1. Focus on Systemic Lupus Erythematosus (SLE)
(Relates to Chapter 65, “Nursing Management: Arthritis and Connective Tissue Diseases,” in the textbook)
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2. Systemic Lupus Erythematosus
Chronic multisystem inflammatory autoimmune disease
Associated with abnormalities of immune system
Results from interactions among genetic, hormonal, environmental, and immunologic factors
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3. Systemic Lupus Erythematosus
Affects the
Skin
Joints
Serous membranes
Renal system
Hematologic system
Neurologic system
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4. Systemic Lupus Erythematosus
SLE affects 2 to 8 persons per 100,000 in United States.
Most cases occur in women of child-bearing years.
African, Asian, Hispanic, and Native Americans 3 times more likely to develop than whites
Women are 10 times more likely to develop SLE than men.
SLE is characterized by variability within and among persons.
Its chronic unpredictable course is marked by alternating periods of exacerbation and remission.
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5. Etiology and Pathophysiology
Etiology is unknown.
Most probable causes
Genetic influence
Hormones
Environmental factors
Certain medications
Multiple susceptibility genes from the HLA complex show associations with SLE, including HLA-DR3.
Onset or exacerbation of disease symptoms sometimes occurs after the onset of menarche, with the use of oral contraceptives, and during and after pregnancy.
The disease tends to worsen in the immediate postpartum period.
Sun exposure and sunburn are the most common environmental triggers.
Medications include procainamide (Pronestyl), hydralazine (Apresoline), and a number of antiseizure drugs.
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6. Etiology and Pathophysiology
Autoimmune reactions directed against constituents of cell nucleus, DNA
Antibody response related to B and T cell hyperactivity
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7. Clinical Manifestations
Ranges from a relatively mild disorder to rapidly progressing, affecting many body systems
Most commonly affects the skin/muscles, lining of lungs, heart, nervous tissue, and kidneys
No characteristic pattern occurs in the progressive involvement of SLE.
Any organ can be affected by an accumulation of circulating immune complexes.
Generalized complaints such as fever, weight loss, arthralgia, and excessive fatigue may precede exacerbation of disease activity.
{See next slide for figure.}
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8. Multisystem Involvement of SLE
Fig Multisystem involvement in systemic lupus erythematosus.
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9. Clinical Manifestations
Dermatologic
Cutaneous vascular lesions
Butterfly rash
Oral/nasopharyngeal ulcers
Alopecia
Cutaneous vascular lesions can appear in any location but are most likely to develop in sun-exposed areas.
About 20% of patients have discoid (round coin-shaped) lesions.
A small number of patients have persistent lesions, photosensitivity, and mild systemic disease in a syndrome referred to as subacute cutaneous lupus.
{See next slide for figure.}
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Butterfly Rash of SLE
Fig Butterfly rash of systemic lupus erythematosus.
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11. Clinical Manifestations
Musculoskeletal
Polyarthralgia with morning stiffness
Arthritis
Swan neck fingers
Ulnar deviation
Subluxation with hyperlaxity of joints
Polyarthralgia with morning stiffness is often the patient’s first complaint and may precede by many years the onset of multisystem disease.
Arthritis occurs in more than 90% of patients with SLE.
{See next slide for figure.}
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Swan Neck Deformity
Fig Typical deformities of rheumatoid arthritis. D, Swan neck deformity.
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13. Clinical Manifestations
Cardiopulmonary
Tachypnea
Pleurisy
Dysrhythmias
Accelerated CAD
Pericarditis
Cardiac involvement may include dysrhythmias resulting from fibrosis of the sinoatrial and atrioventricular nodes.
This occurrence is an ominous sign of advanced disease, contributing significantly to the morbidity and mortality seen in SLE.
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14. Clinical Manifestations
Renal
Lupus nephritis
Ranging from mild proteinuria to glomerulonephritis
Primary goal in treatment is slowing the progression.
Lupus nephritis (LN) occurs in approximately 50% of patients with SLE.
Treatment typically includes corticosteroids, cytotoxic agents (cyclophosphamide [Cytoxan]), immunosuppressive agents (azathioprine [Imuran]), and cyclosporine.
