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Systemic lupus erythematosus: Unmasking a great imitator

ROONEY, JOAN RN, CRNP, MSN

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The sooner your patient with systemic lupus erythematosus is diagnosed and begins treatment, the better her outcome may be. Find out how to assess a patient at risk for this chronic disease and what you can do to help her stay healthy.

Learn to recognize the many faces of this baffling disorder, then teach your patient ways to maintain her health and prevent symptom flares.

A CHRONIC, MULTISYSTEM autoimmune disease, systemic lupus erythematosus (SLE) can wreak havoc throughout the body. Its name, lupus—Latin for wolf—was coined in medieval times to describe skin lesions resembling wolf bites. But signs and symptoms can be vague or nonspecific. Because SLE is easily confused with other disorders, it's been called the great imitator.

The course and severity of SLE varies widely among patients. According to the latest available data from the Centers for Disease Control and Prevention (CDC), the survival rate is about 90% to 95% at 2 years after diagnosis and 75% or less at 20 years. With treatment, many patients keep serious problems at bay.

Because a patient with SLE deals with recurring disease flares and remissions, she faces both physical and psychosocial challenges. Make sure you're prepared to offer education, clinical care, and emotional support by reviewing the information and practical guidelines in this article.

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Immune system malfunction

In autoimmune disorders such as SLE, the body produces autoantibodies to its own cells. This causes the immune system to attack parts of the body that it normally protects, leading to inflammation and tissue and organ damage.

The Lupus Foundation of America estimates that about 1.5 million Americans have one of three lupus forms: SLE, discoid (cutaneous) lupus erythematosus, or drug-induced lupus. This article will focus on SLE because it's the most common and the most serious form (see Who's at risk for SLE?). The other forms have more limited effects.

  • Discoid lupus erythematosus is a chronic skin disorder with scaling plaques that may cause scarring. Lesions appear on the neck and head, particularly the ears and scalp. Scalp involvement causes hair loss. A small percentage of patients with discoid lupus develop SLE.
  • Drug-induced lupus usually resolves when the triggering drug is discontinued. Although many drugs can cause this disorder, procainamide, hydralazine, isoniazid, quinidine, and phenytoin are common culprits. The signs and symptoms of drug-induced lupus, which are similar to those of SLE, include fever, malaise, rash, or pain from arthritis, myalgia, and arthralgia.
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Waxing and waning symptoms

Systemic lupus erythematosus is characterized by remissions and exacerbations (called flares) of symptoms. Early signs and symptoms, such as fatigue, fever, and weight loss, may be nonspecific and mimic those of other disorders. Here's how SLE can affect a patient's major body systems.

  • Musculoskeletal. Arthralgia, myalgia, and arthritis are the most common manifestations of SLE. The patient may experience morning stiffness, joint pain and swelling, muscle achiness, and even muscle weakness. The signs and symptoms may come and go, or reappear in different parts of the body. Unlike rheumatoid arthritis, the arthritis of lupus doesn't destroy bone and cartilage.
  • Dermatologic. Most patients with SLE are photosensitive; sun exposure exacerbates both skin and systemic signs and symptoms. The most easily recognized skin disorder associated with SLE is the malar rash, a flat or raised butterfly-shaped erythematous rash that extends over the cheeks and bridge of the nose. It may be constant or come and go, and it worsens with sun exposure. Another dermatologic symptom, alopecia (hair loss), may occur all over the body or in patches.
  • Renal. Nephritis is an early manifestation of renal involvement. A patient may not notice symptoms until she has advanced nephrotic syndrome or renal failure. Proteinuria, casts, and red cells in the urine indicate renal complications. The patient may need a kidney biopsy to determine the stage of disease and guide treatment decisions.
  • Cardiovascular. Serious cardiac problems associated with SLE include pericarditis, myocarditis, endocarditis, and accelerated atherosclerosis leading to myocardial infarction. Vasculitis (inflammation of blood vessels) can also be part of the pathology of SLE.
  • Gastrointestinal. Peritonitis can cause diffuse abdominal pain. Anorexia, nausea, and vomiting may also occur. Intestinal vasculitis, a life-threatening complication, may lead to ischemia, perforation, and sepsis. A patient with SLE may develop pancreatitis or ascites and have abnormal liver enzyme levels.
  • Pulmonary. Potential complications include pulmonary hemorrhage, pulmonary embolism, pulmonary hypertension, lupus pneumonitis, pleuritis, or interstitial lung disease.
  • Hematologic. Anemia, leukopenia, thrombocytopenia, and lymphopenia are common in SLE.
  • Neurologic. Ranging from mild to severe, possible neurologic problems include severe headache, seizures, peripheral neuropathy, and disturbed mental functioning with delirium or psychosis.
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Difficult diagnosis

Any two patients with SLE can have completely different signs and symptoms, and no patient with SLE has every possible one. Because the diagnosis can be hard to nail down, the American College of Rheumatology has developed clinical and lab criteria to help clinicians assess for SLE. (See Sorting out diagnostic criteria.)

