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- 1. Erythematosus
- 2. Advances
- 3. Tests
- 4. Care
- 5. Medications
- 6. Psychosocial Aspects
- 7. Patient Info.
- 8. Resources
Publication Date: May 2001
Revised September 2006
Lupus: A Patient Care Guide for Nurses and Other Health Professionals
Chapter 1: Lupus Erythematosus
- Definition and Description
- Symptoms of SLE
- Diagnosis of SLE
- Treatment of SLE
- Medications for SLE
- Psychosocial Aspects
- Health Care Implications
The challenge of caring for a patient with lupus erythematosus draws on all the resources, knowledge, and strengths the health care team has to offer. Because of the unpredictable, highly individualized, and frequently changing nature of the disease as well as the intricacy of each patient’s needs, it is impossible to predict the outcome of treatment for one patient from the outcome of treatment for another. Careful listening to the person’s concerns; a cooperative, multidisciplinary approach; and a flexible plan of care will provide the patient with consistent, supportive care and the reassurance that her or his needs are being met.
In 1851, doctors coined this name for the disease because they thought the facial rash that frequently accompanies lupus looked like the bite of a wolf. There are four categories of lupus: systemic lupus erythematosus, cutaneous lupus, drug-induced systemic lupus erythematosus, and neonatal lupus (see “Pregnancy” in Chapter Four). Lupus: A Patient Care Guide for Nurses and Other Health Professionals is concerned primarily with systemic lupus erythematosus.
Systemic lupus erythematosus (SLE, or lupus) is a chronic, inflammatory, multisystem disorder of the immune system. In SLE, the body develops antibodies that react against the person’s own normal tissue. These antibodies are markers for SLE, and are one indicator of many immune system abnormalities that lead to clinical manifestations.
The course is unpredictable and individualized; no two patients are alike. Lupus is not contagious, infectious, or malignant. It usually develops in young women of childbearing years, but many men and children also develop lupus. SLE also appears in the firstdegree relatives of people with lupus more often than it does in the general population, which indicates a hereditary component. However, most cases of SLE occur sporadically, indicating that both genetic and environmental factors play a role in the development of the disease.
Lupus varies greatly in severity, from mild cases requiring minimal intervention to those in which significant and potentially fatal damage occurs to vital organs such as the lungs, heart, kidneys, and brain. For some patients, the disease can be characterized by “flares” of activity interspersed with periods of improvement or remission. A flare, or exacerbation, is increased activity of the disease process with an increase in physical manifestations and/or abnormal laboratory test values. Periods of improvement may last weeks, months, or even years. Other patients have continuous, or chronic, activity.
Although remissions are unusual, some patients never develop severe manifestations, and the outlook is improving for patients who do develop them.
Cutaneous lupus, which affects primarily the skin, is common among patients with lupus erythematosus. The most prevalent and severe form of cutaneous lupus is called chronic cutaneous lupus. It is commonly known as discoid lupus, but it has other forms as well (see “Dermatologic Manifestations” in Chapter 4).
Drug-induced lupus erythematosus develops after the use of certain drugs or biologics and has symptoms similar to those of SLE. The characteristics of this syndrome are pleuropericardial inflammation, pleuritic chest pain, pericarditis, fever, rash, and arthritis. Serologic changes can occur. The clinical and serologic signs usually subside gradually after the offending drug is discontinued. A wide variety of drugs is implicated in this form of lupus (see box, above). More recently, the newer TNF (tumor necrosis factor) inhibitors used to treat rheumatoid arthritis, Crohn’s disease, and ankylosing spondylitis have been associated with the development of lupus symptoms. As with lupus triggered by other drugs, the symptoms resolve when the agent is stopped.
Early symptoms of SLE might be vague, nonspecific, and easily confused with other pathological and functional disorders. Symptoms may be transient or prolonged, and individual symptoms often appear independently of the others. Moreover, a patient may have severe symptoms with few abnormal laboratory test results, and vice versa. The box on the next page lists the range of clinical symptoms seen in patients with lupus over the lifetime of the disease.
The onset of lupus may be acute, resembling an infectious process, or it may be a progression of vague symptoms over several years. As a result, diagnosing SLE is often a challenge. A consistent, thorough medical examination by a doctor familiar with lupus is essential to an accurate diagnosis. This must include a complete medical history and physical examination, laboratory tests, and a period of observation (possibly years). The doctor, nurse, or other health professional assessing a patient for lupus must keep an open mind about the varied and seemingly unrelated symptoms that the patient may describe. For example, a careful medical history may show that sun exposure, use of certain drugs, viral disease, stress, or pregnancy aggravates symptoms, providing a vital diagnostic clue.
