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HANDOUT ON HEALTH Systemic Lupus Erythematosus U.S. Department of Health and Human Services National Institutes of Health National Institute of Arthritis and Musculoskeletal and Skin Diseases

Systemic Lupus Erythematosus - Antiphospholipid Syndrome · Systemic Lupus Erythematosus This booklet is for people who have systemic lupus erythematosus, commonly called SLE or lupus,

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  • U.S. Department of Health andHuman ServicesPublic Health Service

    National Institutes of Health

    National Institute of Arthritis and Musculoskeletal and Skin Diseases

    NIH Publication No. 03–4178September 1997 Revised August 2003

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    Systemic LupusErythematosus

    U.S. Department of Health andHuman Services

    National Institutes of HealthNational Institute of Arthritis and

    Musculoskeletal and Skin Diseases

  • The National Institute of Arthritis and Musculoskeletaland Skin Diseases (NIAMS), a part of the Department ofHealth and Human Services’ National Institutes of Health(NIH), leads the Federal medical research effort in arthri-tis and musculoskeletal and skin diseases. The NIAMSsupports research and research training throughout theUnited States, as well as on the NIH campus in Bethesda,MD, and disseminates health and research information.The National Institute of Arthritis and Musculoskeletaland Skin Diseases Information Clearinghouse is a publicservice sponsored by the NIAMS that provides healthinformation and information sources. Additional informa-tion and research updates can be found on the NIAMSWeb site at www.niams.nih.gov.

    This booklet is not copyrighted. Readers are encouraged to duplicate and distribute as many copies as needed.

    Additional copies of this booklet are available from the National Institute of Arthritis andMusculoskeletal and Skin Diseases InformationClearinghouse, NIAMS/National Institutes ofHealth, 1 AMS Circle, Bethesda, MD 20892–3675,and on the NIAMS Web site on the Internet athttp://www.niams.nih.gov/hi/topics/lupus/slehandout/index.htm.

  • Table of Contents

    Defining Lupus . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 3

    Understanding What Causes Lupus . . . . . . . . . . . . . . . 7

    Symptoms of Lupus. . . . . . . . . . . . . . . . . . . . . . . . . . . . . 8

    Diagnosing Lupus . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 11

    Treating Lupus . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 14

    Lupus and Quality of Life . . . . . . . . . . . . . . . . . . . . . . . 19

    Pregnancy for Women With Lupus . . . . . . . . . . . . . . . . 23

    Current Research . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 24

    Hope for the Future . . . . . . . . . . . . . . . . . . . . . . . . . . . . 30

    Additional Resources . . . . . . . . . . . . . . . . . . . . . . . . . . . 31

    Information Boxes

    Common Symptoms of Lupus. . . . . . . . . . . . . . . . . . . . . 8

    Diagnostic Tools for Lupus . . . . . . . . . . . . . . . . . . . . . . 13

    Warning Signs of a Flare . . . . . . . . . . . . . . . . . . . . . . . . 21

    Preventing a Flare . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 21

    Tips for Working With Your Doctor . . . . . . . . . . . . . . . 22

    Promising Areas of Research . . . . . . . . . . . . . . . . . . . . . 28

  • 3

    Systemic Lupus Erythematosus

    This booklet is for people who have systemic lupus

    erythematosus, commonly called SLE or lupus, as well as

    for their family and friends and others who want to

    better understand the disease. The booklet describes the

    disease and its symptoms and contains information about

    diagnosis and treatment as well as current research

    efforts supported by the National Institute of Arthritis

    and Musculoskeletal and Skin Diseases (NIAMS) and

    other components of the Department of Health and

    Human Services’ National Institutes of Health (NIH).

    It also discusses issues such as health care, pregnancy,

    and quality of life for people with lupus. If you have

    further questions after reading this booklet, you may

    wish to discuss them with your doctor.

    Defining Lupus

    L upus is one of many disorders of the immune systemknown as autoimmune diseases. In autoimmune dis-eases, the immune system turns against parts of the bodyit is designed to protect. This leads to inflammation anddamage to various body tissues. Lupus can affect manyparts of the body, including the joints, skin, kidneys,heart, lungs, blood vessels, and brain. Although peoplewith the disease may have many different symptoms,some of the most common ones include extreme fatigue,painful or swollen joints (arthritis), unexplained fever,skin rashes, and kidney problems.

  • 4

    At present, there is no cure for lupus. However, lupuscan be effectively treated with drugs, and most people

    with the disease can lead active,healthy lives. Lupus is characterizedby periods of illness, called flares,and periods of wellness, or remission.Understanding how to prevent flaresand how to treat them when they dooccur helps people with lupus main-tain better health. Intense research isunderway, and scientists funded bythe NIH are continuing to make greatstrides in understanding the disease,which may ultimately lead to a cure.

    Two of the major questionsresearchers are studying are who getslupus and why. We know that manymore women than men have lupus.Lupus is three times more common inAfrican American women than inCaucasian women and is also morecommon in women of Hispanic,Asian, and Native American descent.In addition, lupus can run in families,but the risk that a child or a brother or

    sister of a patient will also have lupus is still quite low.It is difficult to estimate how many people in the UnitedStates have the disease because its symptoms vary wide-ly and its onset is often hard to pinpoint.

    Lupus can be

    effectively

    treated with

    drugs, and most

    people with the

    disease can lead

    active, healthy

    lives.

