Lupus Care

Systemic Lupus Erythematosus

Systems Potentially Affected by Lupus

General Manifestations of SLE

Dermatologic Manifestations

Musculoskeletal Manifestations

Hematologic Manifestations

Cardiopulmonary Manifestations

Renal Manifestations

Central Nervous System Manifestations

Gastrointestinal Manifestations

Ophthalmologic Manifestations

Pregnancy

Infection

Nutrition

Lupus symptoms tend to present themselves according to the body system affected. These symptoms vary over time in intensity and duration for each patient as well as from patient to patient. To effectively care for a lupus patient, the nurse or other health professional needs an up-to-date knowledge and understanding of the disease, its many manifestations, and its changing and often unpredictable course.

This chapter provides an overview of general and system-specific lupus manifestations and identifies potential problems. Suggested health care interventions for the nonhospitalized lupus patient are given. Many of these interventions can be modified for the hospitalized patient. The information and nursing interventions described in this chapter are not meant to be inclusive, but to provide the practitioner with guidelines for developing a care plan specific to the needs of each lupus patient.

As a care plan is developed, the health professional should keep in mind the importance of frequently reassessing the patient’s status over time and adjusting treatment to accommodate the variability of SLE manifestations. An additional and very important element of working with the lupus patient is to incorporate the patient’s needs and routines in the plan of care. Adjusting nursing interventions and medical protocols to the patient’s needs not only recognizes the value of the patient as an authority on her or his own illness but also can improve patient compliance and result in an improved quality of life.

Working together, the care provider and the patient have much to offer each other. The rewards are tremendous for the patient and family as independence is gained and the trust in the ability to care for oneself is strengthened.

Systemic Lupus Erythematosus

General Manifestations
Fatigue, fever, psychological and emotional effects.

Specific Manifestations
Dermatologic: Butterfly rash, photosensitivity, DLE, subcutaneous LE, mucosal ulcers, alopecia, pain and discomfort, pruritus, bruising.

Musculoskeletal: Arthralgias, arthritis, other joint complications.

Hematologic: Anemia, decreased WBC count, thrombocytopenia, lupus anticoagulants, false-positive VDRL, elevated ESR.

Cardiopulmonary: Pericarditis, myocarditis, myocardial infarction, vasculitis, pleurisy, valvular heart disease.

Renal: Asymptomatic microscopic renal involvement, renal failure, fluid and electrolyte imbalance, urinary tract infection.

Central Nervous System (CNS): General CNS symptomology, cranial neuropathies, cognitive impairment, mental changes, seizures.

Gastrointestinal: Anorexia, ascites, pancreatitis, mesenteric or intestinal vasculitis.

Ophthalmologic: Eyelid problems, conjunctivitis, cytoid bodies, dry eyes, glaucoma, cataracts, retinal pigmentation.

Other Key Issues
Pregnancy: Lupus flare, miscarriage or stillbirth, pregnancy-induced hypertension, neonatal lupus.

Infection: Increased risk of respiratory tract, urinary tract, and skin infections; opportunistic infections.

Nutrition: Weight changes; poor diet; appetite loss; problems with taking medications; increased risk of cardiovascular disease, diabetes, osteoporosis, and kidney disease.

General Manifestations of SLE

Overview
Fatigue is a nearly universal complaint of patients with SLE even when no other manifestations of the disease are present. The cause of this debilitating fatigue is not known. The patient should be evaluated for factors that may exacerbate fatigue, such as overexertion, insomnia, depression, stress, anemia, and other inflammatory diseases. Fatigue in SLE patients may be lessened by adequate rest, healthful diet, exercise, and attention to psychosocial factors.

Many patients with SLE experience changes in weight. At least one-half of patients report weight loss before being diagnosed with SLE. Weight loss in SLE patients may be attributed to a decreased appetite, side effects of medications, gastrointestinal problems, or fever. Weight gain can occur in some patients and may be due in part to prescribed medications, especially corticosteroids, or fluid retention from kidney disease.

Episodic fever is experienced by more than 80% of SLE patients, and there is no particular fever pattern. Although high fevers can occur during a lupus flare, low-grade fevers are more frequently seen. A complicating infection is often the cause of an elevated temperature in a patient with SLE. The patient’s WBC count may be normal to elevated with an infection, but low with SLE alone. However, certain medications, such as immunosuppressives, will suppress the WBC even in the presence of fever. Therefore, it is important to rule out other causes of a fever, including an infection or a drug reaction. Urinary and respiratory infections are common in SLE patients.

