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Description/Etiology
Systemic lupus erythematosus (SLE; also known as lupus) is an incurable inflammatory autoimmune disease
characterized by the production of autoantibodies that cause diverse clinical manifestations, including skin rashes
and damage to multiple organs. SLE has a variable clinical course, with manifestations ranging from mild to life

Systemic Lupus
Erythematosus

threatening. The condition often presents as a relapsing-remitting illness with acute attacks separated by periods of no
symptoms, although some patients have constant symptoms with intermittent acute attacks.
The exact cause of SLE is unknown; certain viruses (e.g., Epstein-Barr virus), as well as genetic, hormonal, and
environmental factors, are thought to contribute to disease onset (see Risk Factors, below). Diagnosis of SLE is
based on patient history, physical examination, and blood tests. According to diagnostic criteria from the American
College of Rheumatology, a patient must have at least four of the following signs and symptoms, consecutively or
concurrently, to be diagnosed with SLE: (1) malar (butterfly) rash on the cheeks and nose; (2) discoid rash anywhere
on the body with scaling and scarring; (3) photosensitivity; (4) oral or nasopharyngeal ulcers; (5) pericarditis
or pleuritis; (6) non-erosive arthritis; (7) kidney dysfunction, including proteinuria; (8) neurological disorder,
including seizures or psychosis; (9) hematologic disorder, including hemolytic anemia, leukopenia, lymphopenia, or
thrombocytopenia; (10) immunologic disorder, including the presence of anti-DNA antibodies, anti-Smith antibodies,
anti-cardiolipin IgG/IgM, lupus anticoagulant, or a false-positive syphilis test; and (11) a positive test for antinuclear
antibodies (ANAs).
SLE treatment goals focus on managing symptoms, controlling disease activity to prevent progressive loss of organ
function, and minimizing SLE-related disability. Treatment options include medications (e.g., corticosteroids,
antimalarial medications, immunosuppressants), physical/occupational therapy (PT/OT), and psychosocial support.
Education is essential to maintain good quality of life with this chronic disease. Most deaths result from cardiovascular
and pulmonary complications or infections.

Facts and Figures


ICD-10
M32

ICD-10-CAN
M32

Authors
Sharon Richman, MSPT
Tanja Schub, BS

Reviewers
Eliza Schub, RN, BSN
Cinahl Information Systems
Glendale, California
Rosalyn Robinson, RN, DNP,
APNP, FNP-BC
Cinahl Information Systems
Glendale, California
Nursing Executive Practice Council
Glendale Adventist Medical Center
Glendale, California

Editor
Diane Pravikoff, RN, PhD, FAAN
Cinahl Information Systems

SLE affects 3050:100,000 people, with an annual incidence of 1.67.6:100,000. Onset is usually between the ages
of 15 and 45. Women are affected 8 times more often than men. Blacks, Hispanics, and Asian Americans are affected
34 times more often than Whites, and often have more severe disease. Women with SLE have a 750-fold increased
risk for coronary heart disease. The 5-year survival rate in patients diagnosed with SLE is 50%.

Risk Factors
Risk factors for SLE include exposure to certain viruses (e.g., Epstein-Barr virus), environmental factors (e.g., sunlight,
thermal burns), and hormonal factors (because SLE is most often diagnosed in women of childbearing age). Genetic
factors likely increase risk for SLE; the disease shows a 2450% concordance in identical twins, and individuals with a
first-degree relative with SLE have an 8-fold increased risk of developing the disease themselves. In addition, a number
of drugs have been shown to induce SLE, including hydralazine, procainamide, isoniazid, chlorpromazine, and some
antiseizure medications.

Signs and Symptoms/Clinical Presentation


Patients with SLE often present with musculoskeletal symptoms, including arthralgias, arthritis, joint swelling and
tenderness, and pain on movement. Cutaneous symptoms include malar rash (present in 50% of SLE patients),
cutaneous erythematous plaques with adherent scales, and areas of hyperpigmentation or hypopigmentation.
Cardiovascular and cardiopulmonary signs and symptoms include pericarditis, early-onset atherosclerosis,
myocarditis, pneumonia, angina, myocardial infarction, thromboemboli, pneumonitis, shrinking lung syndrome,
pulmonary bleeding, and vasculitis. Ocular signs and symptoms include retinopathy and retinal detachment with
vision loss. Central nervous system (CNS) signs and symptoms include impaired cognition, neurosis, psychosis,
seizures, stroke, headaches, mood disorders, and meningitis. Gastrointestinal signs and symptoms include pancreatitis
and bowel perforation. Other signs and symptoms include nephritis, renal failure, Raynauds phenomenon,
miscarriages, pre-eclampsia, fetal growth retardation and premature deliveries, and various infections (e.g., fungal

June 29, 2012

[Candida, Pneumocystis carinii], urinary tract infection, Salmonella, herpes zoster virus, cytomegalovirus).