No single laboratory test can definitely prove or disprove SLE. Initial screening includes a complete blood count (CBC); liver and kidney screening panels; laboratory tests for specific autoantibodies (e.g., antinuclear antibodies [ANA]) such as anti-Ro, anti-La, anti-dsDNA, anti-Sm, anti-RNP, lupus anticoagulant, and anticardiolipin; an anti-phospholipid antibody test; urinalysis; blood chemistries; and erythrocyte sedimentation rate (ESR). Abnormalities in these test results will guide further evaluations. AntidsDNA antibody or anti-Sm antibody are autoantibodies found only in lupus. Specific immunologic studies, such as those of complement components (e.g., C3 and C4) and other autoantibodies (e.g., anti-La, anti-Ro, anti-RNP), can be helpful in diagnosis. At times, biopsies of the skin or kidney using immunofluorescent staining techniques can support a diagnosis of SLE (see Chapter 3, Laboratory Tests Used to Diagnose and Evaluate SLE, for further information). A variety of laboratory tests, x rays, and other diagnostic tools are used to rule out other pathologic conditions and to determine the involvement of specific organs. It is important to note, however, that any single test may not be sensitive enough to reflect the intensity of the patient’s symptoms or the extent of the disease’s manifestations.
The American College of Rheumatology (ACR), an organization of doctors and associated health professionals who specialize in arthritis and related diseases of the bones, joints, and muscles, has developed and refined a set of classification criteria. If at least 4 of the 11 criteria develop at one time or individually over any period of observation, then the patient can be classified as an SLE patient for research purposes. However, a diagnosis of SLE can be made in a patient having fewer than four of these symptoms.
The treatment of SLE is as varied as its course. Although there is no cure for lupus and it is difficult to predict which treatment will be most effective for each patient, there have been significant gains in treating patients, and there is general consensus on several treatments.
A conservative regimen of physical and emotional rest, protection from direct sunlight, a healthful diet, prompt treatment of infections, and avoidance of aggravating factors are the mainstays of lupus therapy. In addition, for female patients, pregnancy must be planned for times when the disease is under control, and the patient is on allowable medications.
This basic component of everyone’s good health is essential for the person with lupus. The fatigue of lupus is not sleepiness or tiredness from physical exertion, but rather a frequent, persistent complaint often described as a “bone-tired feeling” or a “paralyzing fatigue.” Normal rest often does not refresh the patient or eliminate the tiredness due to lupus, and fatigue may persist despite normal laboratory test results. The patient and family need instruction on how to use this tiredness as a guide for scheduling activity and rest. It must be reinforced that this need for rest is not laziness. Eight to 10 hours a night of restful sleep, naps, and “timeouts” during the day are basic guidelines. Physical activity should be encouraged as the patient can tolerate it.
However, in some patients, the fatigue is more related to fibromyalgia than to the activity of their lupus. An individualized exercise routine may promote wellbeing, especially in a patient with fibromyalgia. Some scientists believe that the incidence of fibromyalgia is increased in patients with lupus. They do not know why, but one hypothesis is that inflammation from lupus may lead to the rewiring of the pain pathways in the central nervous system.
A patient’s emotional stressors should be carefully assessed, because they may play a role in triggering a flare. The patient should be instructed on how to avoid these stressful situations. However, the physical manifestations of lupus must be treated as they present themselves while the emotional stresses are explored. Discussions with family members on this issue are essential for providing them with information and obtaining their support. Counseling for both the patient and the family may be an option. Chapter 6, Psychosocial Aspects of Lupus, explores these issues in further detail.
Protection from Direct Sunlight
An abnormal reaction to the ultraviolet (UV) rays of the sun, photosensitivity results in the development or exacerbation of a rash that is sometimes accompanied by systemic symptoms. Photosensitivity is common in Caucasian patients. All people with lupus should avoid direct, prolonged exposure to the sun. Sun-sensitive patients should frequently apply a sunscreen. The best sunscreen is one that protects against both UVA and UVB rays. To get adequate protection, patients should be advised to select either a “broad-spectrum protection” sunscreen with an SPF of at least 15, or one that contains micronized zinc oxide or titanium dioxide, both of which block UVA and UVB light. Sun-sensitive patients should also avoid unprotected exposure between 10 a.m. and 4 p.m., and wear protective clothing, such as wide-brimmed hats and long sleeves. In severe cases, patients may wish to purchase special UV-blocking clothing. People with lupus should be aware that UV rays are reflected off water and snow, and that glass, such as car windows, does not provide total protection from UV rays.
People with lupus should also know that fluorescent and halogen lights may emit UV rays and can aggravate lupus. This may be an issue for patients who work indoors, in places with these kinds of lights. Sunscreen and protective clothing can help minimize exposure, and plastic devices are available that block UV emissions from fluorescent or halogen light bulbs.
Diet and Nutrition
A well-balanced diet is essential in maintaining good health for all people, including people with lupus. A low-fat, low-cholesterol diet is recommended, given the increased risk of heart disease in SLE. When fluid retention, hypertension, kidney disease, or other problems are present, a restricted diet plan may be prescribed. Also, food intolerances and allergies may occur. The health professional should make a note of the patient’s dietary history and suggest diet counseling if appropriate, especially if the patient has a problem with weight gain, weight loss, gastrointestinal (GI) distress, or food intolerances. Nutritional considerations in treating people with lupus are discussed further in Chapter 4, Care of the Lupus Patient.
