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Definition
and Description
Symptoms
of SLE
Diagnosis
of SLE
Treatment
of SLE
Medications
for SLE
Psychosocial
Aspects
Health
Care Implications
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| Care for the patient with lupus erythematosus is a challenge
that 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 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 attended to. |
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Definition
and Description
Lupus
means "wolf." Erythematosus means
"redness." 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. Lupus can be categorized into three groups: discoid
lupus erythematosus, systemic lupus erythematosus, and drug-induced systemic
lupus erythematosus.
Discoid
lupus erythematosus (DLE) is characterized by a skin rash only.
It occurs in about 20% of patients with systemic lupus erythematosus.
The lesions are patchy, crusty, sharply defined skin plaques that may
scar. These lesions are usually seen on the face or other sun-exposed
areas. DLE may cause patchy, bald areas on the scalp and hypopigmentation
or hyperpigmentation in older lesions. Biopsy of a lesion will usually
confirm the diagnosis. Topical and intralesional corticosteroids are usually
effective for localized lesions; antimalarial drugs may be needed for
more generalized lesions. DLE only rarely progresses to systemic lupus
erythematosus.
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| Systemic
lupus erythematosus (SLE, or lupus) is a chronic, inflammatory,
multisystem disorder of the immune system. Lupus: A Patient Care Guide
for Nurses and Other Health Professionals is concerned primarily with
this form of lupus. In SLE, the body develops antibodies that react against
the person's own normal tissue. This abnormal response leads to the many
manifestations of SLE and can be very damaging. 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. African Americans and
Hispanics have a higher frequency of this disease than do Caucasians. SLE
also appears in the first-degree relatives of lupus patients more often
than it does in the general population, which indicates a strong 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, kidney, and brain. The disease is 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.
The disease tends to remit over time. Some patients never develop severe
complications, and the outlook is improving for those patients who do
develop severe manifestations.
Drug-induced
SLE develops after the use of certain drugs and has symptoms
similar to those of SLE. The characteristics of this syndrome are pleuropericardial
inflammation, fever, rash, and arthritis. Serologic changes also 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 SLE.
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| Drugs
Implicated as Activators of SLE
Drugs with proven association
- Chlorpromazine
- Hydralazine
- Isoniazid
- Methyldopa
- Procainamide
Drugs with possible association
- Beta blockers (e.g., acebutolol, atenolol, labetalol, metoprotolol,
oxprenolol, pindolol, practolol, and propranolol)
- Captopril
- Carbamazine
- Cimetidine
- Diphenylhydantoin (phenytoin)
- Ethosuximide
- Methimazole
- Penicillamine
- Phenazine
- Quinidine
From: The
Bulletin on the Rheumatic Diseases, copyright 1991. Used by
permission of the Arthritis Foundation. For more information, call
the Arthritis Foundation's information line: 1-800-283-7800.
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Symptoms
of SLE
Early symptoms of
SLE are usually 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. A range of clinical symptoms
are seen in patients with lupus over the lifetime of the disease.
Diagnosis
of SLE
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]),
a syphilis test (VDRL), urinalysis, blood chemistries, and erythrocyte
sedimentation rate (ESR). Abnormalities in these test results will guide
further evaluations. High-titer anti-nDNA antibody or anti-Sm antibody
are important indications of lupus. Specific immunologic studies, such
as those of complement components (e.g., C3 and C4) and other autoantibodies
(e.g., anti-La and anti-Ro), are used to help evaluate the patient's immune
status and to monitor the activity of the disease. 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 diagnostic criteria. If at least 4 of
the 11 criteria develop at one time or individually over any period of
observation, then the patient is likely to have SLE. However, a diagnosis
of SLE can be made in a patient having fewer than four of these symptoms.
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Symptoms
of SLE
- Arthralgia
- Arthritis
- Fever (>100
F)
- Skin rashes
- Anemia
- Kidney damage
- Pleurisy
- Facial rash
- Photosensitivity
- Alopecia
(hair loss)
- Raynaud's
phenomenon
- Seizures
- Mouth or
nose ulcers
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ACR
Criteria for Diagnosing SLE
- Malar rash
- Discoid rash
- Photosensitivity
- Oral ulcers
- Arthritis
- Serositis (pleuritis or pericarditis)
- Renal disorder (persistent proteinuria or cellular casts)
- Neurological disorder (seizures or psychosis)
- Hematologic disorder (anemia, leukopenia or lymphopenia on two
or more occasions, thrombocytopenia)
- Immunologic disorder (positive LE cell preparation, abnormal
anti-DNA or anti-Sm values, false-positive VDRL syphilis test)
- Abnormal ANA titer
Source: Tan
E. The 1982 required criteria for the classification of systemic
lupus erythematosus. Arthritis and Rheumatism 1982;25:1271-1277.
