Lupus Erythematosus

General
DRG Category: 546
Mean LOS: 5.2 days
Description MEDICAL: Connective Tissue Disorders With CC
DRG Category: 595
Mean LOS: 7.7 days
Description MEDICAL: Major Skin Disorders With Major CC
Classification Section
Nursing Type Primary: acute care
Nursing Type Secondary: gender health
System Primary: immune
System Secondary: not applicable

Introduction
Lupus erythematosus is an autoimmune disease that affects the connective tissue of the body. The
Lupus Foundation of America reports that 1.5 million people have lupus in the United States. The
course of disease is variable and unpredictable, with episodes of remission and relapse. Only a small
percentage of patients (< 10%) have long-lasting remissions.

Lupus takes two forms. Systemic lupus erythematosus (SLE) is a multisystem inflammatory disease
that affects any body system but primarily the musculoskeletal, cutaneous, renal, nervous, and
cardiovascular systems. Discoid lupus erythematosus (DLE) is a less serious form of the disease that
primarily affects the skin. DLE is characterized by skin lesions of the face, scalp, and ears. Long-
standing lesions can cause scarring, hypopigmentation, and redness. Only 5% to 10% of patients with
DLE develop SLE. The multisystem nature of SLE places the patient at risk for multiple complications,
and the disease is ultimately fatal. The survival rates of patients with SLE are about 90% at 5 years and
80% at 10 years. The most common causes of death are renal failure and infections, followed by
neurological and cardiovascular disorders. In the last decades, therapy has reduced mortality from
lupus, with 5-year survival rates of more than 90% and increased the 20-year survival rates to 70%.

Causes
The cause of lupus erythematosus is not known. A familial association has been noted that suggests a
genetic predisposition, but a genetic link has not been identified. Environmental factors, susceptibility
to certain viruses, and an immune system dysfunction with production of autoantibodies are possible
causes. Hormonal abnormality and ultraviolet radiation are considered possible risk factors for the
development of SLE. Some drugs have been implicated as initiating the onset of lupus-like symptoms
and aggravating existing disease; they include hydralazine hydrochloride, procainamide hydrochloride,
penicillin, isonicotinic acid hydrazide, chlorpromazine, phenytoin, and quinidine.

Genetic Considerations
While familial clustering of SLE is relatively rare, twin studies have shown concordance in 24% to 59%
of identical twins and in only 2% to 5% of nonidentical twins, which suggests a significant genetic
predisposition. Genes encoding proteins of the complement system appear to have the strongest
association with SLE. Polymorphisms in the Toll-like receptor-5 (TLR5) gene are also associated with
SLE.

Gender, Ancestry, and Life Span Considerations

SLE occurs most frequently in females between the ages of 15 and 45, with the average age of onset at
30 years. Approximately 90% of people with SLE are women; it is 15 times more likely to occur in
women than men during childbearing years. It is also more prominent in people with Asian,
Latino/Hispanic, and African American ancestry than other populations. DLE is more common in women
than in men, and approximately 60% of cases are female patients in their late 20s or older.

Global Health Considerations

Global data on prevalence vary widely. Prevalence is higher in Western Europe and lower in Africa and
China.
Assessment
History

Initial symptoms may involve one organ only or multiple systems. Symptoms vary from mild and
infrequent to persistent and life-threatening. Take a careful history with a focus on both systemic and
single-organ symptoms. Systemic symptoms include fatigue, malaise, weight loss, anorexia, and fever.

Musculoskeletal and cutaneous symptoms include joint and muscle pain, puffiness of hands and feet,
joint swelling and tenderness, hand deformities, and skin lesions such as the characteristic "butterfly
rash" (fixed reddish and flat rash that extends over both cheeks and the bridge of the nose),
maculopapular rash (small, colored area with raised red pimples), sensitivity to the sun, photophobia,
vascular skin lesions, leg ulcers, oral ulcers, and hair loss.

Other symptoms originate in the genitourinary tract (menstrual abnormalities, amenorrhea,

spontaneous abortion), central nervous system (visual problems, memory loss, mild confusion,
headache, seizures, psychoses, loss of balance, depression), hematologic system (venous or arterial
clotting, bleeding tendencies), cardiopulmonary system (chest pain, shortness of breath, lung
congestion), and gastrointestinal system (nausea, vomiting, difficulty swallowing, diarrhea, and bloody
stools).

Ask if there is a family history of SLE. Establish any immune system dysfunction or recent viral
infections. Ask if the patient has a history of hormonal abnormality or ultraviolet radiation. Ask the
patient if he or she is taking or has taken any of the medications implicated as initiating lupus-like
symptoms.