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general informational overview of the subject for the healthcare professional. Cinahl Information Systems, 1509 Wilson Terrace, Glendale, CA 91206

Assessment

44 Patient History

Patient may report joint pain, morning stiffness, and functional limitations

44 Physical Findings of Particular Interest

Patient may present with fever, hypertension (secondary to renal involvement), lymphadenopathy, and/or splenomegaly
Auscultation may reveal pericardial friction rub and abnormal lung sounds
Integumentary examination of patient may reveal oral ulcers; lesions on the fingertips, elbows, toes, arms, and hands; and alopecia

44 Laboratory Tests That May Be Ordered

CBC may reveal leukopenia, anemia, and/or thrombocytopenia


Blood tests may be positive for the presence of autoantibodies (e.g., ANA, anti-double and single-stranded DNA, anti-Smith); serum complement levels
(e.g., C3, C4, Ch50) may be low; anti-Ig; syphilis test may be false-positive in patients with SLE

Serum creatinine levels and UA may reveal renal impairment

Treatment Goals

44 Relieve Symptoms, Slow Disease Progression, and Minimize Disease-Related Disability

Monitor vital signs, pulse oximetry readings, and for pain; assess all physiologic systems; and review laboratory test results (e.g., arterial blood gases
[ABGs] and EKG readings

Administer prescribed medications, which may include nonsteroidal anti-inflammatory drugs (NSAIDs) to reduce inflammation; topical or intralesional
corticosteroids for cutaneous lesions; antimalarial medications (e.g., hydroxychloroquine) for arthritis, pleuritis, rashes, and pericarditis; systemic
corticosteroids (e.g., prednisone) for life- or organ-threatening disease; immunosuppressive medications (e.g., cyclophosphamide) for organ damage
(e.g., nephritis) and severe thrombocytopenia; and anticoagulants to prevent miscarriage, thromboemboli, and stroke
Monitor treatment efficacy; consult a drug information resource for a complete listing of potential drug complications and adverse effects
Monitor temperature and for signs of dehydration; encourage fluid intake
Provide gentle massage, hot or cold packs, and frequent positional changes; educate about biofeedback, meditation, and the use of energy
conservation techniques
Request referral to PT/OT for exercise to improve functional mobility and increase independence with activities of daily living (ADLs)

44 Manage Psychosocial Aspects of SLE and Provide Emotional Support

Assess patients anxiety level and coping ability; educate and encourage discussion about feelings regarding illness, functional limitations, and the
importance of independently performing ADLs as much as possible without exacerbating symptoms

As appropriate, request referral to a mental health clinician for counseling, chaplain services/spiritual leader for support, and/or a social worker for
identification of local resources for in-home care, support groups, or education programs

Food for Thought


44 Pregnant women with SLE are at increased risk for disease exacerbations and pregnancy complications (e.g. fetal loss); exacerbations during pregnancy
are less common in women in remission for ~6 months prior to conception. In a recent study, researchers found that 59% of women with SLE did not have
contraceptive counseling in the last year (Yazdany et al., 2011)
44 In March 2011, the U.S. Food and Drug Administration (FDA) approved belimumab (Benlysta), a monoclonal antibody specific for soluble human B
lymphocyte stimulator protein (BLyS; i.e., a cytokine important for the survival of B cells that is overexpressed in patients with SLE and other autoimmune
diseases), for treatment of SLE in patients receiving standard therapy; belimumab is the first targeted therapy approved for treatment of SLE

Red Flags
44 Female patients should avoid birth control pills due to risk for thromboembolism
44 Institute seizure precautions for patients with CNS involvement
44 Avoid over-exercise in PT/OT with more frequent, shorter sessions, frequent rests, low resistance and repetitions, careful addition of new activities, and
monitoring for acute symptom flares

What Do I Need to Tell the Patient/Patients Family?


44 Educate about appropriate skin and oral care and the importance of sun protection
44 Educate about the link between acute symptom flares (e.g., fatigue, exacerbation of weakness, joint pain) and potential causes (e.g., stress, exposure to
sunlight, stopping medications, smoking)
44 Recommend pneumococcal vaccination and yearly influenza vaccination
44 Suggest finding more information from Lupus Foundation of America, Inc. (http://www.lupus.org/)

References

Barber, C., Gold, W. L., & Fortin, P. R. (2011). Infections in the lupus patient: Perspectives on prevention. Current Opinion in Rheumatology, 23(4), 358-365.
DynaMed. (2012, March 6). Systemic lupus erythematosus (SLE). Ipswich, MA: EBSCO Publishing. Retrieved June 26, 2012, from http://search.ebscohost.com/login.aspx?direct=true&db=dme&AN=115873&site=dynamed-live&scope=site
Tromp, K., & Salomon-Escoto, K. I. (2012). Systemic lupus erythematosus (SLE). In F. J. Domino (Ed.), The 5-minute clinical consult 2012 (20th ed., pp. 1280-1281). Philadelphia, PA: Wolters Kluwer Health/Lippincott Williams & Wilkins.
Unterman, A., Nolte, J. E., Boaz, M., Abady, M., Shoenfeld, Y., & Zandman-Goddard, G. (2011). Neuropsychiatric syndromes in systemic lupus erythematosus: A meta-analysis. Seminars in Arthritis and Rheumatism, 41(1), 1-11.
Yazdany, J., Trupin, L., Kaiser, R., Schmajuk, G., Gillis, J. Z., Chakravarty, E., & Schwarz, E. B. (2011). Contraceptive counseling and use among women with systemic lupus erythematosus: A gap in health care quality? Arthritis Care
& Research, 63(3), 358-365.