  • 5

    There are several kinds of lupus::

    ■ Systemic lupus erythematosus (SLE) is the formof the disease that most people are referring towhen they say “lupus.” The word “systemic”means the disease can affect many parts of thebody. The symptoms of SLE may be mild orserious. Although SLE usually first affects peoplebetween the ages of 15 and 45 years, it can occurin childhood or later in life as well. This bookletfocuses on SLE.

    ■ Discoid lupus erythematosus is a chronic skindisorder in which a red, raised rash appears onthe face, scalp, or elsewhere. The raised areasmay become thick and scaly and may causescarring. The rash may last for days or years andmay recur. A small percentage of people withdiscoid lupus have or develop SLE later.

    ■ Subacute cutaneous lupus erythematosus refers toskin lesions that appear on parts of the bodyexposed to sun. The lesions do not cause scarring.

    ■ Drug-induced lupus is a form of lupus caused bymedications. Many different drugs can causedrug-induced lupus. Symptoms are similar tothose of SLE (arthritis, rash, fever, and chestpain) and they typically go away completelywhen the drug is stopped. The kidneys and brainare rarely involved.

  • 6

    ■ Neonatal lupus is a rare disease that can occur innewborn babies of women with SLE, Sjögren’ssyndrome, or no disease at all. Scientists suspectthat neonatal lupus is caused by autoantibodies inthe mother’s blood called anti-Ro (SSA) andanti-La (SSB). Autoantibodies (“auto” meansself) are blood proteins that act against the body'sown parts. At birth, the babies have a skin rash,liver problems, and low blood counts. Thesesymptoms gradually go away over severalmonths. In rare instances, babies with neonatallupus may have a serious heart problem thatslows down the natural rhythm of the heart.Neonatal lupus is rare, and most infants ofmothers with SLE are entirely healthy. Allwomen who are pregnant and known to haveanti-Ro (SSA) or anti-La (SSB) antibodies shouldbe monitored by echocardiograms (a test thatmonitors the heart and surrounding blood vessels)during the 16th and 30th weeks of pregnancy.

    It is important for women with SLE or other relatedautoimmune disorders to be under a doctor’s careduring pregnancy. Physicians can now identifymothers at highest risk for complications, allowingfor prompt treatment of the infant at or before birth.SLE can also flare during pregnancy, and prompttreatment can keep the mother healthier longer.

  • 7

    Understanding What Causes Lupus

    L upus is a complex disease, and itscause is unknown. It is likely thata combination of genetic, environ-mental, and possibly hormonal factorswork together to cause the disease.Scientists are making progress inunderstanding lupus, as describedhere and in the “Current Research”section of this booklet. The fact thatlupus can run in families indicatesthat its development has a geneticbasis. Recent research suggests thatgenetics plays an important role; how-ever, no specific “lupus gene” hasbeen identified yet. Studies suggestthat several different genes may beinvolved in determining a person’slikelihood of developing the disease,which tissues and organs are affected,and the severity of disease. However,scientists believe that genes alone donot determine who gets lupus and that other factors alsoplay a role. Some of the factors scientists are studyinginclude sunlight, stress, certain drugs, and infectiousagents such as viruses.

    In lupus, the body’s immune system does not work as itshould. A healthy immune system produces proteinscalled antibodies and specific cells called lymphocytesthat help fight and destroy viruses, bacteria, and otherforeign substances that invade the body. In lupus, theimmune system produces antibodies against the body’shealthy cells and tissues. These antibodies, called autoan-

    It is likely that

    a combination of . . .

    factors work

    together to cause

    the disease.

  • 8

    tibodies, contribute to the inflammation of various partsof the body and can cause damage to organs and tissues.The most common type of autoantibody that develops inpeople with lupus is called an antinuclear antibody(ANA) because it reacts with parts of the cell’s nucleus(command center). Doctors and scientists do not yetunderstand all of the factors that cause inflammation andtissue damage in lupus, and researchers are activelyexploring them.

    Symptoms of Lupus

    E ach person with lupus has slightly different symptomsthat can range from mild to severe and may come andgo over time. However, some of the most commonsymptoms of lupus include painful or swollen joints(arthritis), unexplained fever, and extreme fatigue.A characteristic red skin rash—the so-called butterfly or

    Common Symptoms of Lupus

    ■ Painful or swollen joints and muscle pain■ Unexplained fever■ Red rashes, most commonly on the face■ Chest pain upon deep breathing■ Unusual loss of hair■ Pale or purple fingers or toes from cold or stress

    (Raynaud’s phenomenon)■ Sensitivity to the sun■ Swelling (edema) in legs or around eyes■ Mouth Ulcers■ Swollen glands■ Extreme fatigue

  • 9

    malar rash—may appear across the nose and cheeks.Rashes may also occur on the face and ears, upper arms,shoulders, chest, and hands. Because many people withlupus are sensitive to sunlight (calledphotosensitivity), skin rashes oftenfirst develop or worsen after sunexposure.

    Other symptoms of lupus includechest pain, hair loss, anemia (adecrease in red blood cells), mouthulcers, and pale or purple fingers andtoes from cold and stress. Some peo-ple also experience headaches, dizzi-ness, depression, confusion, orseizures. New symptoms may contin-ue to appear years after the initialdiagnosis, and different symptoms canoccur at different times. In some peo-ple with lupus, only one system of thebody, such as the skin or joints, isaffected. Other people experiencesymptoms in many parts of their body. Just how serious-ly a body system is affected varies from person to per-son. The following systems in the body also can beaffected by lupus.