A newer drug (mycophenolate mofetil [CellCept]) may be more effective and less toxic than cyclophosphamide, which has been the standard of treatment.
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15. Clinical Manifestations
Nervous system
Generalized/focal seizures
Peripheral neuropathy
Cognitive dysfunction
Disorientation
Memory deficits
Psychiatric symptoms
Generalized or focal seizures are the most common manifestation involving the central nervous system (CNS), and occur in as many as 15% of patients with SLE by the time of diagnosis.
Seizures are generally controlled by corticosteroids or antiseizure drugs.
Various psychiatric disorders are reported in SLE, including mood disorders, anxiety, and psychosis, although they may also be related to the stress of having a major illness or to associated drug therapies.
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16. Clinical Manifestations
Hematologic
Formation of antibodies against blood cells
Anemia
Leukopenia
Thrombocytopenia
Some patients develop a tendency toward coagulopathy involving excessive bleeding or blood clot development.
A manifestation of antiphospholipid antibody syndrome is a common cause of hypercoagulability in SLE patients, many of whom benefit from high-intensity treatment with warfarin (Coumadin).
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17. Clinical Manifestations
Infection
Increased susceptibility to infection
Fever should be considered serious.
Patients with SLE appear to have increased susceptibility to infection, possibly related to defects in the ability to phagocytize invading bacteria, deficiencies in production of antibodies, and the immunosuppressive effects of many antiinflammatory drugs.
Infection is a major cause of death, with pneumonia being the most common infection.
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Diagnostic Studies
No specific test
SLE is diagnosed primarily on criteria related to patient history, physical examination, and laboratory findings.
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Diagnostic Studies
For a complete list of diagnostic criteria, see Table in book.
SLE is characterized by the presence of ANA, and its identification establishes the existence of an autoimmune disease.
Other antibodies include anti-DNA, antineuronal, anticoagulant, anti-WBC, anti–red blood cell (RBC), antiplatelet, antiphospholipid, and anti–basement membrane.
High levels of anti-DNA are rarely found in any condition other than SLE, and anti-Sm seems to be found almost exclusively in SLE.
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Collaborative Care
Drug therapy
NSAIDs
Antimalarial drugs
Steroid-sparing drugs
Corticosteroids
Immunosuppressive drugs
NSAIDs continue to be an important intervention, especially for patients with mild polyarthralgias or polyarthritis.
Antimalarial agents such as hydroxychloroquine (Plaquenil) often are used to treat fatigue and moderate skin and joint problems.
Steroid-sparing immunosuppressants such as methotrexate can serve as an alternate treatment and are prescribed in combination with folic acid to decrease minor side effects of corticosteroids.
Immunosuppressive drugs such as azathioprine (Imuran) and cyclophosphamide (Cytoxan) may be prescribed to reduce the need for long-term corticosteroid therapy.
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21. Nursing Management Nursing Assessment
Assess patient’s physical, psychologic, and sociocultural problems with long-term management of SLE.
Assess pain and fatigue daily.
Subjective and objective data that should be obtained from the patient with SLE are presented in Table
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22. Nursing Management Nursing Diagnoses
Fatigue
Acute pain
Impaired skin integrity
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23. Nursing Management Planning
Overall goals
Have satisfactory pain relief.
Comply with therapeutic regimen to achieve maximum symptom management.
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24. Nursing Management Planning
Overall goals (cont’d)
Demonstrate awareness of, and avoid activities that cause, disease exacerbation.
Maintain optimal role function and a positive self-image.
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25. Nursing Management Nursing Implementation
Health promotion
Prevention of SLE is not possible.
Promote early diagnosis and treatment.
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26. Nursing Management Nursing Implementation
Acute intervention
During exacerbation, patient will become abruptly, dramatically ill.
Record severity of symptoms and response to therapy.
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27. Nursing Management Nursing Implementation
Acute intervention (cont’d)
Observe for
Fever pattern
Joint inflammation
Limitation of motion
Location and degree of discomfort
Fatigability
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28. Nursing Management Nursing Implementation
Acute intervention (cont’d)
Monitor weight and I&O.
Collect 24-hour urine sample.
Assess neurologic status.
Explain nature of disease.
Provide support.
Collection of 24-hour urine samples for protein and creatinine clearance may be ordered.
