

Whole body symptoms can include fatigue and weight loss.
Organ-related symptoms include skin rash following sun exposure and easy bruising due to decreased numbers of platelets in the blood.
Whole body symptoms — Most people with lupus develop fatigue, fever, and weight loss occur at some point in their illness.

Fatigue — Fatigue is the most common symptom of lupus, and it is occasionally the most debilitating. It occurs in almost everyone, even when there are no other symptoms of lupus.

Fatigue may be caused by lupus itself, or by underlying issues, such as: depression, unhealthy habits (smoking, unhealthy diet, drug abuse), stress, anemia, use of certain medications, underlying health problems, difficulty sleeping, and/or lack of exercise.

Problems with sleep are usually treated with medicine, and an exercise program can improve energy levels (see ‘Exercise’ below).

Weight changes — Systemic lupus erythematosus can lead to both weight loss and weight gain. Unintentional weight loss is often due to:

Decreased appetite
The side effects of medications
Gastrointestinal disease
Weight gain is usually due to one of two factors:

Salt and water retention associated with kidney disease (see ‘Kidneys’ below)
Increased appetite associated with the use of glucocorticoids (steroids)
Fever — Most people with systemic lupus erythematosus have fevers. Fever related to active lupus usually responds to nonsteroidal anti-inflammatory drugs (NSAIDs) and/or acetaminophen. If fever does not improve with these treatments, call your healthcare provider.

Specific organ symptoms — Systemic lupus erythematosus can affect many organs of the body and can result in a variety of symptoms.

Joint pain and stiffness — Joint pain and stiffness occurs in almost all patients with lupus, and these are often the earliest symptom of lupus. 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. (See “Musculoskeletal manifestations of systemic lupus erythematosus”.)

Skin changes — Most people with systemic lupus erythematosus have skin abnormalities at some time. The most common is a rash, known as the butterfly rash; this appears as redness over the cheeks and nose after being in the sun (picture 1). The rash usually lasts only a few days, but often comes back. (See “Mucocutaneous manifestations of systemic lupus erythematosus”.)

Some people develop circular patches of raised, scaly skin (called discoid lesions), which have a tendency to scar (picture 2). Hair loss (alopecia) is common, but baldness is not. Many people develop ulcers in the mouth, which are usually painless.

The Raynaud phenomenon that causes blood vessels in the fingers and toes to narrow in response to cold temperatures, emotional stress, smoking cigarettes, and/or caffeine (figure 1). As the blood vessels narrow, less blood flows to the area, causing the fingers or toes to become pale, then blue, and/or red. Raynaud is a frequent problem in patients with systemic lupus erythematosus. (See “Patient information: Raynaud phenomenon”.)

Light sensitivity — Between 60 and 100 percent of people with lupus are sensitive to ultraviolet light. This “photosensitivity” causes the person to develop a rash after exposure to ultraviolet light from the sun or fluorescent lights. Some people are also sensitive to UV-A radiation (from sunlight). Glass protects individuals sensitive to UV-B (from sunlight or fluorescent light), but only partially protects those sensitive to UV-A. Blonde, blue eyed, fair skinned individuals are much more photosensitive than brunettes or individuals with darker skin.
Those who are photosensitive should do the following to minimize UV exposure:

Avoid areas of high sun exposure (beaches, snow, lakes), especially between 10 AM and 3 PM
Avoid medications that may cause photosensitivity.
Use a sunscreen with a sun-protection factor (SPF) of 50 or greater daily. The sunscreen should be applied 30 to 60 minutes before going outside and reapplied every four to six hours.
For more information about ways to minimize UV exposure, (see “Patient information: Sunburn prevention”).

Kidneys — Changes in kidney function are common in people with lupus. These changes usually develop during the first few years of the illness. Monitoring for changes in kidney function with urine and blood tests can help to detect these changes.

