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Lupus

WHAT IS LUPUS? are present. Sometimes a person may have a Lupus refers to several forms of a disease of the complete or long-lasting remission, but this does immune system that affects joints, skin, kidneys not necessarily mean the disease has gone away. and other parts of the body. The immune system is your body’s natural defense against infections, Systemic Lupus such as bacteria and viruses. In lupus, the About 70 percent of the people who have immune system produces antibodies that react lupus have the systemic form, or SLE. A systemic with the body’s own tissues. Because of this, disease is one in which several different body sys- lupus is referred to as an autoimmune (aw-toe- tems may be affected. In systemic lupus, the skin, im-MYOON) disease. In most cases the term joints, kidneys, nervous system, lungs, heart “lupus” refers to the form known as systemic and/or blood-forming organs can be affected. lupus erythematosus (sis-TEM-ick LOO-pus About half of the people with systemic lupus e-RIH-them-ah-TOE-sis), or SLE for short. have a form that affects major internal organs, Lupus is an inflammatory condition that may particularly the kidneys. In the other half of be chronic. Inflammation refers to a reaction people with systemic lupus, mainly the skin and that results in pain, heat, redness and swelling. joints are affected. This second type is less likely Chronic (cron-NIK) means the condition is long- to cause serious problems. lasting, which could mean it lasts for the rest of your life. You may not experience symptoms on Discoid Lupus a constant basis, however. About 15 percent of people with lupus have Many people with lupus have changes in signs a form known as discoid (DIS-coyd) lupus or and symptoms known as flares and remissions. cutaneous (cue-TANE-ee-us) lupus. This form A flare is a period when the disease becomes of lupus results in a chronic skin rash that can more active with increased symptoms. During a sometimes cause scars. Discoid lupus may remission, few or no signs and symptoms of lupus affect the skin without affecting other organs.

© 2002. Arthritis Foundation, Inc., Atlanta, GA. All rights reserved. For individual use only. Material in this publication may not be reprinted without permission. Send requests for reprint permissions in writing to 1330 W. Peachtree St., Suite 100, Atlanta, GA 30309. For more information about arthritis, call 800/283-7800.

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Drug-Induced Lupus WHO GETS LUPUS? The least common form of lupus develops as Studies suggest that some people may inherit a result of drugs taken for other medical prob- the tendency to get lupus, and that genes play an lems. This form is called drug-induced lupus. important role in the disease. Researchers have Signs and symptoms are similar to SLE, found that new cases of lupus are more common although people with this form of lupus rarely in families in which someone already has the develop serious organ damage. Many different disease or related autoimmune disease, such as drugs can cause drug-induced lupus. Signs and RA or diabetes (di-uh-BEE-tees). Most scientists symptoms usually improve and disappear once believe that an environmental factor, such as a the drug is stopped. virus, serves to trigger symptoms in people who have a genetic tendency to develop lupus. WHAT CAUSES LUPUS? About 90 percent of people with lupus are The cause of lupus, with the exception of women. In most cases, symptoms first appear drug-induced lupus, is unknown. Doctors and in women of childbearing age (18 to 45). But scientists refer to lupus as an autoimmune dis- lupus also occurs in children and in older peo- ease. The immune system fights off bacteria and ple. African Americans tend to get lupus more viruses in several ways. One way is by creating often than Caucasians. Some studies suggest special types of blood proteins called antibodies the disease also may occur more often in Asian that attack and destroy invading substances. and Hispanic populations than in Caucasians. In lupus, the immune system does not function properly, and produces antibodies (called autoan- SYMPTOMS OF LUPUS tibodies) that react with and damage the body’s You may develop several of the symptoms cells, tissues and organs. This process is known as mentioned in this section or just a few. No two an autoimmune response (auto means self). people with lupus have the same symptoms. In lupus, the many different types of auto- The American College of Rheumatology antibodies are formed, although the most (ACR) has developed guidelines to help doctors common autoantibody is produced against diagnose lupus. If you have four or more of the the nucleus of cells, and therefore called antin- following 11 symptoms listed, it is likely that uclear antibodies, or ANA for short. There you have lupus or a similar condition. are several different types of ANA. Types of ANA such as anti-double stranded DNA (anti Main Symptoms and Signs ds-DNA) or anti-Smith (Sm) antibodies, are If you have four or more of the signs or unique to SLE. symptoms listed below, talk to a doctor who Antinuclear antibodies are found in almost can determine whether you have lupus or one all cases of lupus. ANA also may be found in of many other conditions that can cause similar people with other autoimmune diseases, such symptoms. The 11 main symptoms and signs as rheumatoid arthritis (ROO-ma-toyd ar- that may indicate lupus include: THRY-tis), or RA, and Sjögren’s (SHOW-grens) syndrome, and even can be found in some • A rash across the cheeks and the bridge of the healthy people. nose (called a “butterfly rash”)