Physical Examination
Common symptoms include fever, joint pain, and rash. Inspect the integumentary system
thoroughly, including the mucous membranes, to determine the site of skin rashes and lesions. Check
for lesions and necrosis on the fingertips, toes, and elbows; these may be caused by inflammation of
terminal arterioles. Examine the hairline for any signs of hair loss. Assess the patient's extremities and
joints for signs of arthritis, lymphadenopathy, and peripheral neuropathy. Determine the extent of
range of motion and movement of extremities and level of joint discomfort. Auscultate the lungs and
heart to determine the presence of a pleural or pericardial friction rub. Palpate the spleen and liver to
determine the presence of tenderness, splenomegaly, or hepatomegaly. Examine the patient's urine for
hematuria, proteinuria, and casts.

Assess for fever, pallor, and signs of bleeding, including petechiae and bruising. Check the patient's
blood pressure because increased blood pressure might indicate kidney involvement.

Psychosocial

A patient is facing a chronic and often debilitating disease that can be fatal. The patient may have
problems maintaining professional and family roles and may experience loss over a deteriorating
health status. The loss of childbearing potential is another loss experience for some women. Lupus is
associated with an increased incidence of spontaneous abortion, fetal death, and prematurity. Assess
the patient's and family's ability to cope with the illness. Determine the level of anxiety, fear, and
depression.

Diagnostic Highlights
Tests: Complete blood count, antinuclear antibody, urinalysis, anti-DNA antibody, complement levels,
anti–double-stranded DNA antibody assay, blood urea nitrogen, creatinine, creatinine clearance. To make
the diagnosis of lupus, the patient needs to have at least four of the criteria listed in the following table:
Criteria Explanation
Malar rash Flat or raised rash on the cheeks or cheekbones
Discoid rash Disklike circular raised patches or rashes, often on the face or
scalp
Photosensitivi Skin rash as a result of an unusual reaction to sunlight
ty
Oral ulcers Sores, usually painless, on the lips and in the mouth
Arthritis Tenderness or swelling of two or more peripheral joints
Serositis Pleuritis or pericarditis
Criteria Explanation
Renal Urinalysis shows proteinuria or cellular casts (red blood cell,
disorders hemoglobin, granular, tubular, or mixed casts)
Neurological Seizures, psychosis
disorders
Hematological Hemolytic anemia, leukopenia (decreased white blood cell
disorders count), lymphopenia (decreased lymphocytes),
thrombocytopenia (decreased platelets)
Immunologica Presence of anti-DNA antibody (antibodies to native DNA that
l disorders indicate autoimmune activity), anti-Sm nuclear antigen
(antinuclear antibody active against acidic nuclear proteins),
antiphospholipid antibodies (family of immunoglobulins active
against phospholipids)
Antinuclear Presence of antinuclear antibody (antibodies the body
antibody produces against its own DNA and nuclear material; antibodies
cause tissue damage)

Primary Nursing Diagnosis

Diagnosis: Alteration in comfort related to joint pain or peripheral nerve inflammation or dysfunction;
pain (acute) related to inflammation
Outcomes: Comfort level; Pain control behavior; Pain level; Symptom severity
Interventions: Analgesic administration; Anxiety reduction; Environmental management: Comfort;
Pain management; Medication management; Teaching: Individual; Prescribed activity/exercise

Planning Implementation
Collaborative

Much of the therapy is pharmacologic. General supportive therapy includes adequate sleep and
avoidance of fatigue because mild disease exacerbations may subside after several days of bedrest. A
physical therapy program is important to maintain mobility and range of motion without allowing the
patient to get overtired. If the kidneys are involved, renal dialysis or transplantation may be required.