Lupus can cause inflammation of the filtering device in the kidneys (the glomerulus); this is called glomerulonephritis or lupus nephritis. Damage to the glomeruli can prevent the kidneys from filtering wastes from the bloodstream. (See “Patient information: Glomerular disease overview” and “Types of renal disease in systemic lupus erythematosus”.)
Initially, the kidneys may “leak” protein from the blood into the urine. When severe, this can cause water retention, swelling in the feet and lower legs, and other changes referred to as the nephrotic syndrome. (See “Patient information: The nephrotic syndrome”.)
Left untreated, lupus nephritis can lead to permanent scarring in the kidneys. If the kidneys are 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 — The digestive system can be affected by medications often used to treat lupus, including glucocorticoids and NSAIDs. These medicines can cause upset stomach or less commonly, stomach ulcers. (See “Patient information: Nonsteroidal antiinflammatory drugs (NSAIDs)”.)

However, severe abdominal pain, nausea, and vomiting can occur if lupus affects the pancreas (pancreatitis), the lining of the abdomen (peritonitis), or the large intestine (colitis).

Lungs — A number of lung diseases can occur in people with lupus: (see “Pulmonary manifestations of systemic lupus erythematosus in adults”).

Pain with breathing — Pleurisy is a type of chest pain that is worsened by taking a deep breath. Pleurisy 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 interfere with expansion of the lung. Inflammation of the air sacs of the lung (pneumonitis) or dysfunction and scarring of the supporting tissues between the air sacs (interstitial lung disease) can cause difficulty breathing.
Heart — There are a variety of symptoms that can develop if lupus affects the heart or blood vessels:

Chest pain with exercise — As mentioned above, people with lupus can develop chest pain as a result of pleurisy. In addition, lupus can increase the risk of developing coronary artery disease. Symptoms of coronary artery disease can include pain with exercise or emotional stress that resolves with rest (angina pectoris). Sudden severe chest pain or pressure that does not go away within a few minutes may be a sign of a heart attack (myocardial infarction).
Chest pain due inflammation around the heart — Chest pain can also occur if lupus causes inflammation of the sac that surrounds the heart, a condition known as pericarditis.
Shortness of breath due to heart valve disease — Shortness of breath can be caused by heart as well as lung involvement. The heart valves can become leaky or narrowed as a result of damage to the lining of the heart cavity and surfaces of the normally smooth valves (endocardium).
Nervous system — Lupus can affect the nervous system in many ways. These problems may be related to lupus itself, to treatments used for lupus, or to unknown causes. Some of the most common problems affecting the nervous system include:

Difficulty concentrating and thinking clearly
Confusion or memory loss
Depression and anxiety
Headaches
Seizures
Pain in the hands or feet (called peripheral neuropathy)
Weakness or numbness
Treatments are available for lupus-related nervous system problems. (See “Neurologic manifestations of systemic lupus erythematosus” and “Neuropsychiatric manifestations of systemic lupus erythematosus”.)

Eye — The eye is frequently affected by lupus. The most common symptom is dryness of the eyes with a feeling of grittiness or lack of tearing (called keratoconjunctivitis sicca). This can be treated by using artificial tears.

SYSTEMIC LUPUS ERYTHEMATOSUS DIAGNOSIS — The American College of Rheumatology has created criteria to classify patients with systemic lupus erythematosus. While these criteria were developed to aid in research, they are often used in medical practice to help with the diagnostic process. These criteria are shown in the table (table 1) [1]. (See “Diagnosis and differential diagnosis of systemic lupus erythematosus in adults”.)

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.

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 and 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. If you have questions about your diet, discuss these with your healthcare provider. In general:

People with active lupus and fever may require more calories.
Glucocorticoids (prednisone) increase appetite, potentially causing you to gain a lot of weight. Try to control your appetite and stay active. Weigh yourself daily while taking prednisone and talk to your healthcare provider if you gain more than 5 pounds.
If your cholesterol or triglyceride levels become elevated, you may be advised to eat a diet that is low in fat. (See “Patient information: High cholesterol and lipids (hyperlipidemia)”.)
If you have swelling (edema) in your feet or lower legs, decrease the amount of salt and sodium in your diet. (See “Patient information: Low sodium diet”.)
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 multivitamin.
If you take glucocorticoids every day or are a postmenopausal woman, you should take 1000 to 1500 mg of calcium and 400 to 800 units of vitamin D per day to minimize bone loss. (See “Patient information: Calcium and vitamin D for bone health”.)
Drinking a moderate amount of alcohol (one drink or less for women and two drinks or less per day for men) is usually safe for people with lupus. However, alcohol can interact with medications used to treat lupus. Talk to your healthcare provider if you have questions.
Herbal and other dietary supplements not recommended and may even cause harm.
Exercise — Being inactive while ill can cause you to lose muscle and energy quickly. A separate article discusses how to incorporate exercise into your life. (See “Patient information: Arthritis and exercise”.)

Immunizations — Vaccines to prevent pneumonia and the flu are recommended for people with lupus. However, these vaccines may be less effective in people with lupus compared to those without lupus.

In contrast, vaccines that contain live viruses (eg, measles, mumps, rubella, polio, varicella, and smallpox) are not recommended for people with lupus, especially if you currently take prednisone. (See “Patient information: Adult vaccines”.)

Medication precautions — A number of medications are known to worsen lupus. You should not take these medications if there is an acceptable alternative. Sulfa-containing antibiotics and penicillin are examples of medicines that should be avoided.

Birth control pills that contain low doses of estrogen (which includes most birth control pills) are safe for most women with systemic lupus erythematosus. However, certain people (eg, those with Raynaud phenomenon, an increased risk of blood clots, kidney disease) probably should not take any medicines that contain estrogen. (See “Menstrual function; menopause; and hormonal contraceptives in women with systemic lupus erythematosus”.)

Pregnancy — Women with lupus should avoid becoming pregnant during lupus flares due to the high risk of miscarriage. This is especially true in women with significant lupus-related organ damage.

The chances of having an uncomplicated pregnancy and healthy newborn are improved by waiting to become pregnant until lupus has been under control for at least six months. (See “Patient information: Systemic lupus erythematosus and pregnancy”.)

Treatment of specific organs — A number of medications are commonly used in the treatment of lupus, including nonsteroidal anti-inflammatory drugs (NSAIDs), antimalarials, glucocorticoids, and immunosuppressive agents.

Joint pain — A group of medicines, called nonsteroidal antiinflammatory drugs (NSAIDs) can usually relieve joint pain caused by to lupus-related arthritis and inflammation. NSAIDs include ibuprofen (Advil®, Motrin®), or naproxen (Aleve®, Naprosyn®).

Skin symptoms — Antimalarial medications were originally designed as treatments for malaria, but were found to be useful for people with lupus. An example of an antimalarial drug is hydroxychloroquine (Plaquenil®). Antimalarials may be especially helpful for people with skin symptoms and joint pain that have not fully responded to NSAIDs. Antimalarial therapy may also help to protect the body from lupus-related organ damage [2].

Glucocorticoids and immunosuppressives — Glucocorticoids (also called steroids) may be used alone or in combination with medications that suppress the immune system (called immunosuppressive medicines). Examples of immunosuppressive medicines include mycophenolate, cyclophosphamide, azathioprine, and belimumab. These treatments are generally reserved for people with significant organ damage, particularly of the kidneys, blood, lungs, or nervous system or those needing high doses of glucocorticoids to treat their condition.

The benefit of glucocorticoids and immunosuppressive 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 (osteopenia and osteoporosis), and an increased risk of infection.

Other options — A number of other treatment approaches for lupus have been tried or are under investigation. These include stem cell transplantation (bone marrow transplantation), anti-B cell antibodies (including rituximab and epratuzumab), and others.

HOW WILL SYSTEMIC LUPUS ERYTHEMATOSUS AFFECT MY 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 survival rate of people with systemic lupus erythematosus has dramatically increased over the last several decades from approximately 40 percent still alive at five years in the 1950s to current survival rates of approximately 90 percent at 10 years. The improvement in survival is probably due to multiple factors, including the ability to recognize the disease earlier with more sensitive diagnostic tests, begin treatment earlier, and promptly treat complications.