© 2002. Arthritis Foundation, Inc., Atlanta, GA. All rights reserved. For individual use only. Material in this www.arthritis.org publication may not be reprinted without permission. Send requests for reprint permissions in writing to 1330 W. Peachtree St., Suite 100, Atlanta, GA 30309. For more information about arthritis, call 800/283-7800. 800-283-7800

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• Scaly, disk-shaped rash on the face, neck, ears, • Hair loss scalp and/or chest • Sjögren’s syndrome, a chronic condition that • Sensitivity to sunlight, such as severe rashes causes dryness of the eyes and mouth.Women or fever from minimal sun exposure may experience vaginal dryness. • Painless sores on the tongue, inside the mouth • Depression or difficulty concentrating and/or in the nose • Arthritis (pain, stiffness and swelling in the joints) • Pain in your chest and side when you breathe, DIAGNOSIS indicating inflammation of the lining of the heart Lupus can be hard to diagnose. It is important (pericarditis) or lungs (pleurisy) to see a rheumatologist (ROO-ma-tall-o-jist), a • Kidney problems doctor who specializes in arthritis and related • Neurologic (brain) problems, including seizures diseases, like lupus. The rheumatologist will begin and mental problems by asking you questions and conducting a • Low white or red blood cell count physical exam. You’ll also have laboratory tests, • Presence of specific autoantibodies measured including ones to see if you have too few red in the blood blood cells, white blood cells or platelets (blood • The presence of antinuclear antibodies, the most cells that help to control bleeding and clotting). commonly seen autoantibody in SLE Blood chemistry tests and urine studies can help determine whether your organs, such as Other Symptoms and Signs of Lupus the kidneys and liver, are functioning normally. If your rheumatologist thinks you may have People with lupus can develop other symptoms lupus, he or she will order a blood test called an and signs, including serious organ involvement, ANA, which detects a group of autoantibodies not included in the 11 criteria described above. found in the blood of people with lupus. These These symptoms and signs include: autoantibodies attack the body’s own cells. However, this test does not diagnose lupus. • Blood clots Other tests to find certain antibodies, such as • Strokes anti-DNA or anti-Smith, may be helpful in • Heart attacks diagnosing lupus or related diseases. Antibodies • Eye inflammation to phospholipids, including cardiolipin, also • Fever are common in lupus. They are associated with • Weakness, fatigue an increased risk for blood clotting, strokes and • Weight loss recurrent miscarriages. Tests to measure the • Raynaud’s (ray-NODES) phenomenon, a condi- level of complement proteins in your blood tion in which the fingers, toes, nose and/or ears may be obtained. Levels of complement are may become unusually sensitive to cold and may often low or reduced in people with lupus. turn white or blue when exposed to the cold. Other blood tests may be conducted for • Muscle aches diagnosis and as an aid in following disease • Swollen lymph nodes activity. Because kidney problems often occur, • Loss of appetite you’ll need a urinalysis, which is an examina-