Pharmacologic Highlights:
Medication or Dosage Description Rationale
Drug Class
Corticosteroids Varies with Prednisone, 1–2 mg/kg Control SLE in most
drug PO qd; severe or life-
methylprednisolone threatening cases
500 mg IV (glomerulonephritis,
debilitation from
symptoms)
Choline 500 mg–1.5 g Nonaceylated Manages the
magnesium PO bid/tid; salicylates inflammatory
trisalicylate maintenance process
dose: 1–4.5
g/day PO
NSAIDs Varies with Salicylates: Aspirin, Treat the joint pain
drug choline, salsalate; and swelling; should
nonsalicylates: be avoided in
diclofenac, ibuprofen, patients with active
Medication or Dosage Description Rationale
Drug Class
indomethacin, nephritis
naproxen, sulindax
Hydroxychloroqu 400–600 mg Antimalarial Reduces rash,
ine (Plaquenil) PO daily for 5– photosensitivity,
10 days, arthralgias, arthritis,
gradually alopecia, and
increasing dose malaise
until effective;
maintenance is
usually 200–
400 mg/day PO
Immunosuppress Varies with Azathioprine (Imuran), Suppress immune
ives drugs cyclophosphamide system and reduce
(Cytoxan, Neosar), consequences of
thalidomide (Thalomid), disease
methotrexate (Trexall),
immune globulin IV
(Sandoglobulin,
Gammagard,
Gamimune, Gammar-P)
Other Drugs: Monoclonal antibody (rituximab [Rituxan]). Topical steroids are often used to treat skin
rashes. Anticonvulsants may be necessary if seizures occur. When the disease is not responsive to
conventional therapies, trials of immunosuppressive (cytotoxic or alkylating) agents may be
considered. These agents, however, have serious side effects, including the risk of malignancy and
bone marrow suppression.
Independent

The pain and discomfort of SLE can be physically and mentally debilitating. Encourage the patient to
maintain activity when the symptoms are mild or in remission. Encourage patients to pace all activity
and to allow for adequate rest. Hot packs may relieve joint pain and stiffness. If the patient has
Raynaud's phenomenon (abnormal vasoconstriction of the extremities), use warmth to relieve
symptoms and protect the patient's hands from injury.

Support the patient's self-image by encouraging good grooming. Suggest hypoallergenic cosmetics,
shaving products, and hair products. Encourage the patient to use a hair stylist or barber who
specializes in caring for people with scalp disorders and to protect all body surfaces from direct
sunlight. The patient should use sunscreen with a protective factor of at least 20 and wear a hat and
long sleeves while in the sun. Note that certain drugs (tetracycline) and food (figs, parsley, celery)
augment the effects of ultraviolet light and therefore should be avoided.

Fatigue and stress can lead to exacerbations of the illness. Explore ways for the patient to get adequate
rest. Because the patient's immune system may have a diminished capacity, encourage the patient to
avoid exposure to illness.

Explore the meaning of the chronic illness and coping strategies with the patient. Allow adequate time
to discuss fears and concerns. A referral to a support group or counselor may also be necessary.

Evidence Based Practice Health Policy

Hanly, J.G., Su, L., Omisade, A., Farewell, V.T., & Fisk, J.D. (2012). Screening for cognitive impairment in
systemic lupus erythematosus. The Journal of Rheumatology, 39(7), 1371–1377.
 Investigators compared a group of 68 patients with SLE to a group of 33 patients with rheumatoid arthritis
and found increased perceived cognitive impairment among the patients with SLE (mean Cognitive Symptom
Inventory scores, 33.6 versus 29.4; p = 0.041).
 Among patients with SLE, decreased cognitive functioning was significantly associated with increased
reports of anxiety and depression (p < 0.001).
Documentation Guidelines
 Physical changes: Vital signs, particularly blood pressure and temperature; daily weight, intake and output;
signs of bleeding or tarry stools, petechiae, bruising, pallor
 Physical changes: Location site and description of any skin lesions or rashes and overall condition of the
skin
 Physical changes: Presence of any seizure activity, visual disturbances, headaches, personality changes, or
memory deficits
 Tolerance to activity, level of fatigue, patient's ability to perform activities of daily living and range of
motion of extremities; note the extent of joint involvement and the presence of tingling, numbness, or weakness

Discharge and Home Healthcare Guidelines

Teach the patient the purpose, dosage, and possible side effects of all medications. Explain to the
patient the disease process, the purpose of treatment regimens, and the importance of compliance.
Teach the patient when to seek medical attention. Teach the patient to wear a Medic Alert bracelet
noting the disease and medications so appropriate action can be taken in an emergency.

Teach the female patient the importance of planning pregnancies with medical supervision because
pregnancy is likely to cause an exacerbation of the disease.

Discuss all precipitating factors that need to be avoided, including fatigue, vaccination, infections,
stress, surgery, certain drugs, and exposure to ultraviolet light. Teach the patient how to minimize
ultraviolet exposure. Teach the patient to avoid strenuous exercise instead striving for a balance. Stress
the importance of adequate nutrition. Small, frequent meals may be better tolerated. Any cosmetics
should be approved by the physician and should be hypoallergenic. Encourage the patient to contact
the Arthritis Foundation, the Lupus Foundation, and other appropriate support groups that are available
in the area.