    ■ Kidneys: Inflammation of the kidneys (nephritis)can impair their ability to get rid of waste prod-ucts and other toxins from the body effectively.There is usually no pain associated with kidneyinvolvement, although some patients may noticeswelling in their ankles. Most often, the only indi-cation of kidney disease is an abnormal urine orblood test. Because the kidneys are so important

    Symptoms

    can range

    from mild

    to severe and

    may come and

    go over time.

  • 10

    to overall health, lupus affecting the kidneys gen-erally requires intensive drug treatment to preventpermanent damage.

    ■ Lungs: Some people with lupus develop pleuritis,an inflammation of the lining of the chest cavitythat causes chest pain, particularly with breathing.Patients with lupus also may get pneumonia.

    ■ Central nervous system: In some patients, lupusaffects the brain or central nervous system. Thiscan cause headaches, dizziness, memory distur-bances, vision problems, seizures, stroke, orchanges in behavior.

    ■ Blood vessels: Blood vessels may becomeinflamed (vasculitis), affecting the way blood cir-culates through the body. The inflammation maybe mild and may not require treatment or may besevere and require immediate attention.

    ■ Blood: People with lupus may develop anemia,leukopenia (a decreased number of white bloodcells), or thrombocytopenia (a decrease in thenumber of platelets in the blood, which assist inclotting). Some people with lupus may have anincreased risk for blood clots.

    ■ Heart: In some people with lupus, inflammationcan occur in the heart itself (myocarditis andendocarditis) or the membrane that surrounds it(pericarditis), causing chest pains or other symp-toms. Lupus can also increase the risk of athero-sclerosis (hardening of the arteries).

  • 11

    Diagnosing Lupus

    Diagnosing lupus can be difficult. It may take monthsor even years for doctors to piece together the symp-toms to diagnose this complex disease accurately.Making a correct diagnosis of lupus requires knowledgeand awareness on the part of the doctor and good com-munication on the part of the patient. Giving the doctor acomplete, accurate medical history (for example, whathealth problems you have had and for how long) is criti-cal to the process of diagnosis. This information, alongwith a physical examination and the results of laboratorytests, helps the doctor consider other diseases that maymimic lupus, or determine if the patient truly has the dis-ease. Reaching a diagnosis may take time as new symp-toms appear.

    No single test can determine whether a person has lupus,but several laboratory tests may help the doctor to makea diagnosis. The most useful tests identify certain autoan-tibodies often present in the blood of people with lupus.For example, the antinuclear antibody (ANA) test iscommonly used to look for autoantibodies that reactagainst components of the nucleus, or “command cen-ter,” of the body’s cells. Most people with lupus test pos-itive for ANA; however, there are a number of othercauses of a positive ANA besides lupus, including infec-tions, other autoimmune diseases, and occasionally as afinding in healthy people. The ANA test simply providesanother clue for the doctor to consider in making a diag-nosis. In addition, there are blood tests for individualtypes of autoantibodies that are more specific to peoplewith lupus, although not all people with lupus test posi-tive for these and not all people with these antibodieshave lupus. These antibodies include anti-DNA, anti-Sm,

  • 12

    anti-RNP, anti-Ro (SSA), and anti-La (SSB). The doctormay use these antibody tests to help make a diagnosis oflupus.

    Some tests are used less frequentlybut may be helpful if the cause of aperson’s symptoms remains unclear.The doctor may order a biopsy of theskin or kidneys if those body systemsare affected. Some doctors may ordera test for anticardiolipin (or antiphos-pholipid) antibody. The presence ofthis antibody may indicate increasedrisk for blood clotting and increasedrisk for miscarriage in pregnantwomen with lupus. Again, all thesetests merely serve as tools to give thedoctor clues and information in mak-ing a diagnosis. The doctor will lookat the entire picture—medical history,symptoms, and test results—to deter-mine if a person has lupus.

    Other laboratory tests are used tomonitor the progress of the diseaseonce it has been diagnosed. A com-

    plete blood count, urinalysis, blood chemistries, and theerythrocyte sedimentation rate (ESR) test can providevaluable information. Another common test measures theblood level of a group of substances called complement.People with lupus often have increased ESRs and lowcomplement levels, especially during flares of the dis-ease. X rays and other imaging tests can help doctors seethe organs affected by SLE.

    It may take

    months or even

    years for doctors

    to piece together

    the symptoms to

    accurately

    diagnose this

    complex disease.

  • 13

    Diagnostic Tools for Lupus

    ■ Medical history

    ■ Complete physical examination

    ■ Laboratory tests:

    ■ Complete blood count (CBC)■ Erythrocyte sedimentation rate (ESR)■ Urinalysis■ Blood chemistries■ Complement levels■ Antinuclear antibody test (ANA)■ Other autoantibody tests (anti-DNA,

    anti-Sm, anti-RNP, anti-Ro [SSA], anti-La [SSB])

    ■ Anticardiolipin antibody test■ Skin biopsy■ Kidney biopsy

  • 14

    Treating Lupus

    D iagnosing and treating lupus are often a team effortbetween the patient and several types of health careprofessionals. A person with lupuscan go to his or her family doctor orinternist, or can visit a rheumatolo-gist. A rheumatologist is a doctor whospecializes in rheumatic diseases(arthritis and other inflammatory dis-orders, often involving the immunesystem). Clinical immunologists (doc-tors specializing in immune systemdisorders) may also treat people withlupus. As treatment progresses, otherprofessionals often help. These mayinclude nurses, psychologists, socialworkers, nephrologists (doctors whotreat kidney disease), hematologists(doctors specializing in blood disor-

    ders), dermatologists (doctors who treat skin disease),and neurologists (doctors specializing in disorders of thenervous system).

