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Systemic lupus erythematosus
Description
An in-depth report on the causes, diagnosis, treatment, and prevention of lupus.
Alternative Names
Lupus
Highlights
Systematic Lupus Erythematosus (SLE) Overview
SLE is an autoimmune disease that causes a chronic inflammatory condition. The inflammation triggered
by SLE can affect many organs in the body, including skin, joints, kidneys, lung, and nervous system.
Women (especially African-American and Asian women) have a higher risk than men for developing SLE.
Symptoms and Diagnosis
SLE has a wide range of symptoms. The most common symptoms are joint pain, skin rash, and fever.
Symptoms can develop slowly or appear suddenly. Many patients with SLE have flares, in which
symptoms suddenly worsen and then settle down for long periods of time. Diagnosing SLE is
complicated because symptoms vary widely and can resemble other conditions. A doctor will base an
SLE diagnosis on certain specific criteria including symptom history and the results of blood tests for
antinuclear antibodies.
Treatment
No drug can cure SLE, but many different drugs can help control symptoms and relieve discomfort. The
choice of drugs depends on the severity of the condition as well as other factors. Patients with mild SLE
may be helped by nonsteroidal anti-inflammatory drugs (NSAIDs) while patients with more severe SLE
may require corticosteroids or immunosuppressants. Belimumab (Benlysta) is a biologic drug that is
used along with standard drug treatments for patients with active lupus. Researchers are working to
develop new drugs and treatments for SLE.
Living with SLE
Patients can make lifestyle changes to help cope with SLE. These include:
Avoide excessive sunlight exposure, and wear sunscreen (ultraviolet light is the one of the main triggers
of flares)
Get plenty of rest (fatigue is another common SLE symptom)
Engage in regular light-to-moderate exercise to help fight fatigue and heart disease, and to keep joints
flexible
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Don't smoke and avoid exposure to second-hand tobacco smoke
Guidelines for Lupus Nephritis
In 2012, the American College of Rheumatology issued updated guidelines for the treatment of lupus-
associated kidney disease (lupus nephritis). Recommendations include:
All patients with signs of lupus nephritis should receive a kidney biopsy to evaluate and classify the
severity of the condition.
Patients with lupus nephritis should be sure to maintain blood pressure at 130/80 mm Hg or below.
For drug therapy, most patients are treated with hydroxychloroquine (Plaquenil, generic). The
immunosuppressants cyclophosphamide (Cytoxan, generic) or mycophenolate mofetil (CellCept,
generic) are recommended for patients with moderate-to-severe lupus nephritis. Immunosuppressants
are not recommended for patients with very mild or very severe lupus nephritis.
Race makes a difference during the induction stage of therapy. Patients who are Hispanic or African
American tend to respond better to mycophenolate. Cyclophosphamide appears better for white
patients of European ethnicity.
Introduction
Systemic lupus erythematosus (SLE) is a chronic, often life-long, autoimmune disease. It can be mild to
severe, and affects mostly women. SLE may affect various parts of the body, but it most often manifests
in the skin, joints, blood, and kidneys. The name describes the disease:
Systemic is used because the disease can affect organs and tissue throughout the body.
Lupus is Latin for wolf. It refers to the rash that extends across the bridge of the nose and upper
cheekbones and was thought to resemble a wolf bite.
Erythematosus is from the Greek word for red and refers to the color of the rash.
There are several different forms of lupus. SLE is the most common type and is the type of lupus that
can lead to serious systemic complications. Other forms of lupus include:
Cutaneous lupus erythematosus refers to lupus that is confined to the skin and does not affect other
parts of the body. About 10% of people with this type of lupus go on to develop SLE.
Discoid lupus erythematosus is a type of cutaneous lupus that produces a potentially scarring disc-
shaped rash on the face, scalp, or ears.
Drug-induced lupus is a temporary and mild form of lupus caused by certain prescription medications.
They include some types of high blood pressure drugs (such as hydralazine, ACE inhibitors, and calcium
channel blockers) and diuretics (hydrochlorothiazide). Symptoms resolve once the medication is
stopped.
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Neonatal lupus is a rare condition that sometimes affects infants born to mothers who have SLE. Babies
with neonatal lupus are born with skin rash, liver problems, and low blood counts and may develop
heart problems.
Causes
Systemic lupus erythematosus is an autoimmune disorder. In a normal immune system, the body
releases proteins (antibodies) to fight viruses, toxins and other potentially harmful foreign substances
(antigens). With lupus and other autoimmune diseases, the immune system does not work properly. It
produces autoantibodies that mistakenly attack and destroy the bodys own healthy cells and tissue.
These autoantibodies also trigger inflammation, which can lead to organ damage.