Careful assessment of neurologic status includes observing for visual disturbances, headaches, personality changes, seizures, and forgetfulness.
Psychosis may indicate CNS disease or may be the effect of corticosteroid therapy.
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29. Nursing Management Nursing Implementation
Ambulatory and home care
Emphasize health teaching.
Reiterate that adherence to treatment does not necessarily halt progression.
Minimize exposure to precipitating factors.
Teach the patient that a variety of factors may increase disease activity, such as fatigue, sun exposure, emotional stress, infection, drugs, and surgery.
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30. Nursing Management Nursing Implementation
Lupus and pregnancy
Infertility can result from SLE’s regimen.
Women with serious SLE should be counseled against pregnancy.
Neonatal lupus erythematosus (NLE) may occur in infants born of women with SLE.
The SLE patient should understand that spontaneous abortion, stillbirth, and intrauterine growth retardation are common problems with pregnancy.
They occur because of deposits of immune complexes in the placenta and because of inflammatory responses in the placental blood vessels.
For the best outcome, pregnancy should be planned at a point when disease activity is minimal.
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31. Nursing Management Nursing Implementation
Psychosocial issues
Counsel patient and family that SLE has good prognosis.
Physical effects can lead to isolation, self-esteem, and body image disturbances.
Assist patient in developing goals.
Families are anxious about hereditary aspects and want to know whether their children will also have SLE.
Consultation with a dermatologist may be recommended for appropriate treatment and cosmetic products to conceal the rash.
However, pain and fatigue are cited most frequently as interfering with quality of life.
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32. Nursing Management Evaluation
Expected outcomes
Completion of priority activities
Verbalization of having more energy
Expression of satisfaction with pain relief measures
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33. Nursing Management Evaluation
Expected outcomes (cont’d)
Performance of activities of daily living without pain
Limitation of direct exposure to sun and use of sunscreen
No open skin lesions
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34. Nursing Management Evaluation
Expected outcomes (cont’d)
Expression of satisfaction with activity level
Pacing of activities to match level of tolerance
Expression of confidence in ability to manage SLE over time and in home environment
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Audience Response Question
A patient is undergoing diagnostic testing for symptoms of polyarthralgia, fatigue, and hair loss. Laboratory results include the presence of anti-DNA, antinuclear antibodies, and anti-Smith in the blood. The nurse recognizes that these findings are most likely to be related to:
1. Systemic sclerosis.
2. Rheumatoid arthritis.
3. Chronic fatigue syndrome.
4. Systemic lupus erythematosus.
Answer: 4
Rationale: No specific test is diagnostic for systemic lupus erythematosus (SLE), but a variety of abnormalities may be present in the blood. SLE is characterized by the presence of antinuclear antibodies (ANA), and its identification establishes the existence of an autoimmune disease. Other antibodies include anti-DNA, antineuronal, anticoagulant, anti-WBC, anti–red blood cell (RBC), antiplatelet, antiphospholipid, and anti–basement membrane. Tests that are most specific for SLE include anti–double-stranded DNA and anti-Smith (Sm). High levels of anti-DNA are rarely found in any condition other than SLE, and anti-Sm seems to be found almost exclusively in SLE. The lupus erythematosus (LE) cell prep test is a nonspecific test for SLE and is positive in other rheumatic diseases.
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Case Study
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Case Study
36-year-old woman was admitted 8 years ago with polyarthritis, facial and palmar erythema, and general malaise.
She was diagnosed with probable systemic lupus erythematosus.
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Case Study
She was started on prednisone 100 mg/every other day.
Within a few weeks of taking prednisone, she developed cushingoid syndrome.
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Case Study
She has also had intermittent tonic - clonic (grand mal) seizures that are treated with Dilantin.
During the past year, her lab studies indicate early renal failure.
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Case Study
She has had occasional UTIs that have responded to treatment.
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Discussion Questions
What common clinical manifestations of SLE does she have?
What psychosocial issues should you discuss with her?
What patient teaching should you do with her?
Polyarthritis, facial and palmar erythema, and general malaise.
Concerns over her long-term prognosis, family planning, consultation about managing rash, and stress management.
Discuss the avoidance of triggers (e.g., sun exposure, stress)
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