Page 3 of 10 PRACTICAL HELP FROM THE ARTHRITIS FOUNDATION tion of your urine. If protein is found, you may the amount or frequency of medication you be asked to collect all the urine you pass in a take without talking to your doctor. Changing 24-hour period for analysis. If your doctor sus- your schedule on your own can make it more pects kidney problems, you also may have a difficult for your doctor to evaluate what is hap- kidney biopsy. This is when a small piece of tis- pening in your disease and how the medication sue from one of your kidneys is removed and is working. Taking a drug without medical examined. This procedure requires an overnight advice could cause harm, either by interacting hospital stay. with other medications you take or by causing Your doctor may request a chest X-ray or side effects. heart studies such as an electrocardiogram (EKG) or an echocardiogram to determine if the dis- NONSTEROIDAL ANTI-INFLAMMATORY DRUGS ease is affecting your lungs or heart. What they do: Nonsteroidal anti-inflammatory drugs (NSAIDs) can help control the TREATMENTS arthritis and inflammation associated with lupus. The treatment plan for lupus includes tak- Some examples of NSAIDs include aspirin, ing medications to reduce the inflammation and ibuprofen and naproxen. NSAIDs are available reduce the activity of the immune system, balanc- in prescription and over-the-counter forms. ing rest with exercise and eating a proper diet. Side effects: NSAIDs can cause stomach Lupus is an unpredictable disease. Signs of irritation, bleeding from the stomach or the disease appear and disappear, sometimes for intestines, ulcers or diarrhea. Your doctor may no apparent reason. Because lupus has so many prescribe anti-ulcer medications to help protect different forms and can change, finding the right you from these side effects. Newer NSAIDs treatment for you may take time. Your treatment called COX-2 specific inhibitors may further will depend on the symptoms you experience minimize stomach side effects. and the organs affected. Once an effective treat- Tips: Some people may feel stomach upset ment program has been started, continue to fol- when they take large doses of NSAIDs. You may low it. If your symptoms change, let your doctor be able to ease this side effect by taking your know so that you can work together to adjust medication with meals or with a large glass of your program. water or milk. You may want to try safety-coated tablets, which help protect the stomach. Taking Medications an antacid about 30 minutes after meals and at Medications are a necessary part of treatment bedtime also can help protect your stomach. for most people with lupus. The particular med- NSAIDs can affect the platelets in the blood ication prescribed by your doctor will depend and can extend bleeding time. If you are sched- on the extent of disease and how active it is. The uled for surgery, you may need to temporarily type and amount of medications prescribed stop taking these drugs. Care should be taken may change over time, depending on the signs when using NSAIDs with blood thinners, such and symptoms of lupus that are present. as warfarin. COX-2 specific inhibitors do not Many medications can take several weeks or increase bleeding time. Most NSAIDs can rarely months to start working. You should not change damage the liver and decrease kidney function.

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The damage usually can be reversed when related to cortisol, a hormone that occurs natu- you stop taking the medication or take it in rally in the body and controls many necessary smaller doses. body functions. During times when your lupus is very active, DISEASE-MODIFYING ANTIRHEUMATIC DRUGS your doctor may prescribe large doses of gluco- What they do: Disease-modifying anti- corticoids. Lower doses may be used to treat rheumatic drugs (DMARDs) may be used to lupus when it doesn’t threaten internal organs. As treat symptoms of lupus, such as arthritis, skin symptoms and laboratory test results improve, rashes and pleurisy, which is inflammation of your doctor may reduce the dose over time. the lining of the lungs. These drugs can help Side effects: Common side effects of high treat and prevent lupus rashes and systemic doses of glucocorticoids include weight gain, problems such as fever and fatigue. rounding of the face, easy bruising, slow heal- The most common DMARD used for treating ing and mood swings. Other possible side lupus is hydroxychloroquine. Prescribing hydrox- effects include fluid retention with swelling of ychloroquine for mild disease may decrease the the legs and high blood pressure; triggering or risk of lupus spreading. These medications are worsening of diabetes; increased risk of infec- usually prescribed long term, because stopping tion; the death of bony tissue, called osteonecro- them may result in a lupus flare. sis (ah-stee-oh-ne-CRO-sis); acne; and, rarely, Side effects: The most common side effects stomach bleeding. of hydroxychloroquine are mild nausea, vomit- Using glucocorticoids over a long period of ing and diarrhea. time may lead to cataracts, glaucoma, muscle weakness and/or thinning of the bones, called Medication Tip osteoporosis (ah-stee-oh-po-RO-sis). Your doctor may recommend that you take calcium and In rare cases that usually follow prolonged vitamin D supplements or other medications to use, hydroxychloroquine may injure the retina, help prevent bone loss. the lining of the eye that enables you to see. Tips: If you are taking glucocorticoids, tell Early detection of this unusual occurrence can any doctor or dentist before you have surgery minimize damage. See an eye specialist who is or undergo any other type of invasive proce- familiar with this toxicity regularly – usually dure. Your doctor may need to increase your every six months – to help reduce the slight dosage at such times to enable your body to risk from this drug. If you notice any changes handle the stress. You also should wear a medi- in your vision while taking hydroxychloro- cal identification bracelet to alert others that quine, call your doctor right away. you take glucocorticoids in case you cannot tell them during a medical emergency.