These tests may help a clinician confirm the diagnosis.

  • The serum antinuclear antibody (ANA) test detects antibodies that mistake the nuclei of body cells for foreign invaders and attack them. Most people with SLE test positive for ANA, but other autoimmune conditions can cause positive results too—for example, rheumatoid arthritis, scleroderma, and autoimmune hepatitis. Autoimmune thyroid disease and some bacterial and viral infections may also cause positive test results, as can certain medications (such as methyldopa and diuretics such as hydrochlorothiazide). And 5% to 15% of people without any connective-tissue disease will test positive.
  • Anti-Smith (anti-Sm) antibodies are specific for SLE but are detected in only 20% to 30% of people with SLE.
  • Anti-double-stranded DNA (anti-dsDNA). High titers of antibodies against native DNA are highly specific for SLE because other autoimmune disorders don't cause this test result. Sixty to seventy percent of patients with SLE have high anti-dsDNA titers.
  • Antiphospholipid (APL) antibodies may be present in up to 50% of SLE patients, but this finding may also be present in people who don't have SLE. Patients with SLE who have arterial or venous thrombosis or repeated fetal losses and at least two positive tests for APL antibodies have antiphospholipid syndrome.
  • A false-positive syphilis test may occur in patients with SLE because cardiolipin, a phospholipid, is a component of the antigenic mixture that is used in the assay. Chronically false-positive Venereal Disease Research Laboratory (VDRL) or rapid plasma reagin (RPR) tests may be a diagnostic clue that a patient has SLE.
  • A recently approved in vitro diagnostic immunoassay system can detect six autoantibodies simultaneously using one diluted sample of human serum. The ENA IgG BeadChip Test is made by BioArray Solutions for use with its Array Imaging System 400. The test can detect extractable nuclear antigens (ENAs) and then, after a short incubation with fluorescent antihuman immunoglobulin G, display them on a microarray on a silicon chip. This information can be combined with clinical findings and other test results to diagnose autoimmune disorders such as SLE, Sjögren's syndrome, scleroderma, and myositis.
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When the wolf bites…

Although SLE isn't curable, it can be treated and managed with medications and lifestyle modifications, such as limiting sun exposure. Goals of treatment are to:

  • decrease inflammation
  • suppress the overactive immune system
  • prevent symptom flares
  • minimize complications related to the disease or therapy.

Many patients with SLE respond well to one or more of the following medications.

Nonsteroidal anti-inflammatory drugs (NSAIDS) treat musculoskeletal problems and certain systemic symptoms, such as fever.

Antimalarial drugs such as hydroxychloroquine (Plaquenil) and chloroquine (Aralen) help some patients control SLE arthritis, rashes, mouth ulcers, fatigue, and fever. Because these drugs take effect slowly, tell the patient that she may not notice improvement for several months. Encourage her to stick with therapy even if she doesn't feel better right away.

Corticosteroids have multiple uses. For example, they may be used to treat cutaneous manifestations, arthritis, and serositis. A patient with major organ involvement may need high-dose steroids, up to 1 mg per kilogram of body weight daily P.O. Or, as an alternative to daily high-dose oral therapy, she may receive an intravenous (I.V.) bolus injection of 1 gram of methylprednisolone for 3 consecutive days.

Cytotoxic drugs may be prescribed to treat serious complications involving muscles or joints, the central nervous system, or major organs such as the kidneys. Usually used to prevent rejection of transplanted organs, these drugs suppress the hyperactive immune response associated with SLE. Their use may let the clinician reduce the patient's corticosteroid dosage, minimizing steroid-related adverse reactions.

Depending on the drug, cytotoxic agents have varying mechanisms of action. Those most commonly used for SLE include azathioprine (Imuran), cyclophosphamide (Cytoxan), methotrexate (Rheumatrex, Trexall), and cyclosporine (Neoral, Sandimmune). You'll need to closely monitor the patient for serious adverse drug reactions, including bone marrow suppression and increased susceptibility to infection.