Potential Problems

1. Inability to complete activities of daily living (ADL) because of fatigue, weakness, and psychological difficulties
   
2. Changes in weight
   
3. Fever

Nursing Interventions
Objective: Minimize fatigue.

1. Assess patient’s general fatigue level.
   
2. Assess for the presence of depression, anxiety, and other stressors.
   
3. Conduct assessment to determine patient’s daily activities that contribute to fatigue.
   
4. Help patient to develop an energy-conserving plan for completing daily and other activities and work.
   
5. Suggest planning for rest periods as needed throughout the day to conserve energy.
   
6. Encourage patient to get 8–10 hours of sleep at night.
   
7. Encourage exercise as tolerated.

Objective: Maintain weight at optimal range.

1. Assess patient’s prescription and non-prescription drug regimen and dosages.
   
2. Assess the patient’s usual daily dietary intake by asking her or him to keep a food diary.
   
3. Develop a dietary plan with the patient that encourages healthful eating. If the patient has nutrition-related lupus complications, refer her or him to a registered dietitian for specialized counseling.
   
4. Encourage exercise as tolerated.
   
5. Record patient’s weight at each visit.
   
6. Instruct patient to weigh herself or himself at home once a week and record it

Objective: Teach patient to recognize fever and signs and symptoms of infection.

1. Assess patient’s prescription and non-prescription drug regimen and dosages.
   
2. Monitor patient’s WBC count.
   
3. Teach patient to monitor temperature during a lupus flare.
   
4. Teach patient to look for signs and symptoms of infection, particularly urinary and respiratory infections. (Note: The cardinal signs of infection may be masked because of corticosteroids and antipyretic medications.)
   
5. Instruct patient to call physician if signs and symptoms of an infection appear or if a fever is elevated above normal baseline.

Objective: Assist patient in adjusting to physical and lifestyle changes.

1. Allow patient to express feelings and needs.
   
2. Assess patient’s usual coping mechanisms.
   
3. Acknowledge that feelings of denial and anger are normal.
   
4. Explore with patient sources of potential support and community resources.
   
5. Explore possible ways of concealing skin lesions and hair loss.
   
6. Encourage patient to discuss interpersonal and social conflicts that arise.
   
7. Encourage patient to accept help from others, such as counseling or a support group.

Objective: Recognize the signs and symptoms of depression and initiate a plan of care.

1. Assess patient for the major signs and symptoms of depression.
   
2. Assess patient’s interpersonal and social support systems.
   
3. Encourage patient to express feelings.
   
4. Initiate a referral to a mental health counselor or psychiatrist.

Note: For additional information, see the Patient Information Sheets (Chapter 7) on Living With Lupus and Skin Care and Lupus.

For further information and nursing interventions, see the section on infection in this chapter. Also see the Patient Information Sheets (Chapter 7) on Living With Lupus, Preventing Fatigue Due to Lupus, and Fever and Lupus.

Dermatologic Manifestations

Overview
Approximately 80% of patients with SLE have skin manifestations and often suffer from itching, pain, and disfigurement. The classic sign of SLE is the “butterfly” rash extending over the cheeks (malar area) and bridge of the nose. This rash ranges from a faint blush to a severe eruption with scaling. It is photosensitive, and it may be transitory or fixed. Between 55 and 85% of patients develop this rash at some time in the course of the disease.

Other rashes may occur elsewhere on the face and ears, upper arms, shoulders, chest, and hands. DLE is seen in 15–30% of patients with SLE. Subacute cutaneous LE, seen in about 10% of SLE patients, produces highly photosensitive papules that itch and burn. Skin changes, especially the butterfly rash and subacute cutaneous LE, can be precipitated by sunlight.

Some patients may develop mouth, vaginal, or nasal ulcers. Hair loss (alopecia) occurs in about one-half of SLE patients. Most hair loss is diffuse, but it may be patchy. It can be scarring or nonscarring. Alopecia may also be caused by corticosteroids, infection, or immunosuppressive drugs.

Raynaud’s phenomenon (paroxysmal vasospasm of the fingers and toes) frequently occurs in patients with SLE. For most patients, Raynaud’s phenomenon is mild. However, some SLE patients with severe Raynaud’s phenomenon may develop painful skin ulcers or gangrene on the fingers or toes.

Varying levels of pain and discomfort due to skin alterations may occur. Pruritus accompanies many types of skin lesions. Attacks of Raynaud’s phenomenon can cause a deep tingling feeling in the hands and feet that can be very uncomfortable. Both pain and itching may affect a patient’s ability to carry out activities of daily living (ADL).

