SYSTEMIC LUPUS ERYTHEMATOSUS
The onset of disease
The overall prevalence of SLE is estimated to be
1 per 2500 persons. It occurs 10 times more
frequently in women than in men and
approximately three times more frequently in
African Americans than in Caucasians
(Wandstrat, CarrJohnson, Branch, et al., 2006).
Pathophysiology
 SLE is a result of disturbed immune
regulation that causes an exaggerated
production of autoantibodies.
 This immunoregulatory disturbance is
brought about by some combination of
genetic factors, hormonal factors (as
evidenced by the usual onset during the
childbearing years), and environmental
factors (eg, sunlight, thermal burns).
 Certain medications, such as
hydralazine (Apresoline), procainamide
(Pronestyl), isoniazid (INH),
chlorpromazine (Thorazine), and some
antiseizure medications, have been
implicated in chemical or drug-induced
SLE.
 Specifically, B cells and T cells both
contribute to the immune response in
SLE (Croker & Kimberly, 2005). B cells
are instrumental in promoting the onset
and flares of the disease (Tieng &
Peeva, 2008).
Clinical Manifestations
 SLE is an autoimmune systemic disease
that can affect any body system.
 Involvement of the musculoskeletal
system, with arthralgias and arthritis
(synovitis), is a common presenting
feature of SLE.
 Joint swelling, tenderness, and pain on
movement are also common.
Frequently, these are accompanied by
morning stiffness.
may be insidious or acute.
 Several different types of skin
manifestations may occur in patients
with SLE, including subacute cutaneous
lupus erythematosus, which involves
papulosquamous or annular polycyclic
lesions, and discoid lupus
erythematosus, which is a chronic rash
that has erythematous papules or
plaques and scaling and can cause
scarring and pigmentation changes.
 The most familiar skin manifestation
(occurring in more than 50% of patients
with SLE) is an acute cutaneous lesion
consisting of a butterfly-shaped rash
across the bridge of the nose and
cheeks (Bickley, 2007) (Fig. 54-2).
 In some cases of discoid lupus
erythematosus, only skin involvement
occurs.
 In some patients with SLE, the initial
skin involvement is the precursor to
more systemic involvement. The lesions
often worsen during exacerbations
(flares) of the systemic disease and
possibly are provoked by sunlight or
artificial ultraviolet light.
 Oral ulcers, which may accompany skin
lesions, may involve the buccal mucosa
or the hard palate, occur in crops, and
are often associated with
exacerbations.
 Pericarditis is the most common cardiac
manifestation.
 Women who have SLE are also at risk
for early atherosclerosis.
 Serum creatinine levels and urinalysis
are used in screening for renal
involvement.
 Early detection allows for prompt
treatment so that renal damage can be
prevented.
  Renal involvement may lead to
hypertension, which also requires
careful monitoring and management
 Central nervous system involvement is
widespread, encompassing the entire
range of neurologic disease. The varied
and frequent neuropsychiatric
presentations of SLE are now widely
recognized. These are generally
demonstrated by subtle changes in
behavior patterns or cognitive ability.
Diagnosis and Assessment
 Diagnosis of SLE is based on a complete
history, physical examination, and
blood tests
 Typically, assessment reveals classic
symptoms, including fever, fatigue,
weight loss, and possibly arthritis,
pleurisy, and pericarditis.
 Interactions with the patient and family
may provide further evidence of
systemic involvement.
 In addition to the general assessment
performed for any patient with a
rheumatic disease, assessment for
known or suspected SLE has special
features.
 The skin is inspected for erythematous
rashes. Cutaneous erythematous
plaques with an adherent scale may be
observed on the scalp, face, or neck.
 Areas of hyperpigmentation or
depigmentation may be noted,
depending on the phase and type of the
disease.
 The patient should be questioned about
skin changes (because these may be
transitory) and specifically about
sensitivity to sunlight or artificial
ultraviolet light.
 The scalp should be inspected for
alopecia and the mouth and throat for
ulcerations reflecting gastrointestinal
involvement.
 Cardiovascular assessment includes
auscultation for pericardial friction rub,
possibly associated with myocarditis
and accompanying pleural effusions.
The pleural effusions and infiltrations,