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Caring for your patient

Keeping in mind that the course and severity of SLE varies widely, tailor your nursing care to your patient's condition. Patient teaching and psychosocial support should be cornerstones of your nursing care plan. Besides the potentially serious long-term complications of SLE, your patient may also face problems related to her prescribed drug therapy, such as infection, increased cancer risk, and osteoporosis. All these uncertainties are emotionally stressful.

To assess your patient, start by taking a complete health history and physical exam. Because SLE can affect most body systems, be sure to use a systems approach to the physical exam. Assess for signs and symptoms suggesting a disease flare, such as a malar rash following sun exposure, fatigue, fever, mouth sores, hair loss, and unexplained weight loss. Ask her how often she has flares, what triggers them, and what typical symptoms are for her. Her treatment will depend on her manifestations, including what systems are affected. Find out if she has any family history of any form of lupus or other autoimmune disorders.

To monitor the course of the disease and the patient's response to therapy, keep a close eye on lab test results. For example, the complete blood cell (CBC) count may reveal anemia resulting from bone marrow suppression, leukopenia, or thrombocytopenia. Bleeding times may be prolonged from circulating anticoagulant antibodies. The erythrocyte sedimentation rate (ESR) often rises during SLE flare-ups. Urinalysis may show blood, protein, and red and white cell casts.

If your patient is receiving cytotoxic drugs, she's vulnerable to potentially severe adverse reactions (myelosuppression, malignancy, hepatotoxicity, infection), so monitor her closely both during and after drug administration.

When you teach your patient about SLE, use printed materials to provide basic information and to reinforce your teaching. Refer her to a local support group and to the Lupus Foundation for additional assistance and information. (See Selected Web sites.)

To help her avoid photosensitivity reactions and disease flares, teach her to use sunscreen with UVA and UVB protection of at least SPF 15. Also advise her to wear protective clothing, such as wide-brimmed hats and long sleeves, when outside during the day.

Cytotoxic drugs or steroids, which may increase her susceptibility to infection, can also mask typical signs and symptoms of infection. Tell your patient to contact her health care provider if she develops a fever or any other sign of infection, such as burning on urination (which may signal a urinary tract infection). Stress the importance of prompt treatment for any infection.

Encourage your patient to maintain a healthy, active lifestyle. She should get plenty of rest and eat a well-balanced diet. The Lupus Foundation recommends she follow the American Heart Association or American Cancer Society diet. Although she can take a daily multivitamin, she shouldn't take excessive vitamin doses. She may need a special diet if she has hypertension, dyslipidemia, or hyperglycemia from drug therapy.

Tell her to avoid smoking because it increases her risk of cardiovascular disease and can worsen the disease's effects on her heart and blood vessels. She should ask her health care provider whether she can drink alcohol in moderation.

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Taking the bite out of lupus

By understanding SLE and supporting your patient as she gets the treatment she needs, you can help her manage her condition, avoid flares, and lead a normal life.

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Lupus enlightened

In systemic lupus erythematosus, light or another exogenous or endogenous agent can start a cell-mediated immune reaction damaging epidermal cells. The injury releases a large number of antigens, some of which may return to the skin in the form of immune complexes. Immune complexes are also formed in the skin by a reaction of local DNA with antibody, which may be deposited beneath the epidermal basement membrane zone. (Source: Pathology, 3rd edition, E Rubin, JL Farber, Lippincott Williams & Wilkins,1999.)

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Who's at risk for SLE?

Nine times more common in women than in men, systemic lupus erythematosus (SLE) typically strikes between the ages of 15 and 45, although it can also develop in children and older adults. It's more common in women of African-American, Hispanic, Asian, and American Indian descent than in white women. Although the cause of the abnormal immune responses in SLE is unknown, both genetic and environmental factors appear to play a role.

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Sorting out diagnostic criteria

A person with at least four of these eleven criteria over time is considered to have systemic lupus erythematosus.

Malar rash: a flat or raised rash that covers the bridge of the nose and spreads across the cheeks.

Discoid rash: raised scaly disk-shaped patches that cause scarring.

Figure. Di

Figure. Di

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Photosensitivity: a rash caused by exposure to ultraviolet light.

Oral ulcers: oral or nasopharyngeal ulceration, usually painless.

Arthritis: tenderness and swelling in two or more peripheral joints.

Serositis: pleuritis or pericarditis documented by ECG, pericardial rub, or pericardial effusion.

