• Systemic Lupus Erythematosus

    (Lupus; SLE; Lupus, Systemic)

    Definition

    Lupus is an autoimmune disease. It inflames:
    • Joints
    • Tendons
    • Skin
    • Other connective tissue and organs
    It causes the immune system to make antibodies that attack the body's healthy cells and tissue.

    Causes

    The cause of lupus is unknown. Researchers believe it may be a combination of:
    • Genetic factors
    • Environmental factors, which may include:
      • Sunlight (UV rays)
      • Stress
    • Viral or other type of infection
    • Drug-induced (methyldopa, procainamide, hydralazine, isoniazid, chlorpromazine, TNF-blocking drugs)

    Risk Factors

    These risk factors increase your chance of developing lupus. Tell your doctor if you have any of these risk factors:
    • Sex: female to male ratio: 10:1
    • Age: childbearing age (20-45 years)
    • Race: African American, Native American, Asian, and Hispanic

    Symptoms

    Symptoms can be mild or very severe. For some people, only part of the body (such as skin) is affected. For others, many parts are affected. Though symptoms can be chronic, they can flare up and get better on and off.
    Common symptoms:
    • Swollen and/or painful joints
    • Fever
    • Skin rashes over areas exposed to sunlight (especially on the nose and cheeks)
    • Extreme fatigue
    Common Lupus Rash Sites
    Lupus rash
    Facial butterfly rash is hallmark of Lupus.
    Copyright © Nucleus Medical Media, Inc.
    Other symptoms may include:
    A pregnant woman who has lupus may experience complications, like a flair-up of symptoms, high blood pressure, and kidney inflammation. There may also be problems with the pregnancy (such as premature birth, miscarriage, restricted growth in the fetus).

    Diagnosis

    Diagnosing lupus can be difficult. It can take time to identify the condition because you may develop more symptoms over time.
    To aid in making the diagnosis, the American College of Rheumatology (ACR) has created the following criteria. You must have 4 out of the 11 items to be diagnosed with lupus:
    • Butterfly facial rash
    • Rash (or red patches) on sun-exposed areas
    • Skin photosensitivity (easily burned by the sun)
    • Ulcers in the mouth or near the throat
    • Arthritis in at least two joints
    • Inflammation of the lining of the heart or lungs (called serositis)
    • Kidney problems (identified by kidney function tests)
    • Seizures or psychosis that are not caused by another condition
    • Abnormally low number of blood cells
    • Antinuclear antibodies—these are immune chemicals produced by your body that attack the nuclei in your cells. These antinuclear antibodies may contribute to the cause of lupus.
    • Immune dysfunction—in people with lupus, several other antibodies have been found. These antibodies can be detected with lab tests.
    To find out if you do meet the criteria, your doctor will order tests, such as:
    • Blood tests, such as complete blood count, antinuclear antibody (ANA) test
    • Urine test to check kidney functioning
    • Imaging tests (such as MRI scan) if you have neurological symptoms
    Depending on your symptoms, your doctor may diagnosis you with lupus even if you have less than four of the ACR’s criteria.
    Your doctor will also rule out other conditions that may have similar symptoms, such as:

    Treatment

    Treatment options depend on your symptoms.

    Medication

    There are many different kinds of medicines that are used to treat lupus. Examples include:
    • Nonsteroidal anti-inflammatory drugs (NSAIDs)—to relieve joint pain
    • Antimalarial drugs (such as hydroxychloroquine, chloroquine)—to relieve joint pain, fatigue, and rashes
    • Corticosteroids—to reduce inflammation
    • Drugs to suppress the immune system (such as azathioprine, mycophenolate, methotrexate)—to help with symptom relief if the other medicines are not working
    • Prescription steroid cream for rashes (such as fluocinonide cream)
    • Hormonal medicine (dehydroepiandrosterone [DHEA])—to reduce symptoms
    • B-cell therapy (such as rituximab)—experimental medicine to reduce the number of white blood cells
    Your doctor may recommend that you take a combination of medicines.
    Medicines for severe symptoms include:
    • Oral and intravenous corticosteroids to control and limit inflammation in kidney, brain, lung, and heart, as well as in cases of severe anemia
    • Immunosuppressive drugs to suppress the body's autoimmune system
    • Mycophenolate, azathioprine, and cyclophosphamide for kidney disease or other life- or organ-threatening conditions
    • Rituximab for refractory disease

    Other Treatment Options

    In addition to taking medicine, your doctor may recommend that you:
    • Eat a healthy diet. Adding omega-3 fatty acids, found in certain types of fish, may help with symptoms.
    • Exercise regularly. Your doctor can give you advice as to which exercises are safe for you to do.
    • Protect your skin from the sun. Wear sunscreen with an SPF of at least 30 whenever you go out in the sun.
    • Work with a therapist. Counseling may help you to build skills to cope with your condition.