Autoantibodies called antinuclear antibodies (ANA) are detectable in most, although not all, patients
with SLE. Tests for the presence of ANA are used as part of the diagnostic work-up for the condition. (For
more information, see ANA Tests in Diagnosis section of this report.)
Scientists do not know exactly what causes the abnormal immune response associated withautoimmune disorders. It is most likely a combination of genetic and environmental factors. People who
develop an autoimmune disease may have a genetic predisposition that is triggered by some
environmental factor such as sunlight, stress hormones, or viruses. It does not appear that one gene
alone is responsible for lupus. Researchers estimate that 20 - 100 different genetic factors make a
person susceptible to SLE.
Risk Factors
Gender
About 90% of lupus patients are women, most diagnosed when they are in their childbearing years.Hormones may be an explanation. After menopause, women are only 2.5 times as likely as men to
contract SLE. Flares also become somewhat less common after menopause in women who have chronic
SLE.
Age
Most people develop SLE between the ages of 15 - 44. About 15% of patients experience the onset of
symptoms before age 18.
Race and Ethnicity
African-Americans are three to four times more likely to develop the disease than Caucasians and to
have severe complications. Hispanics and Asians are also more susceptible to the disease.
Family History
A family history plays a strong role in SLE. A brother or sister of a patient with the disorder has 20 times
the risk as someone without an immediate family member with SLE.
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Environmental Triggers
In genetically susceptible people, there are various external factors that can trigger symptoms (flares).
Possible SLE triggers include colds, fatigue, stress, chemicals, sunlight, and certain drugs.
Viruses. Some research suggests an association between Epstein-Barr virus (EBV), the cause of
mononucleosis, and increased risk of lupus, particularly for African-Americans.
Sunlight. Ultraviolet (UV) rays found in sunlight are important SLE triggers. UV light is categorized as UVB
or UVA depending on the length of the wave. Shorter UVB wavelengths cause the most harm.
Smoking. Smoking may be a risk factor for triggering SLE and can increase the risk for skin and kidney
problems in women who have the disease.
Chemicals. While no chemical has been definitively linked to SLE, occupational exposure to crystalline
silica has been studied as a possible trigger. (Silicone breast implants have been investigated as a
possible trigger of autoimmune diseases, including SLE. The weight of evidence to date, however, finds
no support for this concern.) Some prescription medications are associated with a temporary lupus
syndrome (drug-induced lupus), which resolves after these drugs are stopped.
Hormone Replacement Therapy. Premature menopause, and its accompanying symptoms (such as hot
flashes), is common in women with SLE. Hormone replacement therapy (HRT), which is used to relieve
these symptoms, increases the risk for blood clots and heart problems as well as breast cancer. It is not
clear whether HRT triggers SLE flares. Women should discuss with their doctors whether HRT is an
appropriate and safe choice. Guidelines recommend that women who take HRT use the lowest possible
dose for the shortest possible time. Women with SLE who have active disease, antiphospholipid
antibodies, or a history of blood clots or heart disease should not use HRT.
Oral Contraceptives. Female patients with lupus used to be cautioned against taking oral contraceptives
(OCs) due to the possibility that estrogen could trigger lupus flare-ups. However, recent evidence
indicates that OCs are safe, at least for women with inactive or stable lupus. Women who have been
newly diagnosed with lupus should avoid OCs. Lupus can cause complications in its early stages. For this
reason, women should wait until the disease reaches a stable state before taking OCs. In addition,
women who have a history of, or who are at high risk for, blood clots (particularly women with
antiphospholipid syndrome) should not use OCs. The estrogen in OCs increases the risk for blood clots.
Complications
Systemic lupus erythematosus (SLE) can cause complications throughout the body.
Complications of the Blood
Almost 85% of patients with SLE experience problems associated with abnormalities in the blood.
Anemia. About half of patients with SLE are anemic. Causes include:
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Iron deficiencies resulting from excessive menstruation
Iron deficiencies from gastro-intestinal bleeding caused by some of the treatments
A specific anemia called hemolytic anemia, which destroys red blood cells
Anemia of chronic disease
Hemolytic anemia can occur with very high levels of the anticardiolipin antibody. It can be chronic or
develop suddenly and be severely (acute).
Antiphospholipid Syndrome. Between 34 - 42% of patients with SLE have antiphospholipid syndrome
(APS). This is a disorder of blood coagulation related to the presence of autoantibodies called lupus
anticoagulant and anticardiolipin. APS can cause blood clots, which most often occur in the deep veins
of the legs, a condition called deep vein thrombosis. Blood clots increase the risk for stroke and
pulmonary embolism (clots in the lungs). Patients with APS are also at high risk for pregnancy
complications, including miscarriage (see Pregnancy Complications below).