Renal disorder: persistent proteinuria or cellular casts.

Neurologic disorder: seizures or psychosis in the absence of other causes.

Hematologic disorder: hemolytic anemia or leukopenia, with a total white blood cell count below 4,000/mcl or lymphopenia, with a lymphocyte count below 1,500/mcl on at least two occasions; or thrombocytopenia, with a platelet count below 100,000/mcl in absence of offending drugs.

Immunologic disorder: positive lupus erythematosus (LE) cell preparation, antibody to native DNA (anti-dsDNA), antibody to Smith nuclear antigen (anti-Sm), or a false-positive test for syphilis for at least 6 months.

Antinuclear antibody (ANA): a positive titer of ANA by immunofluorescence in the absence of drugs known to be associated with drug-induced lupus.

Adapted from The 1982 revised criteria for the classification of systemic lupus erythematosus, Arthritis & Rheumatism, EM Tan et al., November 1982.

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If your patient is pregnant…

Most women with systemic lupus erythematosus (SLE) can have successful pregnancies, although they're considered high risk and may have a flare during pregnancy or after childbirth. Advise your patient to seek prenatal care from an obstetrician who has experience with high-risk pregnancies.

Neonatal lupus, although rare, sometimes occurs in newborns whose mothers have SLE. Signs most commonly include skin rash or hematologic abnormalities. The infant's signs and symptoms usually resolve spontaneously without treatment over time—typically in a few weeks—without long-term effects.

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Teaching your patient to prevent a flare

Give these tips to a patient with systemic lupus erythematosus.

  • Learn to recognize that a flare is coming. You may feel very tired or have pain, a rash, a fever, stomach discomfort, headache, or dizziness just before you have a flare.
  • Tell your health care provider you think a flare is coming.
  • Try to set realistic goals and priorities and develop coping skills to help limit stress.
  • Limit the amount of time you spend in the sun.
  • Eat a healthy diet.
  • Get enough rest and quiet.
  • Exercise moderately when you can.
  • Develop a support system by surrounding yourself with family and friends you trust and feel comfortable with.

Source: The Many Shades of Lupus, National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS) Information Clearing House, National Institutes of Health, Bethesda, Md., NIH Publication No. 01-4958, 2001. http://www.niams.nih.gov/hi/topics/lupus/shades/index.htm. Accessed on July 22, 2005.

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SELECTED WEB SITES

American College of Rheumatology http://www.rheumatology.org

Arthritis Foundation http://www.arthritis.org

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

S.L.E. Lupus Foundation http://www.lupusny.org/

Last accessed on October 3, 2005.

Joan Rooney is a rheumatology nurse practitioner for Rheumatology Disease Associates in Willow Grove, Pa.

The author has disclosed that she has no significant relationship with or financial interest in any commercial companies that pertain to this educational activity.

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SELECTED REFERENCES

, September 1999 (amended 2005).American College of Rheumatology Ad Hoc Committee on Systemic Lupus Erythematosus Guidelines. Guidelines for referral and management of systemic lupus erythematosus in adults. Arthritis & Rheumatism. 42(9):1785–1796
Criscione LG, Pisetsky DS. The pathogenesis of systemic lupus erythematosus. Bulletin on the Rheumatic Diseases. 52(6):1–6, 2003.
Cush JJ, et al. Rheumatology: Diagnosis and Therapeutics, 2nd edition. Philadelphia, Pa., Lippincott Williams & Wilkins, 2000.
    Hahn BH. Systemic lupus erythematosus. In Kasper DL, et al. (eds), Harrison's Principles of Internal Medicine, 16th edition. New York, N.Y., McGraw-Hill, 2005.
      Harris ED Jr, Genovese MC (eds). Primary Care Rheumatology. Philadelphia, Pa., W.B. Saunders, 2000.
        Lane SK, Gravel JW. Clinical utility of common serum rheumatologic tests. American Family Physician. 65(6):1073–1080, March 15, 2002.
        Lupus: A Patient Care Guide for Nurses and Other Health Professionals. Bethesda, Md., National Institutes of Health, 2001.
          Paget S, et al. Manual of Rheumatology and Outpatient Orthopedic Disorders: Diagnosis and Treatment, 4th edition. Philadelphia, Pa., Lippincott Williams & Wilkins, 2000.

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            Petrie M. Systemic lupus erythematosus: An update for clinicians. International Journal of Advances in Rheumatology. 1(2):53-57, 2003.
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