GLUCOCORTICOIDS OTHER IMMUNOSUPPRESSIVE DRUGS What they do: Glucocorticoids (such as pred- What they do: Most immunosuppressive nisone) are drugs that restrain the immune sys- drugs reduce the activity of the immune system. tem and help to reduce inflammation. They are These drugs often are prescribed with gluco-

Page 5 of 10 PRACTICAL HELP FROM THE ARTHRITIS FOUNDATION corticoids when lupus affects major organs. reduce the activity of the immune system. Tell This combination may allow a reduction in your doctor if you develop a fever or any other your dosage of glucocorticoids. new symptoms while taking these medications. The immunosuppressive drugs most often used for lupus are azathioprine, methotrexate Physical Activity and cyclophosphamide. Cyclosporine and Exercising every day is very important. mycophenolate mofetil also are used. Working out may be easier when your lupus is less active and you feel better. However, even DHEA Supplements during a flare, doing gentle range-of-motion (ROM) exercises can keep you from feeling DHEA is short for dehydroepinandrosterone, stiff. Exercise also can help prevent weak mus- a weak male hormone found naturally in the cles so that your muscles are better able to sup- body. Nutritional supplements of this chemical port sore joints. are made from extracts of the wild Mexican A physical therapist can work with you to yam. This supplement has been touted as a set up an exercise program that fits your remedy for a variety of diseases, including schedule, physical condition and other needs. lupus. Some studies have found evidence that This may be as simple as designing a walking DHEA may help to ease certain lupus symp- program you can adjust to fit how you feel toms and reduce the need for medication in each day. In fact, simple ROM exercises such patients with lupus that affects only the skin as stretching can be as effective in improving and joints. your mood, sense of well-being and fatigue level as aerobic activity. How DHEA works is not completely under- stood and the full risks and benefits are not Diet yet known. People with lupus should not take Eating a balanced diet is an important part of this supplement without first seeking a doc- your treatment plan. At times when your lupus tor’s advice. Because nutritional supplements is active and your appetite is poor, taking a daily are regulated differently from drugs, the qual- multivitamin may help your body get the nutri- ity and quantity of ingredients in DHEA prod- ents it may be missing. However, high doses of ucts may vary widely. If you are considering vitamins can have side effects. Some herbs and taking DHEA, consult your doctor about the dietary supplements can make your lupus worse. potential risks and benefits. Be sure to tell your doctor about any vitamins, herbs or dietary supplements you take. Your doctor may suggest a special diet to Side effects: These drugs can have serious help problems caused by lupus symptoms, such side effects. If you are taking an immunosup- as kidney disease. A low-salt diet will help pre- pressive drug, you should have regular blood vent fluid retention that can lead to swelling. If tests because the drug can interfere with the you have kidney disease, you also may need to proper formation of blood cells. You also may limit how much protein you eat so that you get infections more easily because the drugs don’t overwork your kidneys.