1982 American College of Rheumatology. Used with permission of
Lippincott-Raven Publishers.
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Treatment
of SLE
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 known allergens and aggravating factors are the mainstays
of lupus therapy. In addition, for female patients, pregnancy must be
planned for times when the disease is in remission.
Physical Rest
This basic component
of everyone's good health is essential for the lupus patient. 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 to activity and when the person
should stop for rest. It must be reinforced that this need for rest is
not laziness. Restful sleep of 8-10 hours per night, naps, and "timeouts"
during the day are basic guidelines; strict bed rest is usually not required.
Physical activity should be encouraged as the patient can tolerate it.
An individualized exercise routine may facilitate recovery from a flare
and promote well-being.
Emotional Rest
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 the family on this issue are essential
for providing information and in 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
Photosensitivity is an abnormal reaction to the ultraviolet (UV) rays
of the sun and results in the development or exacerbation of a rash that
is sometimes accompanied by systemic symptoms. About one-third of lupus
patients are photosensitive. All lupus patients should avoid direct, prolonged
exposure to the sun. Sun-sensitive patients should frequently apply a
sunscreen with a Sun Protection Factor (SPF) of at least 15, avoid unprotected
exposure between 10 a.m. and 4 p.m., and wear protective clothing, such
as wide-brimmed hats and long sleeves. Lupus patients 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.
Lupus patients 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 in offices lit by 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 lupus patients. There are currently no specific dietary recommendations
or limitations for those with lupus, but a restricted diet plan may be
prescribed when fluid retention, hypertension, kidney disease, or other
problems are present. Food intolerances and allergies may occur, but there
is no evidence that these are more common in lupus patients than in the
general population. 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 lupus patients are discussed further in Chapter
4, Care of the Lupus Patient.
Treatment of Infections
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 anti-inflammatory
therapy is being given. When an infection is being treated, the health
professional should be alert to medication reactions, especially to antibiotics.
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. The safety of oral contraceptives for women with
lupus is currently under investigation. The use of an intrauterine device
(IUD) is not recommended because of the lupus patient's increased risk
of infection.
Surgery
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 (including dental surgery and tooth extraction)
should be postponed until lupus activity subsides.
Immunizations
Immunizations with killed vaccines have not been shown to exacerbate SLE.
However, live vaccines with attenuated organisms are not advisable. A
lupus patient should consult her or his doctor before receiving any immunizations,
even routine ones.
Medications for SLE
SLE management should
include as few medications for as short a time as possible. 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, and immunosuppressives. 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, as these
may aggravate the lupus. 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.
Psychosocial
Aspects
For the lupus patient,
the emotional aspects of dealing with a chronic disease can be overwhelming.
They can also make a patient feel isolated from friends, family, and coworkers.
Grief, depression, and anger are common reactions of patients about their
lupus.
Lupus patients 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 and need guidance and constructive suggestions on helping
the patient. Children of lupus patients, 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. Allowing the patient and her or his family
time and freedom to move through different emotional phases without criticism
and unrealistic expectations 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.
Health
Care Implications
How lupus is defined,
diagnosed, and treated and the psychosocial issues involved have implications
for the way that the nurse or other health professional works with a patient
who has lupus. For example, a newly diagnosed lupus patient 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 lupus patient at one time. This
may increase the patient's confusion and leave gaps in information. Emotional
support to 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 lupus patient
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-to-toe
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 that patient 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), 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.
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Outline Credits Acknowledgments Introduction PDF Order Form Home
1. Erythematosus 2. Advances 3. Tests 4. Care 5. Medications
6. Psychosocial Aspects 7. Patient Info. 8. Resources
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National Institute of Arthritis and
Musculoskeletal and Skin Diseases (NIAMS)
National Institutes of Health (NIH)
Bethesda, Maryland 20892-2350
January 26, 1999
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