    Prevention

    You cannot prevent lupus because the cause is unknown.
    To prevent flare-ups of symptoms:

    RESOURCES

    Lupus Foundation of America, Inc. http://www.lupus.org

    Lupus Research Institute http://www.lupusresearchinstitute.org

    CANADIAN RESOURCES

    Lupus Canada http://www.lupuscanada.org

    Lupus Foundation of Ontario http://vaxxine.com/lupus

    References

    Beers MH, Fletcher AJ. The Merck Manual of Medical Information. New York, NY: Simon and Schuster, Inc; 1999.

    Contreas G, Pardo V, Leclercq B, et al. Sequential therapies for proliferative lupus nephritis. N Engl J Med . 2004;350;971-980.

    Dehydroepiandrosterone (DHEA). EBSCO Natural and Alternative Treatments website. Available at: http://www.ebscohost.com/healthLibrary/. Updated September 2009. Accessed December 4, 2009.

    Dorner T, Lipsky PE. Immunoglobulin variable-region gene usage in systemic autoimmune diseases. Arthritis Rheum . 2001;44:2715-2727.

    DynaMed Editorial Team. Systemic lupus erythematosus (SLE). EBSCO DynaMed website. Available at: https://dynamed.ebscohost.com/about/about-us. Updated July 19, 2010. Accessed July 13, 2010.

    Gescuk BD, Davis JC Jr. Novel therapeutic agents for systemic lupus erythematosus. Curr Opin Rheumatol. 2002;14:515.

    Hejaili F, Moist LM, Clark WF. Treatment of lupus nephritis. Drugs. 2003;63:257-274.

    Lupus. National Institute of Arthritis and Muskuloskeletal and Skin Diseases, National Institutes of Health website. Available at: http://www.niams.nih.gov/Health%5FInfo/Lupus/default.asp. Published September 1997. Updated August 2003. Accessed June 26, 2008.

    Lupus Foundation of America. Treatments for lupus. Lupus Foundation of America website. Available at: http://www.lupus.org/webmodules/webarticlesnet/templates/new%5Flearntreating.aspx?articleid=2245&zoneid=525. Accessed August 31, 2010.

    Mayo Clinic. ANA test. Mayo Clinic website. Available at: http://www.mayoclinic.com/health/ana-test/MY00787/DSECTION=how-you-prepare. Updated July 8, 2010. Accessed August 31, 2010.

    Mayo Clinic. Lifestyle and home remedies. Mayo Clinic website. Available at: http://www.mayoclinic.com/health/lupus/DS00115/DSECTION=lifestyle-and-home-remedies. Updated July 8, 2010. Accessed August 31, 2010.

    Mayo Clinic. Treatments and drugs. Mayo Clinic website. Available at: http://www.mayoclinic.com/health/lupus/DS00115/DSECTION=treatments-and-drugs. Updated July 8, 2010. Accessed August 31, 2010.

    Polsdorfer R. Lifestyle changes to manage systemic lupus erythematosus. EBSCO Health Library website. Available at: http://www.ebscohost.com/healthLibrary. Updated August 30, 2010. Accessed August 31, 2010.

    Polsdorfer R. Medications for systemic lupus erythematosus. EBSCO Health Library website. Available at: http://www.ebscohost.com/healthLibrary/ . Updated December 4, 2009. Accessed August 31, 2010.

    Sherer Y, Gorstein A, Fritzler MJ, Shoenfeld Y. Auto-antibody explosion in systemic lupus erythematosus: more than 100 different antibodies found in SLE patients. Semin Arthritis Rheum . 2004;34:501-537.

    Smolen JS. Therapy of systemic lupus erythematosus: a look into the future. Arthritis Res. 2002;4(suppl)3:S25.

    Symptoms. Lupus Foundation of America website. Available at: http://www.lupus.org/webmodules/webarticlesnet/templates/new%5Flearnunderstanding.aspx?articleid=2235&zoneid=523 . Accessed June 26, 2008.

    12/4/2009 DynaMed's Systematic Literature Surveillance https://dynamed.ebscohost.com/about/about-us : Hartkamp A, Geenen R, Godaert GL, Bijl M, Bijlsma JW, Derksen RH. Effects of dehydroepiandrosterone on fatigue and well-being in women with quiescent systemic lupus erythematosus. A randomized controlled trial. Ann Rheum Dis. 2009 Oct 22. [Epub ahead of print]

    5/6/2011 DynaMed's Systematic Literature Surveillance https://dynamed.ebscohost.com/about/about-us : Smyth A, Oliveira GH, Lahr BD, Bailey KR, Norby SM, Garovic VD. A systematic review and meta-analysis of pregnancy outcomes in patients with systemic lupus erythematosus and lupus nephritis. Clin J Am Soc Nephrol. 2010;5(11):2060-2068.

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