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Simply taking a daily dose of folic acid can Heart Problems help lower levels of a chemical called homocys- People with lupus are at increased risk for teine (ho-mo-SIS-teen). High levels of homo- heart attacks. This may be because lupus can cysteine, which have been linked with heart damage blood vessels. Other heart disease risk problems, have been found in people with lupus. factors, such as hypertension (high blood pres- Ask your doctor about the recommended dose sure), smoking, obesity and high cholesterol of folic acid to help reduce homocysteine. High and homocysteine levels may further damage cholesterol also may be a problem; discuss this the blood vessels. Consult your doctor for ways with your doctor. to reduce or manage these risks. Drinking alcohol has the potential to harm your liver. You should avoid drinking alcohol if Infections you take certain drugs such as methotrexate, People with lupus are more likely to get which increases the risk of liver damage. infections. This is because the immune system may not be working properly and because of SPECIAL CONCERNS medication side effects, especially from gluco- Kidney Problems corticoids and immunosuppressive drugs. For Sometimes, even with the use of glucocorti- some people with lupus, infections can trigger coids and immunosuppressive drugs, kidney disease flares. If you have a fever, which could failure may occur. If this happens, dialysis (di- indicate infection, talk to your doctor. Yearly AL-ih-sis) can help cleanse waste products from flu shots and other vaccines are important to the blood. One procedure, called hemodialysis, protect people with lupus from infections. involves placing catheters in an artery and a vein to filter the blood through a cleansing Sun Exposure machine. Blood is taken from the body, run Exposure to the sun and certain fluorescent through a machine that removes poisons and lighting is known to worsen the skin rashes waste products, and then returned to the body. and/ or other systemic problems linked with Continuous ambulatory peritoneal dialysis lupus in some people. About one-third of peo- (CAPD) is another cleansing procedure in ple with lupus are sun-sensitive. Sun exposure which fluid is placed into and then drained may cause lupus flares, resulting in fever, joint from the abdominal cavity through a tube. This pain or even inflammation of the heart, lungs, can be done at home, but careful supervision is kidneys or nervous system. Too much sun necessary because people with lupus have an exposure for one person, however, may be fine increased risk of infection. for someone else. Degrees of sun-sensitivity When kidney failure is irreversible, a kidney vary from person to person. transplant may be recommended. This procedure often can allow a person with lupus to Sun Safety return to a normal lifestyle, although many medications are required to prevent rejection of A few simple precautions can help protect you the new kidney. from negative reactions to sun exposure. Fol-

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low these steps to help prevent lupus-related rash eventually goes away, usually within six rashes and sunburns from sun-sensitivity. months after the baby’s birth. A very rare heart problem can occur in the baby of a woman who • Avoid outdoor activities during peak sunlight has this specific autoantibody pattern. Talk to your hours (usually 10 a.m. - 4 p.m.). doctor about this risk and options for treatment. • Use sunscreen lotions or sun block regularly. If your doctor advises against pregnancy, or if Choose one with a sun protection factor (SPF) you do not wish to have children, it’s important of 15 or higher. Like all makeup and lotions, to use birth control because you could still sunscreens may cause allergic reactions in become pregnant. An unplanned pregnancy dur- some people. If you have a reaction, stop using ing a period of active disease could cause health that product and try a different one or consult problems and expose the growing fetus to med- your doctor. ications you take for lupus. • Look for products that block both UVA and The safest method of contraception for UVB rays – the label should note if the prod- women with lupus is a diaphragm used with uct blocks both types of ultraviolet rays. SPF contraceptive jelly. Take special care and con- ratings apply only to UVB light; ultraviolet sult your doctor about the benefits and risks of light exposure is greater at higher altitudes. using intrauterine devices (IUDs) because they • If you must be out in the sun, wear a hat to carry a risk of infection. Studies on the safety of shade you from direct sun and clothing to cover birth-control pills for women with lupus are your arms, legs and chest. Clothing made under way. Such information about their safety from special sun-blocking fabrics is available. should be available in a few years. Note: Women with antiphospholipid (an-ty-fos-FO- li-pid) antibody should consult with their doc- Pregnancy and Birth Control tors prior to taking birth-control pills or estro- Pregnancy may present special problems for gen replacement therapy (ERT) because of women with lupus. Lupus may worsen, or flare, increased risk of thrombosis (blood clots). during a pregnancy. Alternately, lupus may get better or stay the same. Dryness Women with lupus have an increased chance Post-menopausal women with lupus or those of having a miscarriage either early or late in their who also have secondary Sjögren’s syndrome pregnancy. Certain abnormal antibodies pre- may experience vaginal dryness. Lubricating sent in the blood of some women with lupus products and estrogen creams can help restore may increase the chance of miscarriage. Be sure moisture and ease discomfort. If you experience to consult your doctor about any plans to vaginal dryness, talk to your doctor about which become pregnant. The good news is that with products may be right for you. careful planning, many women with lupus can have normal pregnancies and healthy babies. MANAGING LUPUS In some cases, a woman with lupus can pass Emotional problems often go along with autoantibodies (anti-Ro and anti-La) through the having a chronic disease. Talking about your placenta, which can cause a rash in the baby. The fears, anger and depression usually helps. It is