Many people go into remission and require no treatment. In one study, approximately 25 percent of patients had remission lasting for at least a year. Remission occurred in 50 percent of those with disease over 18 years’ duration, and in 75 percent of those with disease over 30 years duration. Remission was even seen in some patients who had had severe kidney disease [3].

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 file://clinicaltrials.gov or file://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.

 

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:

Patient information: Systemic lupus erythematosus and pregnancy
Patient information: Raynaud phenomenon
Patient information: Sunburn prevention
Patient information: Glomerular disease overview
Patient information: The nephrotic syndrome
Patient information: Nonsteroidal antiinflammatory drugs (NSAIDs)
Patient information: High cholesterol and lipids (hyperlipidemia)
Patient information: Low sodium diet
Patient information: Calcium and vitamin D for bone health
Patient information: Arthritis and exercise
Patient information: Adult vaccines

Professional Level Information:

Antibodies to DNA, Sm, and RNP
Approach to the patient with macular skin lesions
Clinical features and therapy of membranous lupus nephritis
Coronary heart disease in systemic lupus erythematosus
Diagnosis and differential diagnosis of systemic lupus erythematosus in adults
Diagnostic approach to the neuropsychiatric manifestations of systemic lupus erythematosus
Drug-induced lupus
End-stage renal disease due to lupus nephritis
Epidemiology and pathogenesis of systemic lupus erythematosus
Gastrointestinal manifestations of systemic lupus erythematosus
Hematologic manifestations of systemic lupus erythematosus in adults
Menstrual function; menopause; and hormonal contraceptives in women with systemic lupus erythematosus
Mucocutaneous manifestations of systemic lupus erythematosus
Musculoskeletal manifestations of systemic lupus erythematosus
Neonatal lupus
Neurologic manifestations of systemic lupus erythematosus
Neuropsychiatric manifestations of systemic lupus erythematosus
Non-coronary cardiac manifestations of systemic lupus erythematosus in adults
Overview of the clinical manifestations of systemic lupus erythematosus in adults
Overview of the therapy and prognosis of systemic lupus erythematosus in adults
Pregnancy in women with systemic lupus erythematosus
Pulmonary manifestations of systemic lupus erythematosus in adults
Therapy of diffuse or focal proliferative lupus nephritis
Therapy of resistant or relapsing diffuse or focal proliferative lupus nephritis
Types of renal disease in systemic lupus erythematosus

The following organizations also provide reliable health information.

National Library of Medicine
(www.nlm.nih.gov/medlineplus/healthtopics.html)
National Institute of Arthritis and Musculoskeletal and Skin Disease
(www.niams.nih.gov)
American College of Rheumatology
(www.rheumatology.org)
Arthritis Foundation
(www.arthritis.org)
Lupus Foundation of America
(www.lupus.org)
S.L.E. Lupus Foundation and the Lupus Research Institute
(www.lupusny.org and lupusresearchinstitute.org)
Alliance for Lupus Research
(www.lupusresearch.org)

 

REFERENCES
Hochberg MC. Updating the American College of Rheumatology revised criteria for the classification of systemic lupus erythematosus. Arthritis Rheum 1997; 40:1725.
Fessler BJ, Alarcón GS, McGwin G Jr, et al. Systemic lupus erythematosus in three ethnic groups: XVI. Association of hydroxychloroquine use with reduced risk of damage accrual. Arthritis Rheum 2005; 52:1473.
Urowitz MB, Feletar M, Bruce IN, et al. Prolonged remission in systemic lupus erythematosus. J Rheumatol 2005; 32:1467.
Chambers SA, Rahman A, Isenberg DA. Treatment adherence and clinical outcome in systemic lupus erythematosus. Rheumatology (Oxford) 2007; 46:895.
Smith KG, Jones RB, Burns SM, Jayne DR. Long-term comparison of rituximab treatment for refractory systemic lupus erythematosus and vasculitis: Remission, relapse, and re-treatment. Arthritis Rheum 2006; 54:2970.