Focus on Systemic Lupus Erythematosus (SLE) (Relates to Chapter 65, “Nursing Management: Arthritis and Connective Tissue Diseases,” in the textbook) Copyright © 2011, 2007 by Mosby, Inc., an affiliate of Elsevier Inc.

Systemic Lupus Erythematosus Chronic multisystem inflammatory autoimmune disease Associated with abnormalities of immune system Results from interactions among genetic, hormonal, environmental, and immunologic factors Copyright © 2011, 2007 by Mosby, Inc., an affiliate of Elsevier Inc. 2

Systemic Lupus Erythematosus Affects the Skin Joints Serous membranes Renal system Hematologic system Neurologic system Copyright © 2011, 2007 by Mosby, Inc., an affiliate of Elsevier Inc. 3

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. Copyright © 2011, 2007 by Mosby, Inc., an affiliate of Elsevier Inc. 4

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. Copyright © 2011, 2007 by Mosby, Inc., an affiliate of Elsevier Inc. 5

Etiology and Pathophysiology Autoimmune reactions directed against constituents of cell nucleus, DNA Antibody response related to B and T cell hyperactivity Copyright © 2011, 2007 by Mosby, Inc., an affiliate of Elsevier Inc. 6

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.} Copyright © 2011, 2007 by Mosby, Inc., an affiliate of Elsevier Inc. 7

Multisystem Involvement of SLE Fig. 65-9. Multisystem involvement in systemic lupus erythematosus. Copyright © 2011, 2007 by Mosby, Inc., an affiliate of Elsevier Inc. 8

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.} Copyright © 2011, 2007 by Mosby, Inc., an affiliate of Elsevier Inc. 9

Copyright © 2011, 2007 by Mosby, Inc., an affiliate of Elsevier Inc. Butterfly Rash of SLE Fig. 65-10. Butterfly rash of systemic lupus erythematosus. Copyright © 2011, 2007 by Mosby, Inc., an affiliate of Elsevier Inc. 10

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.} Copyright © 2011, 2007 by Mosby, Inc., an affiliate of Elsevier Inc. 11

Copyright © 2011, 2007 by Mosby, Inc., an affiliate of Elsevier Inc. Swan Neck Deformity Fig. 65-4. Typical deformities of rheumatoid arthritis. D, Swan neck deformity. Copyright © 2011, 2007 by Mosby, Inc., an affiliate of Elsevier Inc. 12

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. Copyright © 2011, 2007 by Mosby, Inc., an affiliate of Elsevier Inc. 13

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. Copyright © 2011, 2007 by Mosby, Inc., an affiliate of Elsevier Inc. 14

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. Copyright © 2011, 2007 by Mosby, Inc., an affiliate of Elsevier Inc. 15

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). Copyright © 2011, 2007 by Mosby, Inc., an affiliate of Elsevier Inc. 16

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. Copyright © 2011, 2007 by Mosby, Inc., an affiliate of Elsevier Inc. 17

Copyright © 2011, 2007 by Mosby, Inc., an affiliate of Elsevier Inc. Diagnostic Studies No specific test SLE is diagnosed primarily on criteria related to patient history, physical examination, and laboratory findings. Copyright © 2011, 2007 by Mosby, Inc., an affiliate of Elsevier Inc. 18

Copyright © 2011, 2007 by Mosby, Inc., an affiliate of Elsevier Inc. Diagnostic Studies For a complete list of diagnostic criteria, see Table 65-14 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. Copyright © 2011, 2007 by Mosby, Inc., an affiliate of Elsevier Inc. 19

Copyright © 2011, 2007 by Mosby, Inc., an affiliate of Elsevier Inc. 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. Copyright © 2011, 2007 by Mosby, Inc., an affiliate of Elsevier Inc. 20

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 65-16. Copyright © 2011, 2007 by Mosby, Inc., an affiliate of Elsevier Inc. 21

Nursing Management Nursing Diagnoses Fatigue Acute pain Impaired skin integrity Copyright © 2011, 2007 by Mosby, Inc., an affiliate of Elsevier Inc. 22

Nursing Management Planning Overall goals Have satisfactory pain relief. Comply with therapeutic regimen to achieve maximum symptom management. Copyright © 2011, 2007 by Mosby, Inc., an affiliate of Elsevier Inc. 23

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. Copyright © 2011, 2007 by Mosby, Inc., an affiliate of Elsevier Inc. 24

Nursing Management Nursing Implementation Health promotion Prevention of SLE is not possible. Promote early diagnosis and treatment. Copyright © 2011, 2007 by Mosby, Inc., an affiliate of Elsevier Inc. 25

Nursing Management Nursing Implementation Acute intervention During exacerbation, patient will become abruptly, dramatically ill. Record severity of symptoms and response to therapy. Copyright © 2011, 2007 by Mosby, Inc., an affiliate of Elsevier Inc. 26

Nursing Management Nursing Implementation Acute intervention (cont’d) Observe for Fever pattern Joint inflammation Limitation of motion Location and degree of discomfort Fatigability Copyright © 2011, 2007 by Mosby, Inc., an affiliate of Elsevier Inc. 27

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. Copyright © 2011, 2007 by Mosby, Inc., an affiliate of Elsevier Inc. 28

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. Copyright © 2011, 2007 by Mosby, Inc., an affiliate of Elsevier Inc. 29

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. Copyright © 2011, 2007 by Mosby, Inc., an affiliate of Elsevier Inc. 30

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. Copyright © 2011, 2007 by Mosby, Inc., an affiliate of Elsevier Inc. 31

Nursing Management Evaluation Expected outcomes Completion of priority activities Verbalization of having more energy Expression of satisfaction with pain relief measures Copyright © 2011, 2007 by Mosby, Inc., an affiliate of Elsevier Inc. 32

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 Copyright © 2011, 2007 by Mosby, Inc., an affiliate of Elsevier Inc. 33

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 Copyright © 2011, 2007 by Mosby, Inc., an affiliate of Elsevier Inc. 34

Copyright © 2011, 2007 by Mosby, Inc., an affiliate of Elsevier Inc. 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. Copyright © 2011, 2007 by Mosby, Inc., an affiliate of Elsevier Inc. 35 35

Copyright © 2011, 2007 by Mosby, Inc., an affiliate of Elsevier Inc. Case Study Copyright © 2011, 2007 by Mosby, Inc., an affiliate of Elsevier Inc. 36

Copyright © 2011, 2007 by Mosby, Inc., an affiliate of Elsevier Inc. 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. Copyright © 2011, 2007 by Mosby, Inc., an affiliate of Elsevier Inc. 37

Copyright © 2011, 2007 by Mosby, Inc., an affiliate of Elsevier Inc. Case Study She was started on prednisone 100 mg/every other day. Within a few weeks of taking prednisone, she developed cushingoid syndrome. Copyright © 2011, 2007 by Mosby, Inc., an affiliate of Elsevier Inc. 38

Copyright © 2011, 2007 by Mosby, Inc., an affiliate of Elsevier Inc. 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. Copyright © 2011, 2007 by Mosby, Inc., an affiliate of Elsevier Inc. 39

Copyright © 2011, 2007 by Mosby, Inc., an affiliate of Elsevier Inc. Case Study She has had occasional UTIs that have responded to treatment. Copyright © 2011, 2007 by Mosby, Inc., an affiliate of Elsevier Inc. 40

Copyright © 2011, 2007 by Mosby, Inc., an affiliate of Elsevier Inc. 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) Copyright © 2011, 2007 by Mosby, Inc., an affiliate of Elsevier Inc. 41