Skin alterations in the lupus patient, particularly those of DLE, can be disfiguring. As a result, patients may experience fear of rejection by others, negative feelings about their body, and depression. Changes in lifestyle and social involvement may occur.

Potential Problems

1. Alteration in skin integrity
2. Alopecia
3. Discomfort (pain, itching)
4. Alteration in body image
5. Depression

Nursing Interventions
Objective: Minimize appearance of lesions.

1. Document appearance and duration of lesions and rashes.
2. Teach patient to minimize direct exposure to UV rays from sun and from fluorescent and halogen light bulbs. (Glass does not provide complete protection from UV rays.)
3. Instruct patient to use a sunscreen with an SPF of 15 or greater and wear protective clothing. Patients who are allergic to PABA will need to find a PABA-free sunscreen.
4. Provide information on hypoallergenic concealing makeup.
5. Instruct patient to avoid topical applications, such as hair dyes and skin creams, and the use of certain drugs that may make her or him more sensitive to the sun.

Objective: Alleviate discomfort.

1. For patients with mouth lesions, suggest a soft-food diet, lip balms, and warm saline rinses.
2. Instruct patient to take medications that may help to alleviate discomfort and itching as ordered. (The doctor may give the patient intralesional steroid injections.)
3. Suggest self-help measures for patients with Raynaud’s phenomenon, including:

• keep warm, particularly in cold weather; use chemical warmers, gloves, socks, hats; avoid air conditioning; use insulated drinking glasses for cold drinks; wear gloves when handling frozen or refrigerated foods;
• quit smoking;
• control stress; and
• exercise as tolerated.

Objective: Help patients to cope with potential psychological manifestations.

1. See the nursing interventions dealing with psychological issues under manifestions in this chapter.
   

Musculoskeletal Manifestations

Overview
Arthralgia or arthritis is experienced by 95% of SLE patients at some time during the course of the disease. Articular pain is the initial symptom in about one-half of patients eventually diagnosed with SLE. Morning stiffness and joint and muscle aching can also occur. Joint pain may be migratory; it is typically symmetric but is asymmetric in many patients. Joints may become warm and swollen. X rays of the joints usually do not show erosion or destruction of bone.

Unlike rheumatoid arthritis, the arthritis of SLE tends to be transitory. Proliferation of the synovium is more limited, and joint destruction is rare. The joints most commonly involved are those of the fingers, wrists, and knees; less commonly involved are the elbows, ankles, and shoulders.

Several joint complications may occur in SLE patients, including Jaccoud’s arthropathy and osteonecrosis. Subcutaneous nodules, especially in the small joints of the hands, are seen in about 5% of patients. Tendinitis, tendon rupture, and carpal tunnel syndrome are seen occasionally.

Potential Dermatologic Manifestations

• Butterfly rash on cheeks and bridge of nose
• Scaly, disk-shaped scarring rash (DLE)
• Erythematous, slightly scaly papules (subacute cutaneous LE)
• Psoriasiform or arcuate (curved) lesions on the trunk of the body (subacute cutaneous LE)
• Itching and burning
• Ulcers in the mouth, vagina, or nasal septum
• Atrophy (including striae or stretch marks)
• Impaired wound healing
• Easy bruising
• Petechiae
• Increased body hair (hirsutism)
• Steroid-induced ecchymosis
• Ulcers or gangrene on fingers or toes
• Alopecia

Potential Problems

1. Pain
2. Alteration in joint function

Nursing Interventions
Objective: Minimize pain from joint and muscle complications.

1. Assess and document joint complaints and appearance. Changes may be transient.
2. Assess patient’s self-management techniques for controlling pain.
3. Teach patient to apply heat or cold as appropriate.
4. Instruct patient in use of prescription and nonprescription pain medications.
5. If ordered by physician, teach patient to apply splints or braces.

Objective: Maintain joint function and increase muscle strength.

1. Suggest warm showers or baths to lessen stiffness and pain.
2. If indicated, refer patients with acutely inflamed joints to a physical therapist for passive range-of-motion (ROM) exercises. The physical therapist may train a family member to assist the patient with ROM exercises at home.
3. Teach patient that an inflamed joint should not bear weight and suggest that patient avoid strenuous activity.
4. If needed, assist patient to obtain crutches, a walker, or a cane.
5. Assist patient in developing a regular exercise plan that can be carried out during periods of remission. This plan should include exercises that promote muscle tone and fitness, minimize fatigue, and increase well-being.
6. Consider referring patient to an occupational therapist.

Note: For additional information, see the Patient Information Sheets (Chapter 7) on Exercise and Lupus, Preventing a Lupus Flare, and Joint Function and Lupus.