Vasculitis. Vasculitis is an inflammation of the blood vessels. If it becomes severe, it can cause blood to
stop flowing to organs and tissues, resulting in potentially life-threatening complications.
Thrombocytopenia. In thrombocytopenia, antibodies attack and destroy blood platelets. In such cases,
blood clotting is impaired, which causes bruising and bleeding from the skin, nose, gums, or intestines.
Leukopenia and Neutropenia. These conditions cause a drop in the number of white blood cells. They
are very common in lupus, but the condition is usually harmless unless the reductions are so severe that
they leave the patient vulnerable to infections.
Blood Cancers. Patients with SLE and other autoimmune disorders have a greater risk for developing
lymph system cancers such as Hodgkins disease and non-Hodgkins lymphoma (NHL).
Heart Complications
The risk for cardiovascular disease, heart attack, and stroke is much higher than average in patients with
SLE, and heart disease is a primary cause of death. The chronic inflammation associated with SLE can
cause plaque build-up in the hearts arteries (atherosclerosis), whichcan lead to coronary heart disease
and heart attack. SLE also affects blood vessels and circulation. In addition, SLE treatments (particularly
corticosteroids) can affect cholesterol, weight, and other factors that harm the heart. [For more
information, see In-Depth Report #03: Coronary artery disease.].
Patients with SLE have an increased risk for developing the following conditions, which put them at risk
for heart attack or stroke:
Atherosclerosis, or plaque buildup in the arteries
Unhealthy cholesterol and lipid (fatty molecules) levels
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Symptoms vary widely and may overlap with psychiatric or neurologic disorders. They may also be
caused by of some medications used for treating SLE.
The most serious CNS disorder is inflammation of the blood vessels in the brain (CNS vasculitis), which
occurs in about 10% of patients with SLE. Fever, seizures, psychosis, and even coma can occur. Other
CNS side effects include:
Irritability
Emotional disorders (anxiety, depression)
Mild impairment of concentration and memory
Migraine and tension headaches
Problems with the reflex systems, sensation, vision, hearing, and motor control
Infections
Infections are a common complication and a major cause of death in all stages of SLE. Patients are not
only prone to the ordinary bacterial and viral infections, but they are also susceptible to fungal and
parasitic infections, which are common in people with weakened immune systems. They also face an
increased risk for urinary tract, herpes, salmonella, and yeast infections. Corticosteroid and
immunosuppressant drug treatments used for SLE also increase the risk for infections.
Gastrointestinal Complications
Many patients with SLE suffer gastrointestinal problems, including nausea, weight loss, mild abdominal
pain, diarrhea, and gastroesophageal reflux disorder (heartburn). SLE can also affect organs located inthe gastrointestinal system, such as the liver, gallbladder, pancreas, and bile ducts.
Joint, Muscle, and Bone Complications
Patients with SLE often experience muscle aches and weakness. Lupus can also cause pain, stiffness, and
swelling in the joints. However, unlike rheumatoid arthritis, the arthritis caused by SLE almost never
leads to destruction or deformity of joints. Patients with SLE also commonly experience reductions in
bone mass density (osteoporosis) and have a higher risk for fractures, whether or not they are taking
corticosteroids (which can increase the risk for osteoporosis). Women who have SLE should have regular
bone mineral density scans to monitor bone health.
Click the icon to see an image of osteoporosis. Click the icon to see an image of osteoporosis.
Eye Complications
Many patients with SLE have problems with dry eyes. Retinal vascular lesions (blood vessel damage due
to reduced blood flow) are also common and may affect vision. Nerve damage in the eyes can also cause
poor vision as well as droopy eyelids.
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Pregnancy Complications
Women with lupus face a higher risk for pregnancy complications, including miscarriage, premature
birth, and preeclampsia. The risk for miscarriage is highest for patients with antiphospholipid antibodies,
which can cause blood clotting in the placenta. Lupus patients with active kidney disease are at
increased risk for preeclampsia (a pregnancy complication that includes high blood pressure and fluidbuild-up). Pregnant women who take corticosteroids face increased risks of gestational diabetes and
high blood pressure.
Despite these obstacles, many women with lupus have healthy pregnancies and deliver healthy babies.
To increase the odds of a successful pregnancy, it is important for women to plan carefully before
becoming pregnant. (See Pregnancy and SLE in Treatment section of this report.)
Prognosis
SLE is a chronic and relapsing inflammatory disease. It is marked by periods of remission (no symptoms)
that alternate with flares of active disease when symptoms suddenly worsen. Flares tend to diminishafter menopause.