    The range and effectiveness of treatments for lupus haveincreased dramatically, giving doctors more choices inhow to manage the disease. It is important for the patientto work closely with the doctor and take an active role inmanaging the disease. Once lupus has been diagnosed,the doctor will develop a treatment plan based on thepatient’s age, sex, health, symptoms, and lifestyle.Treatment plans are tailored to the individual’s needs andmay change over time. In developing a treatment plan,the doctor has several goals: to prevent flares, to treatthem when they do occur, and to minimize organ damage

    Treatment plans

    are tailored to

    the individual’s

    needs and may

    change over time.

  • 15

    and complications. The doctor and patient should reeval-uate the plan regularly to ensure it is as effective as possible.

    NSAIDs: For people with joint or chest pain or fever,drugs that decrease inflammation, called nonsteroidalanti-inflammatory drugs (NSAIDs), are often used.While some NSAIDs, such as ibuprofen and naproxen,are available over the counter, a doctor’s prescription isnecessary for others. NSAIDs may be used alone or incombination with other types of drugs to control pain,swelling, and fever. Even though some NSAIDs may bepurchased without a prescription, it is important that theybe taken under a doctor’s direction. Common side effectsof NSAIDs can include stomach upset, heartburn, diar-rhea, and fluid retention. Some people with lupus alsodevelop liver, kidney, or even neurological complica-tions, making it especially important to stay in close con-tact with the doctor while taking these medications.

    Antimalarials: Antimalarials are another type of drugcommonly used to treat lupus. These drugs were origi-nally used to treat malaria, but doctors have found thatthey also are useful for lupus. A common antimalarialused to treat lupus is hydroxychloroquine (Plaquenil)*.It may be used alone or in combination with other drugsand generally is used to treat fatigue, joint pain, skinrashes, and inflammation of the lungs. Clinical studieshave found that continuous treatment with antimalarialsmay prevent flares from recurring. Side effects of anti-

    * Brand names included in this publication are provided as examples only, and theirinclusion does not mean that these products are endorsed by the National Institutesof Health or any other Government agency. Also, if a particular brand name is notmentioned, this does not mean or imply that the product is unsatisfactory.

  • 16

    malarials can include stomach upset and, extremelyrarely, damage to the retina of the eye.

    Corticosteroids: The mainstay of lupus treatmentinvolves the use of corticosteroid hormones, such asprednisone (Deltasone), hydrocortisone, methylpred-nisolone (Medrol), and dexamethasone (Decadron,

    Hexadrol). Corticosteroids are relatedto cortisol, which is a natural anti-inflammatory hormone. They work byrapidly suppressing inflammation.Corticosteroids can be given bymouth, in creams applied to the skin,or by injection. Because they arepotent drugs, the doctor will seek thelowest dose with the greatest benefit.Short-term side effects of corticos-teroids include swelling, increasedappetite, and weight gain. These sideeffects generally stop when the drug isstopped. It is dangerous to stop takingcorticosteroids suddenly, so it is veryimportant that the doctor and patientwork together in changing the corti-costeroid dose. Sometimes doctorsgive very large amounts of corticos-teroid by vein over a brief period oftime (days) (“bolus” or “pulse” thera-py). With this treatment, the typicalside effects are less likely and slowwithdrawal is unnecessary.

    Long-term side effects of corticosteroids can includestretch marks on the skin, weakened or damaged bones(osteoporosis and osteonecrosis), high blood pressure,

    It is dangerous to

    stop taking

    corticosteroids

    suddenly, so it is

    very important

    that the doctor

    and patient

    work together in

    changing the dose.

  • 17

    damage to the arteries, high blood sugar (diabetes), infec-tions, and cataracts. Typically, the higher the dose and thelonger they are taken, the greater the risk and severity ofside effects. Researchers are working to develop ways tolimit or offset the use of corticosteroids. For example,corticosteroids may be used in combination with other,less potent drugs, or the doctor may try to slowlydecrease the dose once the disease is under control.People with lupus who are using corticosteroids shouldtalk to their doctors about taking supplemental calciumand vitamin D or other drugs to reduce the risk of osteo-porosis (weakened, fragile bones).

    Immunosuppressives: For some patients whose kidneysor central nervous systems are affected by lupus, a typeof drug called an immunosuppressive may be used.Immunosuppressives, such as cyclophosphamide(Cytoxan) and mycophenolate mofetil (CellCept), restrainthe overactive immune system by blocking the produc-tion of immune cells. These drugs may be given bymouth or by infusion (dripping the drug into the veinthrough a small tube). Side effects may include nausea,vomiting, hair loss, bladder problems, decreased fertility,and increased risk of cancer and infection. The risk forside effects increases with the length of treatment. Aswith other treatments for lupus, there is a risk of relapseafter the immunosuppressives have been stopped.

    Other Therapies: In some patients, methotrexate (Folex,Mexate, Rheumatrex), a disease-modifying antirheumaticdrug, may be used to help control the disease. Workingclosely with the doctor helps ensure that treatments forlupus are as successful as possible. Because some treat-ments may cause harmful side effects, it is important toreport any new symptoms to the doctor promptly. It is

  • 18

    also important not to stop or change treatments withouttalking to the doctor first.