which reflect respiratory insufficiency,
are demonstrated by abnormal lung
sounds.
 Papular, erythematous, and purpuric
lesions developing on the fingertips,
elbows, toes, and extensor surfaces of
the forearms or lateral sides of the
hand that may become necrotic suggest
vascular involvement. Joint swelling,
tenderness, warmth, pain on
movement, stiffness, and edema may
be detected on physical examination.
 The joint involvement is often
symmetric and similar to that found in
RA.
 The neurologic assessment is directed
at identifying and describing any central
nervous system changes. The patient
and family members are asked about
any behavioral changes, including
manifestations of neurosis or psychosis.
 Signs of depression are noted, as are
reports of seizures, chorea, or other
central nervous system manifestations.
 No single laboratory test confirms SLE;
rather, blood testing reveals moderate
to severe anemia, thrombocytopenia,
leukocytosis, or leukopenia and
positive.
 Other diagnostic immunologic tests
support but do not confirm the
diagnosis.
 Medical Management
 Treatment of SLE includes management
of acute and chronic disease.
 Although SLE can be life-threatening,
advances in its treatment have led to
 improved survival and reduced  exposure can increase disease activity
morbidity. or cause an exacerbation, patients
 Acute disease requires interventions should be taught to avoid exposure or
directed at controlling increased to protect themselves with sunscreen
disease activity or exacerbations that and clothing. Because of the increased
can involve any organ system. risk of involvement of multiple organ
 Disease activity is a composite of clinical systems, patients should understand
and laboratory features that reflect the need for routine periodic screenings
active inflammation secondary to SLE. as well as health promotion activities.
 Management of the more chronic  A dietary consultation may be indicated
condition involves periodic monitoring to ensure that the patient is
and recognition of meaningful clinical knowledgeable about dietary
changes requiring adjustments in recommendations, given the increased
therapy. risk of cardiovascular disease, including
 The goals of treatment include hypertension and atherosclerosis.
preventing progressive loss of organ  The nurse instructs the patient about
function, reducing the likelihood of the importance of continuing
acute disease, minimizing disease- prescribed medications and addresses
related disabilities, and preventing the changes and potential side effects
complications from therapy. that are likely to occur with their use.
 Management of SLE involves regular  The patient is reminded of the
monitoring to assess disease activity importance of monitoring because of
and therapeutic effectiveness. the increased risk of systemic
involvement, including renal and
Nursing Management
cardiovascular effects.
 The most common nursing diagnoses
include fatigue, impaired skin integrity,
body image disturbance, and lack of
knowledge for self-management
decisions.
 The disease or its treatment may
produce dramatic changes in
appearance and considerable distress
for the patient.
 The changes and the unpredictable
course of SLE necessitate expert
assessment skills and nursing care with
sensitivity to the psychological
reactions of the patient.
 The patient may benefit from
participation in support groups, which
can provide disease information, daily
management tips, and social support.
Because sun and ultraviolet light
Kawasaki Disease  The child acts lethargic or irritable and
may have reddened and swollen hands
 Kawasaki disease (mucocutaneous
and feet.
lymph node syndrome) is a febrile,
 Soon the bulbar mucous membranes of
multisystem disorder that occurs almost
the eyes become inflamed
exclusively in children before the age of
(conjunctivitis) and the child develops a
puberty.
“strawberry” tongue and red, cracked
 It has replaced rheumatic fever as the
lips.
most likely cause of acquired heart
 A variety of rashes occur, often
disease in children.
confined to the diaper area. Cervical
 The peak incidence is in boys under 4
lymph nodes become enlarged.
years of age. The incidence is higher in
 As internal lymph nodes swell, children
late winter and spring.
may develop abdominal pain, anorexia,
 Vasculitis (inflammation of blood
and diarrhea.
vessels) is the principal (and life-