Symptom-free periods can sometimes last for years, but the course of SLE is unpredictable and varies
greatly from person to person. Some patients have a mild form of lupus with occasional skin rashes,
fever, fatigue, or joint and muscle aches. Sometimes lupus remains in a mild form, other times it may
progress to a more severe form. Severe lupus involves serious health complications and extensive
internal organ damage (such as the heart, lungs, kidneys, and brain).
Because of more effective and aggressive treatment, the prognosis for SLE has improved markedly over
the past two decades. Treatment early in the course of the illness that controls the initial inflammation
can help to improve long-term outlook. Over 95% of people with lupus survive at least 10 years, and
many patients have a normal lifespan.
Symptoms
SLE symptoms may develop slowly over months or years, or they may appear suddenly. Symptoms tend
to vary among patients and different symptoms can occur at different times.
Common symptoms of SLE include:
Joint pain and stiffness, which is often accompanied by swelling and redness. The joints most affected
are fingers, wrists, elbows, knees, and ankles.
Skin rash, including the characteristic butterfly rash on the face that extends over the bridge of the
nose and cheeks. Rash can also appear on other parts of the face or other skin areas that are exposed to
sun.
Fever
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Extreme fatigue
Weight loss
Loss of appetite, nausea, and weight loss
Chest pain
Bruising
Menstrual irregularities
Dry eyes
Mouth ulcers
Brittle hair or hair loss
Painful, pale or purple fingers or toes triggered by cold or stress (Raynauds phenomenon)
Anxiety, depression, forgetfulness, and difficulty concentrating
Click the icon to see an image of systemic lupus erythematosus.
Click the icon to see an image of Raynaud's phenomenon.
Conditions with Similar Symptoms
A number of conditions overlap with SLE:
Scleroderma: Hardening of the skin caused by overproduction of collagen
Rheumatoid arthritis: Inflammation of the lining of the joints
Sjgren syndrome: Characterized by dry eyes and dry mouth
Mixed connective tissue disorder: Similar to SLE, but milder
Myositis: Inflammation and degeneration of muscle tissues
Rosacea: Flushed face with pus-filled blisters
Seborrheic dermatitis: Sores on lips and nose
Lichen planus: Swollen rash that itches, typically on scalp, arms, legs, or in the mouth
Dermatomyositis: Bluish-red skin eruptions on face and upper body
Lyme disease: Bulls-eye rash, joint inflammation, and flu-like symptoms
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Diagnosis
SLE can be difficult to diagnose. Symptoms can fluctuate and mimic those of other diseases. A doctor
will make a diagnosis of SLE based on symptoms, medical history, physical exam and blood test for
antinuclear antibodies. The doctor may also order other types of laboratory tests.
The American College of Rheumatology (ACR) has a classification system for helping doctors diagnose, or
exclude, SLE. According to the ACR, at least four of the 11 criteria should be present for a diagnosis of
lupus.
ACR Criteria for Diagnosing Systemic Lupus Erythematosus
1. Butterfly (malar) rash across cheeks and nose
2. Discoid (skin) rash, which appears as scaly raised red patches
3. Photosensitivity
4. Oral (mouth) ulcers
5. Arthritis in two or more joints; joints will have tenderness and swelling but will not have become
deformed
6. Inflammation of the lining around the lungs (pleuritis) or the heart (endocarditis)
7. Evidence of kidney disease
8. Evidence of severe neurologic disease, such as seizures or psychosis
9. Blood disorders, including low red and white blood cell and platelet counts
10. Immunologic abnormalities as evidenced by positive tests for anti-double stranded DNA (anti-
dsDNA), anti-SM, anti-Ro, and anti-LA antibodies
11. Positive antinuclear antibody (ANA) test
Note: A patient must experienced four of the criteria before a doctor can classify the condition as SLE.
These criteria, proposed by the American College of Rheumatology, are not exclusive criteria for
diagnosis, however.
Tests for Autoantibodies (ANA Test)
Antinuclear Antibodies (ANAs). A primary test for SLE checks for antinuclear antibodies (ANA), which
attack the cell nucleus.
High levels of ANA are found in more than 98% of patients with SLE. Other conditions, however, also
cause high levels of ANA, so a positive test is not a definite diagnosis for SLE:
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Antinuclear antibodies may be strongly present in other autoimmune diseases (such as scleroderma,
Sjgren syndrome, or rheumatoid arthritis).
They also may be weakly present in about 20 - 40% of healthy women.
Some drugs can also produce positive antibody tests, including hydralazine, procainamide, isoniazid, and
chlorpromazine.
A negative ANA test makes a diagnosis of SLE unlikely but not impossible. High or low concentrations of
ANA also do not necessarily indicate the severity of the disease, since antibodies tend to come and go in
patients with SLE.