    Alternative and Complementary Therapies: Becauseof the nature and cost of the medications used to treatlupus and the potential for serious side effects, many

    patients seek other ways of treatingthe disease. Some alternativeapproaches people have tried includespecial diets, nutritional supplements,fish oils, ointments and creams, chiro-practic treatment, and homeopathy.Although these methods may not beharmful in and of themselves, andmay be associated with symptomaticor psychosocial benefit, no researchto date shows that they affect the dis-ease process or prevent organ dam-age. Some alternative or complemen-tary approaches may help the patientcope or reduce some of the stressassociated with living with a chronicillness. If the doctor feels theapproach has value and will not beharmful, it can be incorporated intothe patient’s treatment plan.However, it is important not toneglect regular health care or treat-ment of serious symptoms. An open

    dialogue between the patient and physician about the rel-ative values of complementary and alternative therapiesallows the patient to make an informed choice abouttreatment options.

    Because some

    treatments may

    cause harmful

    side effects . . .

    report any new

    symptoms to

    the doctor

    promptly.

  • 19

    Lupus and Quality of Life

    Despite the symptoms of lupus and the potential side-effects of treatment, people with lupus can maintain ahigh quality of life overall. One key to managing lupus isto understand the disease and its impact. Learning to rec-ognize the warning signs of a flare can help the patienttake steps to ward it off or reduce its intensity. Manypeople with lupus experience increased fatigue, pain, arash, fever, abdominal discomfort, headache, or dizzinessjust before a flare. Developing strategies to prevent flarescan also be helpful, such as learning to recognize yourwarning signals and maintaining good communicationwith your doctor.

    It is also important for people with lupus to receive regu-lar health care, instead of seeking help only when symp-toms worsen. Results from a medical exam and laborato-ry work on a regular basis allows the doctor to note anychanges and to identify and treat flares early. The treat-ment plan, which is tailored to the individual’s specificneeds and circumstances, can be adjusted accordingly. Ifnew symptoms are identified early, treatments may bemore effective. Other concerns also can be addressed atregular checkups. The doctor can provide guidance aboutsuch issues as the use of sunscreens, stress reduction, andthe importance of structured exercise and rest, as well asbirth control and family planning. Because people withlupus can be more susceptible to infections, the doctormay recommend yearly influenza vaccinations or pneu-mococcal vaccinations for some patients.

    Women with lupus should receive regular preventivehealth care, such as gynecological and breast examina-tions. Men with lupus should have the prostate-specific

  • antigen (PSA) test. Both men and women need to havetheir blood pressure and cholesterol checked on a regularbasis. If a person is taking corticosteroids or antimalarialmedications, an eye exam should be done at least yearlyto screen for and treat eye problems.

    Staying healthy requires extra effortand care for people with lupus, so itbecomes especially important todevelop strategies for maintainingwellness. Wellness involves closeattention to the body, mind, and spirit.One of the primary goals of wellnessfor people with lupus is coping withthe stress of having a chronic disorder.Effective stress management variesfrom person to person. Someapproaches that may help includeexercise, relaxation techniques such asmeditation, and setting priorities forspending time and energy.

    Developing and maintaining a goodsupport system is also important. Asupport system may include family,friends, medical professionals, com-munity organizations, and supportgroups. Participating in a supportgroup can provide emotional help,

    boost self-esteem and morale, and help develop orimprove coping skills. (For more information on supportgroups, see the “Additional Resources” section at the endof this booklet.)

    Learning to

    recognize the

    warning signs of a

    flare can help

    the patient take

    steps to ward

    it off or reduce its

    intensity.

    20

  • 21

    Warning Signs of a Flare

    ■ Increased fatigue■ Pain■ Rash■ Fever■ Abdominal discomfort■ Headache■ Dizziness

    Preventing a Flare

    ■ Learn to recognize your warning signals■ Maintain good communication with

    your doctor

  • 22

    Learning more about lupus may also help. Studies haveshown that patients who are well-informed and partici-pate actively in their own care experience less pain,make fewer visits to the doctor, build self-confidence,and remain more active.

    Tips for Working With Your Doctor

    ■ Seek a health care provider who is familiar withSLE and who will listen to and address yourconcerns.

    ■ Provide complete, accurate medical information.

    ■ Make a list of your questions and concerns in advance.

    ■ Be honest and share your point of view with the health care provider.

    ■ Ask for clarification or further explanation if you need it.

    ■ Talk to other members of the health care team, such as nurses, therapists, or pharmacists.

    ■ Do not hesitate to discuss sensitive subjects (for example, birth control, intimacy) with your doctor.

    ■ Discuss any treatment changes with your doctor before making them.

  • 23

    Pregnancy for Women With Lupus

    A lthough a lupus pregnancy is considered high risk,most women with lupus carry their babies safely tothe end of their pregnancy. Women with lupus have ahigher rate of miscarriage and premature births comparedwith the general population. In addi-tion, women who have antiphospho-lipid antibodies are at a greater risk ofmiscarriage in the second trimesterbecause of their increased risk ofblood clotting in the placenta. Lupuspatients with a history of kidney dis-ease have a higher risk of preeclamp-sia (hypertension with a buildup ofexcess watery fluid in cells or tissuesof the body). Pregnancy counselingand planning before pregnancy areimportant. Ideally, a woman shouldhave no signs or symptoms of lupusand be taking no medications for atleast 6 months before she becomespregnant.

    Some women may experience a mildto moderate flare during or after their pregnancy; othersdo not. Pregnant women with lupus, especially those tak-ing corticosteroids, also are more likely to develop highblood pressure, diabetes, hyperglycemia (high bloodsugar), and kidney complications, so regular care andgood nutrition during pregnancy are essential. It is alsoadvisable to have access to a neonatal (newborn) inten-sive care unit at the time of delivery in case the babyrequires special medical attention.

    Pregnancy

    counseling

    and planning

    before pregnancy

    are important.