Hematologic Manifestations

Overview
Abnormal blood conditions are common in patients with SLE. Problems include anemia, thrombocytopenia, and other clotting disorders.

Anemia, which is common in SLE patients, reflects insufficient bone marrow activity, shortened RBC life span, or poor iron uptake. Aspirin, NSAIDs, and prednisone can cause stomach bleeding and exacerbate the condition. There is no specific therapy for this type of anemia. Immune-mediated anemia (or hemolytic anemia), which is due to antibodies directed at RBCs, is treated with corticosteroids.

Thrombocytopenia may occur and may respond to low-dose corticosteroids. Mild forms may not need to be treated, but a severe form requires high-dose corticosteroid or cytotoxic drugs. The major clinical features of APLs and APL syndrome are venous thrombosis, arterial thrombosis, and thrombocytopenia with a history of positive anticardiolipin antibody (ACL) tests.

Abnormal laboratory tests may include a false-positive VDRL test for syphilis. Fluorescent treponemal antibody absorption (FTA-ABS) and microhemagglutination-Treponema pallidum (MHA-TP) tests, which are more specific tests for syphilis, are almost always negative if the patient does not have syphilis. An elevated erythrocyte sedimentation rate (ESR) is a common finding in active SLE, but it does not always mirror disease activity.

Potential Problems

1. Inability to complete ADL because of fatigue and weakness
2. Anemia
3. Potential for hemorrhage
4. Potential to develop venous or arterial thromboses
5. Increased risk of infection

Nursing Interventions
Objective: Minimize fatigue.

1. Refer to the nursing interventions for fatigue in this chapter.

Objective: Recognize anemia and develop plan of care.

1. Monitor patient for signs and symptoms of anemia and for altered laboratory values.
2. Develop a plan with patient to conserve energy.
3. Teach patient the basics of good nutrition.
4. Instruct patient to take iron preparation medications as prescribed.

Objective: Minimize episodes of bleeding.

1. Assess patient for signs and symptoms of bleeding, such as petechiae, bruises, GI bleeding, blood in urine, ecchymoses, nose bleeds, bleeding from the gums, heavy menses, and bleeding between menstrual periods.
2. Teach patient why she or he is at risk of bleeding (low platelet count, anemia, thrombocytopenia) and to report episodes to physician.
3. Encourage patient to wear a medical alert bracelet or carry a card.
   
4. Teach patient measures to prevent bleeding, such as use of a soft toothbrush or an electric shaver.

Objective: Decrease risk of infection.

1. See the nursing interventions for infection in this chapter.

Note: For more information, see Laboratory Tests Used To Diagnose and Evaluate SLE (Chapter 3) and the Patient Information Sheet (Chapter 7) on Preventing Fatigue Due to Lupus.

Overview
Cardiac abnormalities contribute significantly to morbidity and mortality in SLE and are one of the most important clinical manifestations of the disease. In addition, involvement of the lungs and pleurae is common. Pericarditis, an inflammation of the pericardium, is the most common cardiac abnormality in SLE. Myocarditis, an inflammation of the heart muscle, may also occur, but is rare. Myocardial infarction, caused by atherosclerosis, has been reported in SLE patients below the age of 35 years.

Vasculitis (inflammation of the blood vessels) and serositis (inflammation of serous membranes) are frequently part of the autoimmune pathology of SLE. These conditions respond well to corticosteroids. Vasculitis may cause many different symptoms, depending on the system(s) most affected. Serositis most commonly presents as pleurisy or pericarditis. Pleuritic chest pain is common. Pleurisy is the most common respiratory manifestation in SLE. Attacks of pleuritic pain can also be associated with pleural effusions. Many patients complain of chest pain, but pericardial changes are not often demonstrated on clinical evaluation.

Potential Problems

1. Alterations in cardiac function
2. Potential for impaired gas exchange and ineffective breathing patterns
3. Alteration in tissue perfusion

Nursing Interventions
Objective: Detect changes in cardiac function.

1. Assess patient for signs and symptoms of potential cardiac problems.
2. Teach patient signs and symptoms of cardiac problems, including warning signs of a heart attack; reinforce the importance of reporting them to the physician.
3. Educate patient about medications.
4. Educate patient about a healthful diet and regular exercise as tolerated.

Objective: Maintain adequate gas exchange and effective breathing patterns.

1. Assess quality and depth of respirations; auscultate breath sounds.
2. Suggest measures to relieve pain, such as relaxation techniques, biofeedback, rest, and pain medications as ordered.
3. Encourage patients who smoke to quit.