In general, the ANA test is considered a screening test:
If SLE-like symptoms are present and the ANA test is positive, other tests for SLE will be administered.
If SLE-like symptoms are not present and the test is positive, the doctor will look for other causes, or the
results will be ignored if the patient is feeling healthy.
ANA Subtypes. Doctors may also test for specific ANA subtypes.
Anti-double stranded DNA (Anti-ds DNA) is more likely to be found only in patients with SLE. It may play
an important role in injury to blood vessels found in SLE, and high levels often indicate kidney
involvement. Anti-ds DNA levels tend to fluctuate over time and may even disappear.
Anti-Sm antibodies are also usually found only with SLE. Levels are more constant and are more likely to
be detected in African-American patients. Although many lupus patients may not have this antibody, its
presence almost always indicates SLE.
When the ANA is negative but the diagnosis is still strongly suspected, a test for anti-Ro (also called anti-
SSA) and anti-La (also called anti-SSB) antibodies may identify patients with a rare condition called ANA
negative, Ro lupus. These autoantibodies may be involved in the sun-sensitive rashes experienced by
patients with SLE and are also found in association with neonatal lupus syndrome, in which a pregnant
mother's antibodies cross the placenta and cause inflammation in the developing child's skin or heart.
Antiphospholipid Antibodies. Up to half of patients with SLE have antiphospholipid antibodies, which
increase the risk for blood clots, strokes, and pregnancy complications. If a doctor suspects SLE blood
abnormalities, tests may be able to detect the presence of the two major antiphospholipid antibodies:
lupus coagulant antibody and anticardioplin antibody.
As with the ANA, these antibodies have a tendency to appear and disappear. Patients who have these
autoantibodies as well as blood clotting problems or frequent miscarriages are diagnosed with
antiphospholipid syndrome (APS), which often occurs in SLE but can also develop independently.
Other Blood Tests
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Complement. Blood tests of patients with SLE often show low levels of serum complement, a group of
proteins in the blood that aid the body's infection fighters. Individual proteins are termed by the letter
"C" followed by a number. Common complement tests measure C3, C4, C1q, and CH50. Complement
levels are especially low if there is kidney involvement or other disease activity.
Blood Count. White and red blood cell and platelet counts are usually lower than normal and, dependingon severity, are used to determine complications, such as anemia or infection.
Erythrocyte Sedimentation Rate (ESR). An erythrocyte sedimentation rate (ESR or sed rate) measures
how fast red blood cells (erythrocytes) fall to the bottom of a fine glass tube that is filled with the
patient's blood. A high sed rate indicates inflammation.
C-Reactive Protein (CRP). High levels of this blood protein indicate inflammation. Like the ESR, the CRP
test cannot tell where the inflammation is located or what is causing it.
Click the icon to see an image of the formed elements of blood. Click the icon to see an image of the
formed elements of blood.
Skin Tests
If a skin rash is present, the doctor may take a biopsy (a tissue sample) from the margin of a skin lesion.
A test known as a lupus band detects immunoglobulin G (IgG) antibodies, which are located just below
the outer layer of the tissue sample. They are much more likely to be present with active SLE than with
inactive disease.
Tests for Complications of SLE
Kidney Damage (Lupus Nephritis). Kidney damage in patients already diagnosed with SLE may be
detected from the following tests:
Blood tests that measure creatinine, a protein metabolized in muscles and excreted in the urine. High
levels suggest kidney damage, although kidney problems can also be present with normal creatinine
levels.
Urine tests to measure protein levels
Tests for detecting anti-ds DNA antibodies and blood complements.
All patients who show signs of lupus nephritis should have a kidney biopsy to evaluate and classify the
extent of kidney damage.
Patients who are diagnosed with lupus nephritis should continue to receive urine and blood tests every
13 months to monitor their condition. Regular blood pressure measurements are also important to
ensure that the patients blood pressure does not go above 130/80 mm Hg.
Lung and Heart Involvement. A chest x-ray may be performed to check lung and heart function. An
electrocardiogram and an echocardiogram are administered if heart disease is suspected.
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Click the icon to see an image of an electrocardiogram. Click the icon to see an image of an
electrocardiogram.
Treatment
No treatment cures systemic lupus erythematosus, but many therapies can suppress symptoms and
relieve discomfort. There are also different treatments for the complications associated with lupus.
Treatment of SLE varies depending on the extent and severity of the disease.