 Joints may swell and redden, simulating
threatening) finding because it can lead
an arthritic process.
to formation of aneurysm and
 White blood cell count and the ESR are
myocardial infarction (Rowley &
both elevated.
Shulman, 2007).
 About 10 days after the onset, a
 The cause of Kawasaki disease is
subacute phase begins.
unknown, but it apparently develops in
 The skin desquamates, particularly on
genetically predisposed individuals after
the palms and soles.
exposure to an as-yet-unidentified
 The platelet count rises; this increases
infectious agent. After the infection
the possibility of clotting, which could
(perhaps an upper respiratory
result in necrosis of distant body cells,
infection), altered immune function
particularly the fingertips, if they no
occurs.
longer receive adequate blood.
 An increase in antibody production
 Aneurysms may form in coronary
creates circulating immune (antibody–
arteries, compromising heart activity.
antigen) complexes that bind to the
Sudden death from accumulating
vascular endothelium and cause
thrombi or rupture of an aneurysm may
inflammation.
occur, making this the most dangerous
 The inflammation of blood vessels leads
phase.
to aneurysms, platelet accumulation,
 The convalescent phase (stage II) begins
and the formation of thrombi or
at about the 25th day and lasts until 40
obstruction in the heart and blood
days. Stage III lasts from 40 days until
vessels.
the ESR returns to normal.
Assessment  To be diagnosed with Kawasaki disease,
a child must manifest fever and four of
 Kawasaki disease begins with an acute
the typical symptoms shown in Box
phase (stage I) of high fever (102° to
41.11, plus echocardiographic
104° F [39.0° to 40.0° C]) that does not
confirmation of artery disease.
respond to antipyretics
  Children are followed by sequential
echocardiograms to monitor for
development of aneurysms.
1. Fever of 5 or more days’ duration
2. Bilateral congestion of ocular conjunctivae
3. Changes of the mucous membrane of the
upper respiratory tract, such as reddened
pharynx; red, dry, fissured lips; or
protuberance of tongue papillae (“strawberry”
tongue)
4. Changes of the peripheral extremities, such
as peripheral edema, peripheral erythema,
desquamation of palms and soles
5. Rash, primarily truncal and polymorphous
6. Cervical lymph node swelling
Therapeutic Management
 The administration of acetylsalicylic acid
(aspirin) or ibuprofen decreases
inflammation and blocks platelet
aggregation.
 Abciximab is a platelet receptor
inhibitor specific for Kawasaki disease
(Karch, 2009). IV immune globulin (IVIG)
can also be administered to reduce the
immune response (Oates-Whitehead et
al., 2009).
 Caution parents that children should
not receive routine immunizations
while taking IVIG or the immunization
will be ineffective. Steroids, which may
increase aneurysm formation, are
contraindicated.
 If the child is left with coronary artery
disease from stenosis of the coronary
arteries, coronary artery bypass surgery
may be necessary in the future.
 A child with Kawasaki disease is
uncomfortable from the joint
involvement, edema, pruritic rash,
abdominal discomfort, and the frequent
blood sampling necessary to monitor
the platelet count.
 The high fever can lead to dry, cracked
lips. Ibuprofen, administered for its
anti-inflammatory action, helps reduce
both thepain and itchiness (which is a
low level of pain).
 Provide additional comfort measures
such as rocking and holding.
 Protect edematous areas from
pressure; make certain clothing is not
constricting and irritating areas of rash.
 Applying lip balm protects lips from
drying and cracking.
 Because the fever remains high, offer
extra fluid to help maintain hydration
and reduce mouth tenderness.
 Keep the child free of heavy blankets or
clothing and prevent overexertion.
Monitor IV fluid to prevent fluid
overload.
 Children with Kawasaki disease lose
their appetite and generally eat poorly
because of the systemic illness, mouth
soreness from cracks and fissures, and
abdominal pressure from swollen
lymph nodes.
 Carefully monitor and record the child’s
intake and output. Encourage the child
to continue brushing his or her teeth
(use a soft toothbrush or a padded
tongue blade), even though the oral
mucous membrane is tender.
 Soft, nonirritating foods such as gelatin
(Jell-O) may be better tolerated than
foods that require chewing and acidic