  • 24

    Current Research

    L upus is the focus of intense research as scientists tryto determine what causes the disease and how it canbest be treated. Some of the questions they are workingto answer include: Why are women more likely than mento have the disease? Why are there more cases of lupusin some racial and ethnic groups? What goes wrong inthe immune system, and why? How can we correct theway the immune system functions once something goeswrong? What treatment approaches will work best tolessen lupus symptoms? How do we cure lupus?

    To help answer these questions, scientists are developingnew and better ways to study the disease. They are doinglaboratory studies that compare various aspects of theimmune systems of people with lupus with those of otherpeople both with and without lupus. They also use micewith disorders resembling lupus to better understand theabnormalities of the immune system that occur in lupusand to identify possible new therapies.

    The National Institute of Arthritis and Musculoskeletaland Skin Diseases (NIAMS), a component of theDepartment of Health and Human Services’ NationalInstitutes of Health (NIH), has a major focus on lupusresearch in its oncampus program in Bethesda, Maryland.By evaluating patients with lupus and their relatives,researchers oncampus are learning more about how lupusdevelops and changes over time. The NIAMS also fundsmany lupus researchers across the United States. Someof these researchers are studying the genetic factors thatincrease a person’s risk for developing lupus. To help sci-entists gain new knowledge, the NIAMS also has estab-lished Specialized Centers of Research devoted specifi-

  • 25

    cally to lupus research. In addition, the NIAMS is fund-ing lupus registries that gather medical information aswell as blood and tissue samples from patients and theirrelatives. This gives researchers across the countryaccess to information and materialsthey can use to help identify genesthat determine susceptibility to thedisease.

    Identifying genes that play a role inthe development of lupus is an activearea of research. For example,researchers suspect that a geneticdefect in a cellular process calledapoptosis, or “programmed celldeath,” exists in people with lupus.Apoptosis is similar to the processthat causes leaves to turn color inautumn and fall from trees; it allowsthe body to eliminate cells that havefulfilled their function and typicallyneed to be replaced. If there is a problem in the apoptosisprocess, harmful cells may stay around and do damage tothe body’s own tissues. For example, in a mutant mousestrain that develops a lupus-like illness, one of the genesthat controls apoptosis is defective. When it is replacedby a normal gene, the mice no longer develop signs ofthe disease. Scientists are studying what role genesinvolved in apoptosis may play in human disease devel-opment.

    Studying genes for complement, a series of proteins inthe blood that play an important part in the immune sys-tem, is another active area of lupus research.Complement acts as a backup for antibodies, helping

    Scientists are

    developing new

    and better

    ways to study

    the disease.

  • 26

    them destroy foreign substances that invade the body. Ifthere is a decrease in complement, the body is less ableto fight or destroy foreign substances. If these substancesare not removed from the body, the immune system may

    become overactive and begin to makeautoantibodies.

    Recent large studies of families withlupus have identified a number ofgenetic regions that appear to beassociated with risk of SLE.Although the specific genes and theirfunction remain unknown, intensivework in mapping the entire humangenome offers promise that thesegenes will be identified in the nearfuture. This should provide knowl-edge of the complex factors that con-tribute to lupus susceptibility.

    NIAMS-funded researchers areuncovering the impact of genetic,socioeconomic, and cultural factors

    on the course and outcome of lupus in Hispanics, AfricanAmericans, and Caucasians. Preliminary data show thatAfrican American and Hispanic lupus patients typicallyhave more kidney damage compared with Caucasians.In addition, NIAMS-funded researchers found thatAfrican American lupus patients have more skin damagecompared with Hispanics and Caucasians, and that thedeath rate from lupus is higher in African Americans andHispanics compared with Caucasians.

    It is thought that autoimmune diseases, such as lupus,occur when a genetically susceptible individual encoun-

    Identifying genes

    that play a role in

    the development

    of lupus is an

    active area

    of research.

  • 27

    ters an unknown environmental agent or trigger. In thiscircumstance, an abnormal immune response can be initi-ated that leads to the signs and symptoms of lupus.Research has focused on both the genetic susceptibilityand the environmental trigger. Although the environmen-tal trigger remains unknown, microbial agents such asEpstein-Barr virus and others have been considered.Researchers also are studying other factors that mayaffect a person’s susceptibility to lupus. For example,because lupus is more common in women than in men,some researchers are investigating the role of hormonesand other male-female differences in the developmentand course of the disease. A current study funded by theNIH is focusing on the safety and effectiveness of oralcontraceptives (birth-control pills) and hormone replace-ment therapy in women with lupus. Doctors have wor-ried about the wisdom of prescribing oral contraceptivesor estrogen replacement therapy for women with lupusbecause of a widely held view that estrogens can makethe disease worse. Oral contraceptives and estrogenreplacement therapy do not, as once feared, appear tointensify lupus symptoms. Scientists do not know theeffects of oral contraceptives on women with antiphos-pholipid antibody syndrome.

    Patients with lupus are at risk of developing atheroscle-rotic vascular disease (hardening of the blood vessels thatcan cause heart attack, angina, or stroke). The increasedrisk is due partly to having lupus and partly to steroidtherapy. Preventing atherosclerotic vascular disease inlupus patients is a new area of study. NIAMS-fundedresearchers are studying the most effective ways to man-age cardiovascular risk factors and prevent cardiovascu-lar disease in adult lupus patients.

  • 28

    In childhood lupus, researchers are evaluating the safetyand effectiveness of drugs called statins that lower LDL

    (or bad) cholesterol levels as amethod of preventing fat buildup inthe blood vessels.