Four drugs are specifically FDA-approved for the treatment of lupus:
Prednisone
Aspirin
Hydroxychloroquine (Plaquenil, generic)
Belimumab (Benlysta)
Belimumab (Benlysta) is the newest of these drugs. Approved by the FDA in 2011, it is the first new
lupus drug in over 50 years and the first drug developed specifically for treating lupus. Belimumab is a
biologic monoclonal antibody drug that inhibits a protein called B lymphocyte stimulator. It is given by
infusion in a doctors office. Most other lupus drug treatments are given as pills taken by mouth.
Other drugs that have not been specifically approved for lupus are also commonly used to treat the
condition. Researchers are conducting many investigational drug studies, including trials of new biologic
drugs.
Treating Mild Systemic Lupus Erythematosus
Less intensive treatments may be effective for symptoms of mild lupus. They include:
Creams and sunblocks for rashes
Nonsteroidal anti-inflammatory drugs for fever, arthritis, and headache
Hydroxychloroquine or similar antimalarial drugs for pleurisy, mild kidney involvement, and
inflammation of the tissue surrounding the heart
Treating Severe Systemic Lupus Erythematosus
More aggressive treatment is needed if there is serious disease progression, as evidenced by:
Hemolytic anemia
Low platelet count with an accompanying rash (thrombocytopenia purpura)
Major involvement in the lungs or heart
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Significant kidney damage
Acute inflammation of the small blood vessels in the extremities or gastrointestinal tract
Severe central nervous system symptoms
The primary approach to treating severe SLE is to suppress the inflammation and overactive immune
system with corticosteroids or immunosuppressant drugs. Other types of medications, such as drugs to
control high blood pressure, may also be prescribed.
Treating Specific Complications
The major complications of the disease must be treated as separate disorders, keeping in mind the
specific aspects of SLE.
Pregnancy and SLE
Women with lupus who conceive face high-risk pregnancies that increase the risks for themselves andtheir babies. It is important for women to understand the potential complications and plan accordingly.
The most important advice is to try to avoid becoming pregnant when lupus is active. Research suggests
that the following factors predict a successful pregnancy:
Disease state at time of conception. Doctors strongly recommend that women wait to conceive until
their disease state has been inactive for at least 6 months.
Kidney (renal) function. Women should make sure that their kidney function is evaluated prior to
conception. Poor kidney function can worsen high blood pressure and cause excess protein in the urine.
These complications increase the risk for preeclampsia (high blood pressure doing pregnancy) and
miscarriage. Patients with severe kidney damage (lupus nephritis) may need to have the baby delivered
early.
Lupus-related antibodies. Antiphospholipid and anticardiolipin antibodies can increase the risks for
blood clots, preeclampsia, miscarriage, and stillbirths. Anti-SSA and anti-SSB antibodies can increase the
risk for neonatal lupus erythematosus, a condition that can cause skin rash and liver and heart damage
to the newborn baby. Levels of these antibodies should be tested at the start of pregnancy. Certain
medications (aspirin, heparin) and tests (fetal heart monitoring) may be needed to ensure a safe
pregnancy.
Medication use during pregnancy. Women with active disease may need to take low-dosecorticosteroids, combined with azathioprine if necessary, but women with inactive disease should avoid
these drugs. High doses of steroids can increase a pregnant womans risks for diabetes and high blood
pressure. Pregnant women should not take mycophenolate mofetil (CellCept, generic),
cyclophosphamide (Cytoxan), or methotrexate (Rheumatrex) because they can cause birth defects.
Treatment for Mild SLE
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Creams and Sunblocks
Creams. Steroid creams are often used for skin lesions. However, many patients with cutaneous lupus
do not respond to steroids, particularly if they have eruptions that are caused by sun sensitivity. A cream
derived from vitamin A (Tegison) may help some lesions that do not clear up with steroid creams.
Sun Protection. Sun protection is essential. Patients should always use sunblock creams (not just
sunscreens) and always wear hats and clothing made of tightly woven fabrics.
Nonsteroidal Anti-inflammatory Drugs (NSAIDs)
NSAIDs block prostaglandins, the substances that dilate blood vessels and cause inflammation and pain.
They can help relieve joint pain and swelling, and muscle pain. There are dozens of NSAIDs.
Over-the-counter NSAIDs include aspirin, ibuprofen (Motrin, Advil, generic), naproxen (Aleve, generic),
ketoprofen (Actron, Orudis KT, generic).
Prescription NSAIDs include prescription forms of ibuprofen naproxen and ketoprofen, diclofenac
(Voltaren, generic), and tolmetin (Tolectin, generic).
Side Effects. Regular, long-term use of NSAIDs can cause ulcers and gastrointestinal bleeding, which can
lead to anemia. To avoid these problems, its best to take NSAIDs with food or immediately after a meal.
Long-term use of NSAIDs (with the exception of aspirin) can also increase the risk for heart attack and
stroke.