Page 8 of 10 PRACTICAL HELP FROM THE ARTHRITIS FOUNDATION important to confide in your family, friends • A study of people with SLE who have diffi- and doctor. Joining a support group and shar- culty with memory and concentration to ing your frustrations with others who have lupus uncover associated signs, symptoms, medica- may make you feel better emotionally and help tions, and behavioral and psychosocial impact. you solve specific problems. Reading about lupus can help you under- Alliance for Lupus Research stand your symptoms and what to expect from The Alliance for Lupus Research (ALR) is a the disease. You’ll feel more in control of your new, nonprofit organization formed as a joint condition and be able to communicate more undertaking by the family of Robert Wood effectively with your doctor about treatments. Johnson IV and the Arthritis Foundation to There may be times, though, when attending provide an aggressive and concerted effort to support groups or reading about lupus may not prevent, treat and cure systemic lupus erythe- be enough to overcome a sense of feeling over- matosus (SLE), or lupus. For more information whelmed. In that case, seeking counseling from a about ALR, please call 800/867-1743 or visit professional trained in helping people with chronic www.lupusresearch.org diseases may be helpful. Your doctor or local chap- ter of the Arthritis Foundation can give you sug- THE ARTHRITIS FOUNDATION gestions about agencies and mental health profes- The mission of the Arthritis Foundation is sionals such as social workers, counselors and psy- to improve lives through leadership in the chologists who may be able to offer their support. prevention, control and cure of arthritis and related diseases. LUPUS RESEARCH The Arthritis Foundation supports research Researchers are gaining knowledge that will with the greatest potential for advances and has contribute to improvements in the quality of invested more than $320 million in these efforts life of people with lupus. This information is since its inception in 1948. Additionally, the leading to the development and testing of vari- Arthritis Foundation supports key public policy ous techniques for helping people with lupus and advocacy efforts at a local and national gain the knowledge, attitudes and coping skills level in order to make a difference on behalf of they need to take control of their condition. 70 million people living with arthritis. Ongoing Arthritis Foundation-funded stud- As your partner in taking greater control of ies are aimed at finding better ways to control arthritis, the Arthritis Foundation also offers a lupus and improve the quality of life of those large number of programs and services nation- affected by it. Some examples include: wide to make life with arthritis easier and less painful and to help you become an active part- • Studies to increase understanding of how best ner in your own health care. to prevent premature cardiovascular disease Contact us at (800) 283-7800 or visit us on in women with lupus; the Web at www.arthritis.org to become an • Studies of how estrogens foster the produc- Arthritis Advocate or to find out how you can tion of autoantibodies; and become involved.

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The Arthritis Foundation acknowledges with appre- ciation Robert Lahita, MD, PhD, St. Vincent’s Medical Center, New York; Michael Lockshin, MD, Cornell University, New York; Susan Manzi, MD, MPH, University of Pittsburgh; and Laura Robbins, DSW, Hospital for Special Surgery, New York, for their assistance with this booklet.

For more information: The Arthritis Foundation offers a wide variety of books, brochures and videos about different forms of arthritis, treatment and self-management techniques to help you take control of your arthritis. To order any of these products, become an Arthritis Foundation member or to subscribe to the Arthritis Foundation's award-winning consumer health magazine, Arthritis Today, call (800) 283-7800. Call or visit our Web site (www.arthritis.org) to find out how you can take control of your arthritis and start living better today!

This brochure has been reviewed by the AMERICAN COLLEGE OF RHEUMATOLOGY.

MISSION STATEMENT: The mission of the Arthritis Foundation is to improve lives through leadership in the prevention, control and cure of arthritis and related diseases.

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