fluids, such as orange juice, that might
sting.
 Observe for signs of gastrointestinal
obstruction, such as vomiting.
 Most children with Kawasaki disease
recover fully, but a few will need
cardiac bypass surgery to treat
 aneurysms that developed in the
coronary arteries.
 Nephroblastoma (Wilms’ Tumor)
Nephroblastoma (Wilms’ tumor) is a
malignant tumor that rises from the
metanephric mesoderm cells of the
upper pole of the kidney (McLean &
Castellino, 2008).
 It accounts for 20% of solid tumors in
childhood; there is no increased
incidence based on sex or race. It occurs
in association with congenital
anomalies such as aniridia (lack of color
in the iris), cryptorchidism,
hypospadias, pseudohermaphroditism,
cystic kidneys, hemangioma, and talipes
disorders.
 Some children with the disorder have a
deletion on chromosome 11. Without
therapy, metastatic spread by the
bloodstream is most often to the lungs,
regional lymph nodes, liver, bone, and,
eventually, brain.
Assessment
 A nephroblastoma is usually discovered
early in life (6 months to 5 years; peak
at 3 to 4 years), although it apparently
arises from an embryonic structure
present in the child before birth.
 Nephroblastomas distort the kidney
anteriorly so that the tumor is felt as a
firm, nontender abdominal mass.
 Parents sometimes are aware that their
Therapeutic Management
infant has a mass in the abdomen but
bring the infant to their health care
provider thinking that it is hard stool
from chronic constipation.
 Fathers may discover the tumor when
they toss a baby in the air, catch the
infant by the abdomen, and feel the
abdominal mass.
 Parents often report that the mass
seemed to appear overnight. This
actually can happen, because tumors
can hemorrhage into themselves,
doubling their size in a matter of hours.
Because of this, nephroblastoma may
manifest with hematuria and a low-
grade fever.
 The child may be anemic from lack of
erythropoietin formation by the
diseased kidney. Although hypertension
may also occur because of excessive
renin production, blood pressure is not
taken routinely in children of this age,
so the tumor is rarely discovered by this
method.
 A CT scan or ultrasound reveals the
primary tumor and any points of
metastasis.
 Kidney function studies, such as
glomerular filtration rate or blood urea
nitrogen, will be done to assess function
of the kidneys before surgery.
 Little time, however, can be allotted for
preoperative testing, because these
tumors metastasize rapidly as a result
of the large blood supply to the kidneys
and adrenal glands.
 It is important that the child’s abdomen
not be palpated any more than is
necessary for diagnosis, because
handling appears to aid metastasis.
Place a sign reading “No Abdominal
Palpation” over the child’s crib to
prevent this.”
 The tumor will be removed by
nephrectomy (excision of the affected
kidney).
 This is usually followed immediately by
radiation therapy (omitted in stage I
tumors) and by chemotherapy with
dactinomycin, doxorubicin, or
vincristine.
  The chemotherapy may be given at III:
varying intervals for as long as 15
Regional spread of disease beyond the kidney
months.
with residual abdominal disease postoperatively
 A second surgical procedure may be
IV:
scheduled after 2 or 3 months to
remove any remaining tumor. Metastases to lung, liver, bone, distant lymph
 If tumor involvement is bilateral, the nodes, or other distant sites V Bilateral disease
operative decisions obviously become
more complex. If the tumors are small,
both kidneys can be removed, leaving
functioning kidney cells intact. Or only
the kidney with the larger tumor may
be removed.
 Tumor sites are then treated with both
radiation and chemotherapy.
 Complications can occur from
nephroblastoma therapy.
 Both small bowel obstruction from
fibrotic scarring and hepatic damage
from radiation to the lesion can occur.
 Nephritis in the kidney also is a
possibility. In girls, radiation-related
damage to the ovaries may result in
sterility.
 Radiation to the lungs may result in
interstitial pneumonia; spine radiation
can result in scoliosis.
 Therapy for nephroblastoma is so
effective that about 90% of children
who had no metastatic spread at
diagnosis survive for at least 5 years.

STAGING

Stage Description I:

Tumor confined to the kidney and completely

removed surgically

II:

Tumor extending beyond the kidney but

completely removed surgically