Other NSAID side effects may include:
Upset stomach
Dyspepsia (burning, bloated feeling in pit of stomach)
Drowsiness
Skin bruising
High blood pressure
Fluid retention
Headache
Rash
Reduced kidney function
Long-term use may increase the risk of heart disease
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Patients who have kidney problems associated with lupus (lupus nephritis) should be especially cautious
about using NSAIDs. Patients with lupus who take NSAIDs on a regular basis should have their liver and
kidney function tested every 3 - 4 months.
An ulcer is a crater-like lesion on the skin or a mucus membrane, such as the lining of the stomach,
caused by an inflammatory, infectious, or malignant condition. Patients can take certain medicines to
suppress the acid in the stomach causing the erosion of the stomach lining. Endoscopic therapy can be
used to stop bleeding from the ulcer.
Antimalarial Drugs
A doctor may prescribe antimalarial drugs for mild SLE when skin problems and joint pains are the
predominant symptoms:
Hydroxychloroquine (Plaquenil, generic) is the most common antimalarial drug used for lupus. This drug
is effective as maintenance therapy to reduce flares in patients with mild or inactive disease.
Hydroxychloroquine may help protect against blood clots in people with antiphospholipid syndrome,
high cholesterol levels, and bone loss. It is also an important treatment for patients with lupus kidney
disease.
Other antimalarial drugs include chloroquine (Aralen, generic) or quinacrine (Atabrine, generic).
Treatment may start initially with high doses in order to accumulate high levels of the drug in the
bloodstream. It is not known exactly why antimalarials work. Some researchers believe they inhibit the
immune response, and others think they interfere specifically with inflammation.
Side Effects. Side effects of antimalarials may include:
Skin rash
Change in skin color (yellow in the case of quinacrine)
Gastrointestinal problems
Headache
Hair loss
Muscle aches
Eye damage
The most serious side effect is damage to the retina, although this is very uncommon at low doses. Eye
damage after taking hydroxychloroquine is reversible when caught in time and treated, but it is not
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reversible if the damage develops after taking chloroquine. An eye exam is advisable about every 6
months.
Antimalarials may also be used in combination with other anti-SLE drugs, including immunosuppressants
and corticosteroids. It should be noted that smoking significantly reduces the effectiveness of
antimalarial drugs.
Treatment for Severe SLE
Corticosteroids
Severe SLE is treated with corticosteroids, also called steroids, which suppress the inflammatory
process. Steroids can help relieve many of the complications and symptoms, including anemia and
kidney involvement.
Oral prednisone (Deltasone, Orasone, generic) is usually prescribed. Other drugs include
methylprednisolone (Medrol, Solumedrol, generic), hydrocortisone, and dexamethasone (Decadron,
generic).
Some people need to take oral prednisone for only a short time; others may require it for a long
duration. An intravenous administration of methylprednisolone using "pulse" therapy for 3 days can
help reduce flare-ups in the joints. Combinations with other drugs, particularly immunosuppressants
such as azathioprine, may be beneficial.
Regimens vary widely, depending on the severity and location of the disease. Most patients with SLE can
eventually function without prednisone, although some may have to choose between the long-term
toxicity of corticosteroids and the complications of active disease. In certain situations (for example, at
the start of treatment for lupus nephritis ), steroids may be given intravenously for a few days.
Side Effects of Long-Term Oral Corticosteroids. Unfortunately, serious and even life-threatening
complications are associated with long-term oral steroid use:
Osteoporosis
Cataracts
Glaucoma
Diabetes
Fluid retention
Susceptibility to infections
Weight gain
High blood pressure
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Acne
Excess hair growth
Wasting of the muscles
Menstrual irregularities
Irritability
Insomnia
Psychosis
Withdrawal from Long-Term Use of Oral Corticosteroids. Long-term use of oral steroid medications
suppresses secretion of natural steroid hormones by the adrenal glands. After withdrawal from these
drugs, this adrenal suppression persists and it can take the body a while (sometimes up to a year) to
regain its ability to produce natural steroids again.
No one should stop taking any steroids without first consulting a doctor, and if steroids are withdrawn,
regular follow-up monitoring is necessary. Patients should discuss with their doctors measures for
preventing adrenal insufficiency during withdrawal, particularly during stressful times, when the risk
increases.
Immunosuppressant Drugs
Drugs known as immunosuppressants are often used, either alone or with corticosteroids, for very
active SLE. Immunosuppressants are particularly recommended when kidney or neurologic involvement
or acute blood vessel inflammation is present. These drugs suppress the immune system by damagingcells that grow rapidly, including those that produce antibodies.