    One out of five lupus patients experi-ences symptoms such as headaches,dizziness, memory disturbances,stroke, or changes in behavior thatresult from changes in the brain orother parts of the central nervous sys-tem. Such lupus patients have what iscalled “neuropsychiatric” lupus.NIAMS-funded scientists are apply-ing new tools such as brain imagingtechniques to discover cellular activi-ty and specific genes that may causeneuropsychiatric lupus. By uncover-ing the mechanisms responsible forcentral nervous system damage inlupus patients, researchers hope tomove closer to improved diagnosis

    and treatment for patients with neuropsychiatric lupus.

    Promising Areas of Research

    ■ Identifying lupus susceptibility genes■ Searching for environmental agents that

    cause lupus■ Developing drugs or biologic agents to

    treat lupus

    Research has

    focused on both

    the genetic

    susceptibility and

    the environmental

    trigger.

  • 29

    Researchers are focusing on finding better treatments forlupus. A primary goal of this research is to develop treat-ments that can effectively minimize theuse of corticosteroids. Scientists aretrying to identify combination therapiesthat may be more effective than single-treatment approaches. Another goal isto improve the treatment and manage-ment of lupus in the kidneys and cen-tral nervous system. For example, a 20-year study supported by the NIAMSand the NIH found that combiningcyclophosphamide with prednisonehelped delay or prevent kidney failure,a serious complication of lupus.

    On the basis of new information aboutthe disease process, scientists are usingnovel “biologic agents” to selectivelyblock parts of the immune system.Development and testing of these newdrugs, which are based on compoundsthat occur naturally in the body, com-prise an exciting and promising new area of lupusresearch. The hope is that these treatments not only willbe effective, but also will have fewer side effects.Preliminary research suggests that white blood cellsknown as B cells may play a key role in the developmentof lupus. Biologics that interfere with B cell function orblock the interactions of immune cells are active areas ofresearch. These targeted treatments hold promise becausethey have the advantage of reduced side effects andadverse reactions compared with conventional therapies.Clinical trials are testing the safety and effectiveness ofrituximab (also called anti-CD20) in treating people with

    Scientists

    are using novel

    “biologic agents”

    to selectively

    block parts

    of the immune

    system.

  • 30

    lupus. Rituximab is a genetically engineered antibodythat blocks the production of B cells. Other treatmentoptions currently being explored include reconstructingthe immune system by bone marrow transplantation. Inthe future, gene therapy also may play an important rolein lupus treatment.

    Hope for the Future

    W ith research advances and a better understanding oflupus, the prognosis for people with lupus today isfar brighter than it was even 20 years ago. It is possibleto have lupus and remain active and involved with life,family, and work. As current research efforts unfold,there is continued hope for new treatments, improve-ments in quality of life, and, ultimately, a way to preventor cure the disease. The research efforts of today mayyield the answers of tomorrow, as scientists continue tounravel the mysteries of lupus.

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    Additional Resources

    National Institute of Arthritis and Musculoskeletal andSkin DiseasesNIAMS/National Institutes of Health1 AMS CircleBethesda, MD 20892-3675(301) 495-4484 or 877-22-NIAMS (226-4267) (free of charge)Fax: (301) 718-6366TTY: (301) 565-2966www.niams.nih.gov

    The National Institute of Arthritis and Musculoskeletaland Skin Diseases Information Clearinghouse is a publicservice sponsored by the NIAMS that provides healthinformation and information sources. The clearinghouseprovides information on lupus. Fact sheets, additionalinformation, and research updates can also be found onthe NIAMS Web site at www.niams.nih.gov.

    Clinicaltrials.gov

    The Department of Health and Human Services’ NationalInstitutes of Health, through its National Library ofMedicine, has developed ClinicalTrials.gov to providepatients, family members, and members of the publiccurrent information about clinical research studies. Youcan search for trials by disease, location, treatment, or byfunding organization at the Web site clinicaltrials.gov.

  • American College of RheumatologyAssociation of Rheumatology Health Professionals 1800 Century Place, Suite 250Atlanta, GA 30345(404) 633-3777Fax: (404) 633-1870www.rheumatology.org

    The American College of Rheumatology (ACR) is anorganization of doctors and associated health profession-als who specialize in arthritis and related diseases of thebones, joints, and muscles. The Association ofRheumatology Health Professionals, a division of ACR,aims to enhance the knowledge and skills of rheumatol-ogy health professionals and to promote their involve-ment in rheumatology research, education, and qualitypatient care. The association also works to advance andpromote basic and continuing education in rheumatologyfor health professionals who provide care to people withrheumatic diseases.

    Alliance for Lupus Research, Inc.28 West 44th Street, Suite 1217New York, NY 10036 (212) 218-2840(800) 867-1743 (free of charge)www.lupusresearch.org

    The Alliance for Lupus Research, Inc. (ALR), is a non-profit organization devoted exclusively to the support ofpromising research for the prevention, treatment, andcure of lupus. Through accelerated, focused, goal-orient-ed research programs, the ALR aims to promote basicand clinical sciences to achieve major advances leadingto a better understanding of the causes of lupus.

    32

  • American Autoimmune Related Diseases Association22100 Gratiot AvenueEastpointeEast Detroit, MI 48021-2227(586) 776-3900(800) 598-4668 (free of charge)E-mail: [email protected]

    The American Autoimmune Related DiseasesAssociation (AARDA) is the only national nonprofitvoluntary health agency dedicated to bringing a nationalfocus and collaborative effort to the over 100 knownautoimmune diseases through education, awareness,research, and patient services. By collaborating with theNational Coalition of Autoimmune Patient Groups(NCAPG), AARDA supports legislative advocacy forautoimmune disease patients. AARDA provides freepatient education information, physician and agencyreferrals, forums and symposia, and a quarterly newsletter.