Objective: Ensure adequate tissue perfusion.

1. Assess skin color and temperature; check for lesions.
2. Check capillary refill in the nailbeds.
3. Assess for presence of edema and pain in the extremities.
4. Stress the importance of not smoking.
5. Teach patient the basics of good foot care.
6. Teach patient to avoid cold temperatures and to keep the hands and feet warm, especially in winter months.
7. Teach patient signs and symptoms of vascular impairment that need to be reported to the physician, including a change in skin color or sensation or appearance of lesions.

Objective: Recognize the signs and symptoms of thromboses; refer for immediate medical attention.

1. Teach patient the signs and symptoms of potential venous or arterial thrombosis and reinforce the need to contact a physician immediately.

Note: For additional information, see the Patient Information Sheet (Chapter 7) on Serious Conditions Associated With Lupus.

Renal Manifestations

Overview
Renal damage is one of the most serious complications of SLE. The majority of lupus patients have some degree of asymptomatic microscopic kidney damage. Less than 50% have clinical renal disease, and most of those with renal disease have one of the milder forms. Kidney damage may necessitate treatment with corticosteroids, cytotoxic agents, dialysis, or renal transplantation.

Renal biopsy can be helpful in making decisions about drug treatments and determining prognosis by assessing the presence of active renal disease versus scarring.

Potential Problems

1. Impaired renal function
2. Fluid and electrolyte imbalance
3. Increased risk of infection

Nursing Interventions
Objective: Promptly recognize renal involvement and prevent complications.

1. Document any patient complaints or assessment findings that may indicate renal involvement.
2. Teach patient to watch for signs and symptoms of renal complications and report them promptly to the physician: headache, facial swelling, peripheral edema, dizziness, “foamy” urine (proteinuria), “coke-colored” urine (hematuria), or nocturia and urinary frequency.
3. Assess patient for early signs of heart or liver failure.
4. Refer patient to a dietitian for counseling on dietary changes to accommodate alterations in renal status.
5. Teach patient to take prescribed medications as ordered.
6. Stress the importance of referral and followup care with nephrologist if necessary.

Objective: Decrease fluid retention and edema.

1. Monitor electrolyte values.
2. Assess breath sounds and instruct patient to report shortness of breath or dyspnea.
3. Teach patient to maintain balanced fluid intake and output.
4. Monitor patient for signs and symptoms of extracellular fluid overload.
5. Instruct patient to weigh herself or himself daily to monitor fluid retention.
6. Monitor patient’s blood pressure and teach patient how to monitor it at home.

Objective: Minimize risk of infection.

1. Teach patient to watch for the signs and symptoms of urinary tract infection and to report them to the physician.
2. Instruct patient that corticosteroid therapy may mask the usual symptoms of infection and that she or he may have an altered immune response because of medications used to control SLE.
3. Teach patient to take antibiotics for urinary tract infection as prescribed.

Note: For additional information, see the Patient Information Sheet (Chapter 7) on Serious Conditions Associated With Lupus.

Central Nervous System Manifestations

Overview
Neurologic manifestations of SLE are common and vary from mild to severe. They can be difficult to diagnose and distinguish from other diseases. All portions of the nervous system may be affected, including the CNS. Definite diagnosis of CNS lupus may be difficult, as symptoms may be related to medications, other medical conditions, or to individual reactions to chronic illness.

Cranial or peripheral neuropathy occurs in 10–15% of patients; it is probably secondary to vasculitis in small arteries supplying nerves. Cerebrovascular accidents (strokes) are reported in approximately 15% of patients. Between 10 and 20% of patients experience seizures. Although cognitive impairment is believed to be very common, there are few measurements to document it.

Serious CNS involvement ranks behind only kidney disease and infection as a leading cause of death in lupus. However, the majority of SLE patients with CNS complications do not develop a life-threatening disease.

Potential Problems

1. Alteration in mental status, cognition, and perception
2. Altered ability to perform ADL and meet family responsibilities
3. Potential for injury

Nursing Interventions
Objective: Develop plan for patient to perform ADL appropriately and independently.

1. Assess and document patient’s mental status to determine her or his capabilities:

• general appearance;
• unusual body movements;
• speech patterns and word use;
• alertness and orientation to time, place, and person;
• memory of remote and recent past;
• perception of self and environment;
• affect and emotional stability;
• ability to solve problems; and
• presence of depression.

2. Support patient’s need to maintain some control over daily activities and decisions:

• encourage patient to plan and participate in daily routines;
• set aside time to develop trust and rapport with patient, and be consistently truthful (patients are keenly aware of inconsistencies in information provided).