Specific Immunosuppressants. The main immunosuppressants used for treating lupus are:
Cyclophosphamide (Cytoxan, generic)
Mycophenolate mofetil (CellCept, generic)
Azathioprine (Imuran, generic)
Cyclosporine (Sandimmune, generic), tacrolimus (Prograf, generic), and methotrexate (Rheumatrex) are
other immunosuppressants that are sometimes used.
For treating patients with lupus nephritis, the choice of immunosuppressant depends on the severity of
the condition and the patients race. The American College of Rheumatologys guidelines recommend:
For patients with moderate-to-severe lupus nephritis (class III or IV), either cyclophosphamide or
mycophenolate mofetil may be used. Cyclophosphamide is given intravenously. Mycophenolate is given
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in pill form. Intravenous corticosteroids may also be initially given, followed by oral prednisone for a few
weeks. Later treatment may include azathioprine.
For patients with more severe (class IV or V) types of lupus nephritis, steroids plus mycophenolate may
be considered.
For patients with very severe lupus nephritis (class VI), immunosuppresants are of little help and kidney
transplantation is the main option.
Race makes a difference. During the initial (induction) treatment stages, mycophenolate works better
for Hispanic or African American patients whereas cyclophosphamide is best for white patients of
European ethnicity.
Patients who do not respond to mycophenolate or cyclophosphamide may benefit from the biologic
drug rituximab (Rituxan) or possibly caclineurin inhibitors such as tacrolimus or cyclosporine.
Side Effects. The most frequent side effects of immunosuppressants include:
Stomach and intestinal problems
Skin rash
Mouth sores
Hair loss
Serious side effects of immunosuppressants include:
Low blood cell counts
Anemia
Menstrual irregularity
Early menopause
Ovarian failure
Infertility
Herpes zoster (shingles)
Liver and bladder toxicity
Increased risk of cancer
Kidney damage
Biologic Drugs
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Belimumab (Benlysta) is a monoclonal antibody drug that is used along with standard lupus drug
treatments such as corticosteroids, antimalarials, immunosuppressants, and NSAIDs. Approved in 2011,
belimumab is the first new lupus drug in over 50 years and was the first drug developed specifically for
treating lupus. The drug works by targeting and reducing the abnormal B cells that are thought to play a
role in lupus.
Belimumab is given directly into a vein by intravenous infusion. The infusion is given in a doctors office
or other clinical setting and takes about an hour. The patient receives an infusion every 2 weeks for the
first three treatments. After that, the patient receives an infusion once every 4 weeks.
Studies suggest that belimumab may reduce the likelihood of severe flares and may possibly help
patients reduce their doses of steroid medicine. However, in these studies, African-Americans and other
patients of African descent did not seem to respond to belimumab. Additional studies are being
conducted to determine if belimumab is safe and effective for these patients.
Belimumab has many side effects. The most common ones are nausea, diarrhea, and fever. Serious side
effects may include infections, heart problems, and depression including thoughts of suicide. The drug is
very expensive and some insurers may not pay for it.
Lifestyle Changes
Staying Active
People with SLE should try to maintain a healthy and active lifestyle. Light-to-moderate exercise,
interspersed with rest periods, is good for the heart, helps fight depression and fatigue, and can help
keep joints flexible.
Preventing Infections
Patients should be sure they are fully immunized and should minimize their exposure to crowds or
people with contagious illnesses. Careful hygiene, including dental hygiene, is also important.
Avoiding SLE Triggers
It is very important that patients with SLE avoid excessive exposure to sunlight. Simple preventive
measures include avoiding overexposure to ultraviolet rays and wearing protective clothing and
sunblocks. There is some concern that allergy shots may cause flare ups in certain cases. Patients who
may benefit from them should discuss risks and benefits with an SLE specialist. In general, patients with
SLE should use only hypoallergenic cosmetics or hair products. Cigarette smoking is a major trigger forSLE flares. It is very important that patients with SLE not smoke and avoid exposure to second-hand
cigarette smoke.
Reducing Stress
Chronic stress has profound physical effects and influences the progression of SLE. Getting adequate
rest of at least 8 hours and possibly napping during the day may be helpful. Maintaining social
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relationships and healthy activities may also help prevent the depression and anxiety associated with
the disease.
Resources
www.lupus.org -- Lupus Foundation of America
www.lupusny.org -- SLE Lupus Foundation
www.niams.nih.gov -- National Institute of Arthritis and Musculoskeletal and Skin Diseases
www.rheumatology.org -- American College of Rheumatology
www.lupusresearchinstitute.org -- Lupus Research Institute
Source: Systemic lupus erythematosus | University of Maryland Medical Center
http://umm.edu/health/medical/reports/articles/systemic-lupus-erythematosus#ixzz2lOKkmr76
University of Maryland Medical Center
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