Treatment of Infection
Prompt recognition and treatment of infection is essential for those with lupus. However, cardinal signs of infection may be masked because of SLE treatments. For example, a fever may be suppressed because antiinflammatory therapy is being given. When an infection is being treated, the health professional should be alert to medication reactions, especially to antibiotics. Sulfonamide antibiotics may increase the risk of a lupus flare.
Pregnancy and Contraception
Spontaneous abortion and premature delivery are more common for women with SLE than for healthy women. To minimize risks to both mother and baby, a pregnant woman with lupus should be closely supervised by an obstetrician familiar with lupus and high-risk pregnancies. As long as a woman is not hypercoagulable, oral contraceptives may be considered for contraception, as they do not increase flares.
Surgery may exacerbate the symptoms of SLE. Hospitalization may be required for otherwise minor procedures, and postoperative discharge may be delayed. If it is elective, the surgery should be postponed until lupus activity is under control.
Immunizations with killed vaccines have not been shown to exacerbate SLE. Influenza and pneumococcal vaccines are routinely recommended for SLE patients. A patient with lupus should consult her or his doctor before receiving any immunizations, even routine ones.
Some patients never require medications, and others take them only as needed or for short intervals, but many require constant therapy with variable doses. Despite their usefulness, no drugs are without risks. Medications frequently used to control the symptoms are nonsteroidal anti-inflammatory drugs (NSAIDs), antimalarials, corticosteroids, immunosuppressives, and intravenous immunoglobulins. Other medications may be necessary to control specific manifestations. Before prescribing a medication, it is helpful to scrutinize a patient’s past response to treatments. A careful drug history should be taken; in particular, hypersensitivities or allergies to certain drugs should be noted. Patient and family education about medications and their side effects is essential. Chapter 5, Medications Used to Treat Lupus, presents more detailed information on this issue, and Chapter 7, Patient Information, includes relevant information for patients.
For the patient with lupus, the emotional aspects of dealing with a chronic disease can be overwhelming. They can also make the patient feel isolated from friends, family, and coworkers. Grief, depression, and anger are common reactions of patients with lupus.
Those with lupus and their families deal with the disease in strikingly different ways. Managing the ups and downs of the disease may put strains on relationships and marriages. Younger patients may fail to assert their independence or develop a life away from home if they feel they cannot cope with their disease on their own. Family members are often confused and frightened over the changes they see. They need guidance on and constructive suggestions for helping the patient. Children of people with lupus, particularly those too young to really understand the disease, may need special help in coping with their parent’s illness. It is in these areas that the patient, family, and support systems need to be assessed, encouraged, and guided so that they work together as a team. By allowing the patient and her or his family the time and freedom to move through different emotional phases without criticism and unrealistic expectations, you will facilitate acceptance of the disease. The health professional can have a major role in helping a patient adjust, and can help with referrals to a social worker, counselor, or community resource, if needed. Chapter 6, Psychosocial Aspects of Lupus, discusses these issues in more detail.
The psychosocial issues involved in defining, diagnosing, and treating lupus have implications for the way the nurse or other health professional works with a patient who has lupus. For example, a person who is newly diagnosed with lupus needs help in getting current, accurate information about the disease and in defining realistic expectations and goals. The Patient Information Sheets in Chapter 7 can help. The health professional can clarify information with the patient’s doctor, make rounds with the doctor, and act as a liaison between the patient and the doctor, if needed. Frequently, many doctors are involved in caring for a patient with lupus at one time. This may increase the patient’s confusion and leave gaps in information. Emotional support for the patient is essential. Being available for questions, providing reassurance, and encouraging discussion of fears and anxieties are all crucial roles that the nurse can play.
The person with lupus who is hospitalized during a flare requires symptomatic nursing care. It is important to note that objective data, such as anemia or sedimentation rate, may not support subjective complaints of fatigue or pain. Careful head-totoe assessment and documentation of all symptoms and complaints are important. Symptomatology changes constantly, so frequent reassessment is necessary. Reevaluations validate a patient’s concerns and alert the doctor to changes that may be transient yet significant.
The patient’s tolerance for physical activity and need to control what she or he can do should be respected. The patient should be involved in developing a care plan and daily schedule of activities.
The best way to treat lupus is to listen to the patient, whether she or he was diagnosed today or years ago. The patient’s support systems can be expanded to include pamphlets and books, physical or occupational therapy, vocational rehabilitation, homemaker services, the Visiting Nurses Association (VNA), the Lupus Foundation of America (LFA), the SLE Foundation, and the Arthritis Foundation (AF).
Lupus is a challenge to everyone concerned. The health professional has a key role in its management. Accurate documentation, supportive care, emotional support, patient education, and access to community resources will provide the patient and her or his family with the tools they need to cope effectively.