    Arthritis Foundation1330 West Peachtree StreetAtlanta, GA 30309(404) 872-7100(800) 283-7800, or your local chapter (listed in the tele-phone directory)www.arthritis.org

    The Arthritis Foundation is the major voluntary organiza-tion devoted to supporting arthritis research and providingeducational and other services to individuals with arthritis.It publishes free pamphlets and a magazine for members onall types of arthritis. It also provides up-to-date informationon research and treatment, nutrition, alternative therapies,and self-management strategies for patients with lupus and

    33

  • other autoimmune diseases. Chapters nationwide offerexercise programs, classes, support groups, physician refer-ral services, and free literature. For more information, callyour local chapter, listed in the white pages of the phonebook, or contact the Arthritis Foundation at the aboveaddress.

    Lupus Clinical Trials Consortium, Inc. (LCTC)47 Hulfish Street, Suite 442Princeton, NJ 08540(609) 921-1532

    The LCTC is a nonprofit organization that encouragesthe identification and testing of promising new therapiesfor lupus. It provides infrastructure support grants to cer-tain academic institutions to support their clinicalresearch activities; encourages lupus clinical researchersfrom those institutions to share their expertise; supportsand conducts educational efforts to show the need forlupus clinical research; and disseminates scientificinsights to advance the discovery of new lupus therapies.

    Lupus Foundation of America (LFA), Inc.2000 L Street, N.W., Suite 710Washington, DC 20036(202) 349-1155(800) 558-0121www.lupus.org

    The LFA assists local chapters in providing services topeople with lupus, works to educate the public aboutlupus, and supports lupus research. Through a network ofmore than 500 branches and support groups, the chaptersprovide education through information and referral ser-vices, health fairs, newsletters, publications, and semi-nars. Chapters provide support to people with lupus, their

    34

  • families, and friends through support group meetings,hospital visits, and telephone help lines.

    Rheuminations, Inc.221 East 48th Street, Ground FloorNew York, NY 10017(212) 593-5180Fax: (212) 593-5181www.dxlupus.org

    Rheuminations, Inc. is a private, nonprofit foundationcommitted to funding excellence in medical research toachieve a better understanding of the causes of lupus andto bring new treatments to market; to educate and toempower those who live with lupus and those who carefor them; to establish a higher level of public awarenessabout the disease.

    SLE Foundation, Inc.149 Madison Ave., Suite 205New York, NY 10016(212) 685-4118www.lupusny.org

    The foundation supports and encourages medicalresearch to find the cause and cure of lupus and improveits diagnosis and treatment. It also provides a wide vari-ety of services to help patients with lupus and their fami-lies. In addition, this voluntary organization conducts abroad-based public education program to raise awarenessof lupus and increase understanding of this serious,chronic, autoimmune disease.

    35

  • Acknowledgments: The NIAMS gratefully acknowl-edges the assistance of Jill P. Buyon, M.D., Hospital forJoint Diseases, New York, New York; Patricia A. Fraser,M.D., Brigham and Women’s Hospital, Boston,Massachusetts; John H. Klippel, M.D., The ArthritisFoundation, Washington, DC; Michael D. Lockshin, M.D.,Barbara Volcker Center for Women and RheumaticDisease, Hospital for Special Surgery, New York, NewYork; Rosalind Ramsey-Goldman, M.D., Dr.P.H.,Northwestern University Medical School, Chicago,Illinois; George Tsokos, M.D., Uniformed ServicesUniversity of the Health Sciences, Bethesda, Maryland;and Elizabeth Gretz, Ph.D., Barbara Mittleman, M.D.,Susana Serrate-Sztein, M.D., and Peter E. Lipsky, M.D.,NIAMS, NIH, in the preparation and review of this andearlier versions of this publication. Special thanks also goto the many patients who reviewed this publication andprovided valuable input. An earlier version of this bookletwas written by Debbie Novak of Johnson, Bassin, andShaw, Inc.

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  • The National Institute of Arthritis and Musculoskeletaland Skin Diseases (NIAMS), a part of the Department ofHealth and Human Services’ National Institutes of Health(NIH), leads the Federal medical research effort in arthri-tis and musculoskeletal and skin diseases. The NIAMSsupports research and research training throughout theUnited States, as well as on the NIH campus in Bethesda,MD, and disseminates health and research information.The National Institute of Arthritis and Musculoskeletaland Skin Diseases Information Clearinghouse is a publicservice sponsored by the NIAMS that provides healthinformation and information sources. Additional informa-tion and research updates can be found on the NIAMSWeb site at www.niams.nih.gov.

    This booklet is not copyrighted. Readers are encouraged to duplicate and distribute as many copies as needed.

    Additional copies of this booklet are available from the National Institute of Arthritis andMusculoskeletal and Skin Diseases InformationClearinghouse, NIAMS/National Institutes ofHealth, 1 AMS Circle, Bethesda, MD 20892–3675,and on the NIAMS Web site on the Internet athttp://www.niams.nih.gov/hi/topics/lupus/slehandout/index.htm.

  • U.S. Department of Health andHuman ServicesPublic Health Service

    National Institutes of Health

    National Institute of Arthritis and Musculoskeletal and Skin Diseases

    NIH Publication No. 03–4178September 1997 Revised August 2003

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    National Institutes of HealthNational Institute of Arthritis and

    Musculoskeletal and Skin Diseases